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Clinical report
Perivascular epitheliod cell tumors: Study of three gynecological cases
Tumores de células epitelioides perivasculares: estudio de 3 casos ginecológicos
Inmaculada Nicolása,c,
Corresponding author
innipe@hotmail.com

Corresponding author.
, Pere Fustéa, Adela Sacob, Jaume Ordib,c, Aureli Tornéa
a Institute Clinic of Gynecology, Obstetrics, and Neonatology, Hospital Clínic – Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
b Department of Pathology, Hospital Clínic, University of Barcelona, Barcelona, Spain
c Institut de Salut Global de Barcelona (ISGlobal), Barcelona, Spain
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        "titulo" => "Tumores de c&#233;lulas epitelioides perivasculares&#58; estudio de 3 casos ginecol&#243;gicos"
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          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Gynecological PEComas&#46; &#40;a&#41; Uterine&#46; MRI&#58; Solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass &#40;blue arrow&#41; with necrotic areas &#40;white star&#41;&#46; &#40;b&#41; Uterine&#46; PET&#58; Hypermetabolic uterine mass &#40;black arterisk&#41; &#40;SUVmax 22&#41;&#46; &#40;c&#41; Vaginal&#46; MRI&#58; Isointense vaginal lesion in T1&#44; with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm &#40;white arterisk&#41;&#46; &#40;d&#41; Retroperitoneal&#46; MRI&#58; Cystic multiloculated mass with a solid area which extends through the retroperitoneum &#40;left para-aortic side&#41; from the renal area to the left inguinal region &#40;white arrow&#41;&#46; &#40;e&#41; Retroperitoneal&#46; PET&#58; Retroperitoneal lesion with low metabolic activity &#40;white arrow&#41; &#40;SUVmax 1&#46;9&#41;&#46; &#40;f&#41; Pulmonar lymphangioleomyomatosis CT&#58; Multiple fine wall cystic images in lung parenchyma of different sizes greater than 14<span class="elsevierStyleHsp" style=""></span>mm &#40;small white arrows&#41;&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Perivascular epitheliod cell tumors &#40;PEComa&#41; are rare mesenchymal tumors composed of histologically distinctive perivascular epithelioid cells &#40;PEC&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> They are rare in the field of gynecology&#44; heterogeneous clinically&#44; which makes them difficult for gynecologists to consider as a possible diagnostic option&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report three cases &#40;uterine and vaginal PEComa in two menopausal patients and a retroperitoneal PEComa in a premenopausal patient&#41; that led to differential diagnosis with other more common gynecological tumors&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">In 2002 PEComas were classified by the WHO as tumors involving soft tissues &#40;stomach&#44; intestines&#44; lungs&#44; female reproductive organs and genitourinary organs&#41;&#46; They are more frequent in women &#40;ratio over 7&#58;1&#41; and are occasionally associated with the tuberous sclerosis complex &#40;TSC&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> The histological characteristics of these tumors are&#58; co-expression of melanocytic and muscle markers &#40;smooth muscle actin&#44; HMB45&#44; Melan-A&#44; S-100&#41;&#44; epithelioid to spindle cellular shapes with ample clear to eosinophilic cytoplasm and arrangement around blood vessels&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Tumors involving the gynecologic tract account for just over 40&#37; of the cases&#44; and the uterus is the most commonly reported site&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Materials and methods</span><p id="par0025" class="elsevierStylePara elsevierViewall">From January 2013 to December 2017&#44; 3 women with gynecological PEComas were diagnosed at the Gynecology Unit &#40;Hospital Clinic Barcelona&#41;&#46; They had given their informed consent to store and dispose of the biological material obtained during their care process&#44; and to use the clinical information for research purposes&#46; We retrospectively reviewed their clinical records&#44; the follow-up and the last status&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Results</span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0080">Case 1&#58; PEComa of the uterus</span><p id="par0030" class="elsevierStylePara elsevierViewall">A 68-year-old woman&#44; presented abdominal pain of a 1-year evolution and genital bleeding with loss of 5<span class="elsevierStyleHsp" style=""></span>kg of weight over 6 months&#46; Clinical examination highlighted a pelvic mass of approximately 15<span class="elsevierStyleHsp" style=""></span>cm&#46; A gynecological ultrasound showed a distorted uterus infiltrated by a large heterogeneous solid tumor of 15<span class="elsevierStyleHsp" style=""></span>cm and increased vascularization &#40;Doppler score 3&#47;4&#41;&#46; Abdominopelvic magnetic resonance imaging &#91;MRI&#93; showed a solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass with necrotic areas inside with significantly restricted diffusion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>a&#41;&#46; Positron emission tomography with 2-deoxy-2-&#91;fluorine-18&#93; fluoro-<span class="elsevierStyleSmallCaps">d</span>-glucose integrated with computed tomography &#40;<span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT&#41; showed a large hypermetabolic uterine mass&#44; with no signs of loco-regional or distant extension &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>b&#41;&#46; Upon suspicion of a possible malignant uterine-ovarian neoplasm&#44; tumor markers were requested&#46; CA-125&#44; CA19-9&#44; CEA&#44; and HE4 levels were within normal limits&#46; The endometrial biopsy was negative for malignancy&#46; Hysterectomy and double salpingo-oophorectomy were performed by laparotomy&#46; The provisional perioperative diagnosis was PEComa&#44; without being able to rule out other malignancies&#46; Omentectomy and pelvic nodal debulking due to intraoperative suspicion of lymphadenopathies was added&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The definitive histological study showed a large multinodular mass&#44; with areas of yellowish coloration&#44; measuring 16<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>5<span class="elsevierStyleHsp" style=""></span>cm compatible with malignant uterine PEComa &#40;size &#62;5<span class="elsevierStyleHsp" style=""></span>cm&#44; infiltrative growth pattern&#44; necrosis and vascular invasion&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The patient did not receive adjuvant treatment and remained disease free 9 months after surgery&#46;</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Case 2&#46; PEComa of the vagina</span><p id="par0040" class="elsevierStylePara elsevierViewall">A 72-year-old woman&#44; was referred for discomfort&#44; vaginal pressure and difficulty in emptying the bladder&#46; Clinical examination revealed a brownish vaginal tumor of 3<span class="elsevierStyleHsp" style=""></span>cm&#44; with a solid multinodular appearance&#44; pedunculated and inserted in the middle third of the lateral wall of the vagina&#46; MRI showed a well-defined tumor&#44; being iso-hyperintense in T1 and T2&#44; without restricted diffusion&#44; with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm and without extramural extension &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>c&#41;&#46; Cyfra 21&#46;1&#44; CEA and SCC levels were within normal limits&#46; A biopsy was performed&#44; obtaining a histological diagnosis of PEComa&#46; Wide local vaginal excision with free margins was performed&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical and histological criteria were compatible with a benign PEComa &#40;size 3<span class="elsevierStyleHsp" style=""></span>cm&#44; no infiltrative growth pattern&#44; no necrosis or vascular invasion&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The patient was disease free at 5 months postoperatively&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Case 3&#46; PEComa of the retroperitoneum</span><p id="par0050" class="elsevierStylePara elsevierViewall">A 42-year-old&#44; nulliparous woman presented for a second opinion because of a large retroperitoneal mass suspected of recurrence of PEComa&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">The patient referred a history of&#58; bilateral ovarian cystectomy for endometriosis at 20 years of age&#44; myomectomy and right cystectomy for endometriosis at age 26&#44; and myomectomy and uterine adenomatoid tumor excision at 36 years of age&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">At age 36 the patient was diagnosed with a 30<span class="elsevierStyleHsp" style=""></span>mm retroperitoneal lesion of cystic appearance&#46; Resection by laparoscopy of the retroperitoneal lesion was performed with a diagnosis of PEComa&#46; The paraffin block of the tissue from the previous biopsy was reanalyzed in our hospital&#44; and the diagnosis of PEComa was confirmed &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Over a 6-year follow-up the patient showed no evidence of recurrence&#46; Thereafter&#44; a control MRI showed a cystic multiloculated mass with a solid area extending through the retroperitoneum &#40;left para-aortic side&#41; from the renal area to the inguinal region&#44; suggestive of relapse of the basal disease&#44; for which the patient requested a second opinion &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>d&#41;&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleSup">18</span>F-FDG PET&#47;CT showed a multicystic retroperitoneal mass&#44; with a metabolism similar to that of the adjacent tissues &#40;SUVmax 1&#46;9&#41;&#44; suggesting recurrence of PEComa with low metabolic activity&#46; No pathological deposits were identified in the remaining body sites studied &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>e&#41;&#46; A chest scan was performed showing multiple fine wall cystic images varying in size greater than 14<span class="elsevierStyleHsp" style=""></span>mm were observed in the lung parenchyma&#44; being suggestive of lymphangioleomyomatosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>f&#41;&#46;</p><p id="par0075" class="elsevierStylePara elsevierViewall">The Gynecological Tumor Committee of our hospital considered the final diagnosis of lymphangioleomyomatosis associated with a recurrent retroperitoneal PEComa and proposed biological treatment with immunosuppressive therapy&#46; The patient received 2<span class="elsevierStyleHsp" style=""></span>mg of sirolimus daily in an oncological reference center authorized for treatment and monitoring of therapy with mTOR inhibitors&#44; with good tolerance&#46; After 8 months of follow-up&#44; the retroperitoneal lesions disappeared&#44; and the lung lesions remained stable&#46; The patient is undergoing surveillance and remains stable at 21 months of follow-up post relapse&#46;</p></span></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0095">Discussion</span><p id="par0080" class="elsevierStylePara elsevierViewall">In the field of gynecology&#44; PEComas are rare tumors which are difficult to diagnose&#46; These tumors may present with a benign and sometimes malignant behavior&#44; leading to suspicion of malignant oncological entities&#46;</p><p id="par0085" class="elsevierStylePara elsevierViewall">Some clinical&#44; radiological and immunohistochemical characteristics are shared by these tumors&#44; although there is no pathognomonic pattern for PEComas&#46; Cases associated with tuberous sclerosis are rarely reported&#44; and none of our patients were diagnosed with this&#46;</p><p id="par0090" class="elsevierStylePara elsevierViewall">Initially&#44; PEComas were considered to be benign tumors&#44; but with the progressive description of cases a small subset of PEComas were reported to present local recurrences or distant metastases&#46; Therefore&#44; Folpe et al&#46; designed a classification based on several prognostic factors and categorized PEComas into three prognostic groups &#40;benign&#44; uncertain malignant potential or malignant tumors&#41; according to the following six risk factors&#58; &#40;1&#41; tumor size &#8805;5<span class="elsevierStyleHsp" style=""></span>cm&#44; &#40;2&#41; infiltrative growth pattern&#44; &#40;3&#41; high grade nuclear cellularity&#44; &#40;4&#41; mitotic rate &#62;1&#47;50 high power fields&#44; &#40;5&#41; necrosis or &#40;6&#41; vascular invasion&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> In some cases&#44; there is no consensus regarding treatment&#44; and it is not individualized according to prognostic factors&#46; Surgical treatment is the standard procedure&#46; There is no protocol of adjuvant treatment&#44; and there is little information available regarding chemotherapy and radiotherapy in PEComa&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Regarding other adjuvant treatments&#44; some recent results with the use of mTOR inhibitors seem to be promising in refractory or malignant cases&#46; PEComas as well as LAM and AML show constitutive activation of the mTOR pathway &#40;mammalian target of rapamycin complex 1&#41; derived from genetic alterations of the TSC1 or TSC2 locus&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Cases treated with mTOR inhibitors are scarce but encouraging&#46; There are small series of cases in the literature reporting response rates of 50&#8211;100&#37;&#44; with patients being treated with mTOR inhibitors after surgery&#46; Some cases with contraindications for primary surgical treatment demonstrated a reduction of tumor size with the use of mTOR inhibitors&#44; thereafter making surgery possible&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#8211;5</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Review of 25 cases in the literature&#44; demonstrates the heterogeneity of the cases reported to date &#40;different locations&#44; treated with chemotherapy before treatment with mTOR inhibitors&#44; receiving neoadjuvant treatment&#44; without previous surgery&#41; &#40;<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>&#41;&#46; In our evaluation&#44; we defined complete response as radiological disappearance of the lesions&#44; while partial response was considered as a decrease in lesion size and progression to worsening before 12 months&#46; Therefore&#44; response was achieved in 76&#46;9&#37; &#40;20&#47;26&#41; of the cases&#44; being complete in 30&#46;8&#37; &#40;8&#47;26&#41; and partial in 46&#46;1&#37; &#40;12&#47;26&#41;&#44; and 23&#46;1&#37; &#40;6&#47;26&#41; of the cases showed progression&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2&#44;3&#44;5&#8211;10</span></a></p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0105" class="elsevierStylePara elsevierViewall">Here&#44; we presented three cases of PEComa with different clinicopathological features &#40;<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The histological and immunohistochemical study was essential for the diagnosis in our patients&#46; PEComas can co-express melanocytic and muscle immunohistochemical markers&#44; but not all the tumors have to express the same marker&#46;</p><p id="par0110" class="elsevierStylePara elsevierViewall">In our report only the preoperative study of the case of vaginal PEComa was conclusive&#44; given the possibility of histological study prior to surgery&#44; but in the remaining two cases post-surgical study was required&#46; The perioperative diagnostic approach carried out in our center in the case of uterine PEComa is of note&#44; taking into account the infrequent nature of this neoplasm&#46; The complexity of these unusual gynecological tumors justifies the referral of these patients to oncological reference centers&#46;</p><p id="par0115" class="elsevierStylePara elsevierViewall">Radiological studies and the clinical manifestations of PEComas are not specific&#46; Some authors have reported cases and have defended the role of <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT in differentiating malignant and benign PEComas&#44; with cases of malignant PEComas showing intense FDG uptake&#44; and wide SUVmax ranges ranging from 3&#46;2 to 72&#46; However&#44; most benign PEComas exhibited low or negative FDG uptake with a SUVmax less than 2&#46;<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">In the study of our third case&#44; we observed a retroperitoneal lesion with low uptake&#44; which may indicate a benign PEComa&#46; However&#44; the clinical behavior was similar to that of a malignant PEComa with a large retroperitoneal relapse and pulmonary lymphangiomyomatosis&#46; Nonetheless&#44; an <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT study was not performed in the initial diagnosis&#44; and therefore&#44; the initial SUVmax was not known&#46; In this case&#44; the <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT study was performed in the assessment of a relapsed retroperitoneal mass&#44; and there are no reports regarding the value of <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT in cases of recurrence&#46;</p><p id="par0125" class="elsevierStylePara elsevierViewall">Taking into account the lack of literature regarding the diagnosis and treatment of PEComas&#44; further studies are needed in order to develop diagnostic and therapeutic protocols in these patients&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Author contributions</span><p id="par0130" class="elsevierStylePara elsevierViewall">IN&#44; PF and AT selected the patients and wrote the article&#46; AS and JO analyzed the histopathology of the tumors&#46; All the authors approved the final article&#46;</p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0105">Conflict of interest</span><p id="par0135" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest&#46;</p></span></span>"
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              "titulo" => "Case 1&#58; PEComa of the uterus"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Antecedents</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Perivascular epitheliod cell tumor &#40;PEComa&#41; is a rare mesenchymal tumor&#46; They are rare in the field of gynecology&#44; which makes them difficult to consider as a possible diagnostic&#46; We aim to contribute with our experience to ease clinical practice to others gynecologists&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Patients and methods</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We contribute to literature with three gynecological cases&#59; uterine&#44; vaginal and retroperitoneal PEComas&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Results</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">The uterine and vaginal PEComa&#44; have required surgical treatment&#44; and are free of disease at 9 and 5 months respectively&#46; The retroperitoneal PEComa has recurred at 72 months of follow-up in form of retroperitoneal mass and pulmonary lymphangioleomyomatosis&#44; continues treatment with sirolimus with good tolerance and partial response&#46;</p></span> <span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Discussion</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Given the scarcity of cases&#44; the literature consists of case reports and mini-reviews&#46; Some authors have categorized the PEComas based on prognostic factors&#44; but there is no agreement regarding the follow-up and treatment&#46; <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;CT can help characterize these lesions&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The surgery is the standard&#46; In recurrent or malignant cases&#44; there is a lack of evidence regarding chemotherapy and radiotherapy&#46; New therapies with inhibitory m-TOR open a hopeful therapeutic window&#46;</p></span>"
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            "identificador" => "abst0020"
            "titulo" => "Discussion"
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      "es" => array:3 [
        "titulo" => "Resumen"
        "resumen" => "<span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Antecedentes</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">El tumor de c&#233;lula epitelioide perivascular &#40;PEComa&#41; es un tumor mesenquimal raro&#46; Son entidades ginecol&#243;gicas extra&#241;as&#44; lo que dificulta su consideraci&#243;n diagn&#243;stica&#46; Contribuimos con nuestra experiencia para facilitar la pr&#225;ctica cl&#237;nica a otros ginec&#243;logos&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Pacientes y m&#233;todos</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Aportamos 3 casos ginecol&#243;gicos a la literatura&#58; PEComas uterino&#44; vaginal y retroperitoneal&#46;</p></span> <span id="abst0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Resultados</span><p id="spar0040" class="elsevierStyleSimplePara elsevierViewall">Los PEComas uterino y vaginal requirieron tratamiento quir&#250;rgico y est&#225;n libres de enfermedad a los 9 y 5 meses&#44; respectivamente&#46; El PEComa retroperitoneal recidiv&#243; a los 72 meses de seguimiento en forma de masa retroperitoneal y linfangioleiomiomatosis pulmonar&#44; continuando tratamiento con sirolimus&#44; con buena tolerancia y respuesta parcial&#46;</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discusi&#243;n</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Dada la escasez de casos&#44; la literatura consiste en casos cl&#237;nicos y mini revisiones&#46; Algunos autores han categorizado los PEComas en base a factores pron&#243;sticos&#44; pero no hay acuerdo respecto al seguimiento y tratamiento&#46; La <span class="elsevierStyleSup">18</span>F-FDG-PET&#47;TC puede ayudar a caracterizar estas lesiones&#46; La cirug&#237;a es el tratamiento est&#225;ndar&#46; En casos de recurrencia o malignidad&#44; hay falta de evidencia respecto a la quimioterapia y radioterapia&#46; Nuevas terapias con inhibidores mTOR abren una ventana terap&#233;utica esperanzadora&#46;</p></span>"
        "secciones" => array:4 [
          0 => array:2 [
            "identificador" => "abst0025"
            "titulo" => "Antecedentes"
          ]
          1 => array:2 [
            "identificador" => "abst0030"
            "titulo" => "Pacientes y m&#233;todos"
          ]
          2 => array:2 [
            "identificador" => "abst0035"
            "titulo" => "Resultados"
          ]
          3 => array:2 [
            "identificador" => "abst0040"
            "titulo" => "Discusi&#243;n"
          ]
        ]
      ]
    ]
    "multimedia" => array:4 [
      0 => array:7 [
        "identificador" => "fig0005"
        "etiqueta" => "Fig&#46; 1"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
          0 => array:4 [
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        "descripcion" => array:1 [
          "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Gynecological PEComas&#46; &#40;a&#41; Uterine&#46; MRI&#58; Solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass &#40;blue arrow&#41; with necrotic areas &#40;white star&#41;&#46; &#40;b&#41; Uterine&#46; PET&#58; Hypermetabolic uterine mass &#40;black arterisk&#41; &#40;SUVmax 22&#41;&#46; &#40;c&#41; Vaginal&#46; MRI&#58; Isointense vaginal lesion in T1&#44; with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm &#40;white arterisk&#41;&#46; &#40;d&#41; Retroperitoneal&#46; MRI&#58; Cystic multiloculated mass with a solid area which extends through the retroperitoneum &#40;left para-aortic side&#41; from the renal area to the left inguinal region &#40;white arrow&#41;&#46; &#40;e&#41; Retroperitoneal&#46; PET&#58; Retroperitoneal lesion with low metabolic activity &#40;white arrow&#41; &#40;SUVmax 1&#46;9&#41;&#46; &#40;f&#41; Pulmonar lymphangioleomyomatosis CT&#58; Multiple fine wall cystic images in lung parenchyma of different sizes greater than 14<span class="elsevierStyleHsp" style=""></span>mm &#40;small white arrows&#41;&#46;</p>"
        ]
      ]
      1 => array:7 [
        "identificador" => "fig0010"
        "etiqueta" => "Fig&#46; 2"
        "tipo" => "MULTIMEDIAFIGURA"
        "mostrarFloat" => true
        "mostrarDisplay" => false
        "figura" => array:1 [
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        "descripcion" => array:1 [
          "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Pathology and immunohistochemistry analysis of PEComas&#46;</p>"
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      2 => array:8 [
        "identificador" => "tbl0005"
        "etiqueta" => "Table 1"
        "tipo" => "MULTIMEDIATABLA"
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        "detalles" => array:1 [
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            "identificador" => "at1"
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        "tabla" => array:3 [
          "leyenda" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">TSC&#44; tuberous sclerosis complex&#59; ANED&#44; alive with no evidence of disease&#59; AWD&#44; alive with disease&#59; DFS&#44; disease free survival&#46;</p>"
          "tablatextoimagen" => array:1 [
            0 => array:2 [
              "tabla" => array:1 [
                0 => """
                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
                  \t\t\t\t" class=""><thead title="thead"><tr title="table-row"><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Uterine PEComa&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Vaginal PEComa&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Retroperitoneal PEComa&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Age &#40;years&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">68&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">72&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">37&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Initial presentation&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Abnormal uterine bleeding&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Vaginal discomfort&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Endometriosis follow-up&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">TSC&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Tumor size &#40;mm&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">158&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">33&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">30&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Histologic findings&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Epithelioid proliferation of moderate to broad granular cytoplasm with clearing&#46;Rounded nuclei to slightly oval with mild pleomorphismExtensive vascular and capillary networkCells spread forming asymmetrically separate strings by fibrous tractsNecrosis and vascular invasionLess than 1 mitosis&#47;10 CGA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Epithelioid cells with clear cytoplasmRounded nuclei without pleomorphismExtensive vascular and capillary networkCells with a diffuse arrangement separate strings by fibrous tractsAbsence of necrosis or vascular invasionLess than 1 mitosis&#47;10 CGA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Epithelioid perivascular cells with abundant eosinophilic cytoplasmNucleus without pleomorphismCells arranged in small fascicles around of spaces delimited by endotheliumAbsence of necrosis or vascular invasion&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">IHC profile&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Positivity&#58; HMB45 and CD10Negativity&#58; CK7&#44; CK20&#44; CK AE1-AE3&#44; S100&#44; Melan-A&#44; calretinin&#44; inhibin A&#44; CD99&#44; CD68 and smooth muscle actinKi 67&#58; 15&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Positivity&#58; HMB-45&#44; desmin and smooth muscle actinNegativity&#58; CK7&#44; CK20&#44; S-100 and Melan-AKi 67&#58; 5&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Positivity&#58; HMB-45 and smooth muscle actin&#46; Estrogen receptors 30&#37; and progesterone receptors 60&#37;Negativity&#58; Melan-A&#44; S-100Ki-67&#58; 1&#37;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Surgical treatment&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Hysterectomy&#44; double adnexectomy&#44; omentectomy and pelvic lymphadenectomy<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Vagina mass resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Retroperitoneal cyst resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Relapse&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Lymphangioleomyomatosis and retroperitoneal relapse&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Adjuvant treatment&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">No&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">mTOR inhibitor &#8211; sirolimus &#40;ongoing&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Follow-up&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">ANED at 9 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">ANED at 5 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">AWD 93 monthsDFS 72 months&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
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        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Clinicopathologic characteristics of the reported PEComa cases&#46;</p>"
        ]
      ]
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        "etiqueta" => "Table 2"
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                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Cases&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Complete response&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Partial response&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th><th class="td" title="\n
                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t" scope="col" style="border-bottom: 2px solid black">Progression&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t\t\t</th></tr></thead><tbody title="tbody"><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Starbuck et al&#46; &#40;2016&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Flechter et al&#46; &#40;2016&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Bunch and Sunde &#40;2014&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Benson et al&#46; &#40;2014&#41;<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">a</span></a>&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">8&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">6&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Bergamo et al&#46; &#40;2014&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Gennatas et al&#46; &#40;2012&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Italiano et al&#46; &#40;2010&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">2&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Wagner et al&#46; &#40;2010&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">3&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td-with-role" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t ; entry_with_role_rowhead " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Nicolas et al&#46; &#40;2018&#41;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="char" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">1&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NA&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr></tbody></table>
                  """
              ]
              "imagenFichero" => array:1 [
                0 => "xTab2285843.png"
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          "notaPie" => array:1 [
            0 => array:3 [
              "identificador" => "tblfn0010"
              "etiqueta" => "a"
              "nota" => "<p class="elsevierStyleNotepara" id="npar0010">2 cases have been ruled out for not following the treatment&#46;</p>"
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        ]
        "descripcion" => array:1 [
          "en" => "<p id="spar0070" class="elsevierStyleSimplePara elsevierViewall">Reported cases of PEComas treated with m-TOR inhibitors and evolution&#46;</p>"
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    ]
    "bibliografia" => array:2 [
      "titulo" => "References"
      "seccion" => array:1 [
        0 => array:2 [
          "identificador" => "bibs0015"
          "bibliografiaReferencia" => array:10 [
            0 => array:3 [
              "identificador" => "bib0055"
              "etiqueta" => "1"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin&#58; a clinicopathologic study of 26 cases and review of the literature"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:6 [
                            0 => "A&#46;L&#46; Folpe"
                            1 => "T&#46; Mentzel"
                            2 => "H&#46;A&#46; Lehr"
                            3 => "C&#46; Fisher"
                            4 => "B&#46;L&#46; Balzer"
                            5 => "S&#46;W&#46; Weiss"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/01.pas.0000173232.22117.37"
                      "Revista" => array:6 [
                        "tituloSerie" => "Am J Surg Pathol"
                        "fecha" => "2005"
                        "volumen" => "29"
                        "paginaInicial" => "1558"
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                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16327428"
                            "web" => "Medline"
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                        ]
                      ]
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                ]
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            ]
            1 => array:3 [
              "identificador" => "bib0060"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Treatment of advanced malignant uterine perivascular epithelioid cell tumor with mTOR inhibitors&#58; single-institution experience and review of the literature"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "K&#46;D&#46; Starbuck"
                            1 => "R&#46;D&#46; Drake"
                            2 => "G&#46;T&#46; Budd"
                            3 => "P&#46;G&#46; Rose"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.21873/anticanres.11208"
                      "Revista" => array:6 [
                        "tituloSerie" => "Anticancer Res"
                        "fecha" => "2016"
                        "volumen" => "36"
                        "paginaInicial" => "6161"
                        "paginaFinal" => "6164"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/27793946"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
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                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0065"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Clinical activity of mTOR inhibition with Sirolimus in malignant perivascular epithelioid cell tumors&#58; targeting the pathogenic activation of mTORC1 in tumors"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46;J&#46; Wagner"
                            1 => "I&#46; Malinowska-Kolodzie"
                            2 => "J&#46;A&#46; Morgan"
                            3 => "W&#46; Qin"
                            4 => "C&#46;D&#46; Fletcher"
                            5 => "N&#46; Vena"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1200/JCO.2009.25.2981"
                      "Revista" => array:6 [
                        "tituloSerie" => "J Clin Oncol"
                        "fecha" => "2010"
                        "volumen" => "28"
                        "paginaInicial" => "835"
                        "paginaFinal" => "840"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20048174"
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              "identificador" => "bib0070"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "The role of 18F-FDG PET&#47;CT imaging in patient with malignant PEComa treated with mTOR inhibitor"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "L&#46; Sun"
                            1 => "X&#46; Sun"
                            2 => "Y&#46; Li"
                            3 => "L&#46; Xing"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.2147/OTT.S85444"
                      "Revista" => array:6 [
                        "tituloSerie" => "Onco Targets Ther"
                        "fecha" => "2015"
                        "volumen" => "8"
                        "paginaInicial" => "1967"
                        "paginaFinal" => "1970"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/26257526"
                            "web" => "Medline"
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            4 => array:3 [
              "identificador" => "bib0075"
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor&#58; a case report and literature review"
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                        0 => array:2 [
                          "etal" => false
                          "autores" => array:5 [
                            0 => "E&#46; Flechter"
                            1 => "Y&#46; Zohar"
                            2 => "L&#46; Guralnik"
                            3 => "M&#46; Passhak"
                            4 => "G&#46;B&#46; Sela"
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                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
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ISSN: 00257753
Original language: English
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