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(a) Uterine. MRI: Solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass (blue arrow) with necrotic areas (white star). (b) Uterine. PET: Hypermetabolic uterine mass (black arterisk) (SUVmax 22). (c) Vaginal. MRI: Isointense vaginal lesion in T1, with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm (white arterisk). (d) Retroperitoneal. MRI: Cystic multiloculated mass with a solid area which extends through the retroperitoneum (left para-aortic side) from the renal area to the left inguinal region (white arrow). (e) Retroperitoneal. PET: Retroperitoneal lesion with low metabolic activity (white arrow) (SUVmax 1.9). (f) Pulmonar lymphangioleomyomatosis CT: Multiple fine wall cystic images in lung parenchyma of different sizes greater than 14<span class="elsevierStyleHsp" style=""></span>mm (small white arrows).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Inmaculada Nicolás, Pere Fusté, Adela Saco, Jaume Ordi, Aureli Torné" "autores" => array:5 [ 0 => array:2 [ "nombre" => "Inmaculada" "apellidos" => "Nicolás" ] 1 => array:2 [ "nombre" => "Pere" "apellidos" => "Fusté" ] 2 => array:2 [ "nombre" => "Adela" "apellidos" => "Saco" ] 3 => array:2 [ "nombre" => "Jaume" "apellidos" => "Ordi" ] 4 => array:2 [ "nombre" => "Aureli" "apellidos" => "Torné" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S0025775319300557" "doi" => "10.1016/j.medcli.2019.01.009" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775319300557?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S238702061930227X?idApp=UINPBA00004N" "url" => "/23870206/0000015300000002/v2_202004301535/S238702061930227X/v2_202004301535/en/main.assets" ] ] "itemSiguiente" => array:19 [ "pii" => "S0025775318303063" "issn" => "00257753" "doi" => "10.1016/j.medcli.2018.05.007" "estado" => "S300" "fechaPublicacion" => "2019-07-19" "aid" => "4534" "copyright" => "Elsevier España, S.L.U." "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "crp" "cita" => "Med Clin. 2019;153:88-9" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:2 [ "total" => 6 "HTML" => 6 ] "es" => array:11 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Carta científica</span>" "titulo" => "Herpes zóster multiplex: descripción de un caso" "tienePdf" => "es" "tieneTextoCompleto" => "es" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "88" "paginaFinal" => "89" ] ] "titulosAlternativos" => array:1 [ "en" => array:1 [ "titulo" => "Herpes zoster multiplex: Report on one case" ] ] "contieneTextoCompleto" => array:1 [ "es" => true ] "contienePdf" => array:1 [ "es" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figura 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 515 "Ancho" => 989 "Tamanyo" => 67989 ] ] "descripcion" => array:1 [ "es" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">a) Lesiones vesiculosas con distribución herpetiforme y fondo eritematoso, localizadas en dorso del pie, que se extienden hacia el dorso del primer dedo del pie derecho; b) Placas eritematosas con algunas lesiones vesiculosas agrupadas, frágiles, que en algunos casos han roto dejando pequeñas erosiones localizadas a nivel costal derecho.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Ana Varela-Veiga, Benigno Monteagudo, Óscar Suárez-Amor" "autores" => array:3 [ 0 => array:2 [ "nombre" => "Ana" "apellidos" => "Varela-Veiga" ] 1 => array:2 [ "nombre" => "Benigno" "apellidos" => "Monteagudo" ] 2 => array:2 [ "nombre" => "Óscar" "apellidos" => "Suárez-Amor" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2387020619302335" "doi" => "10.1016/j.medcle.2018.05.059" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => false "ES2" => false "LATM" => false ] "gratuito" => false "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020619302335?idApp=UINPBA00004N" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0025775318303063?idApp=UINPBA00004N" "url" => "/00257753/0000015300000002/v2_202005080620/S0025775318303063/v2_202005080620/es/main.assets" ] "itemAnterior" => array:19 [ "pii" => "S0025775318307310" "issn" => "00257753" "doi" => "10.1016/j.medcli.2018.10.028" "estado" => "S300" "fechaPublicacion" => "2019-07-19" "aid" => "4704" "copyright" => "Elsevier España, S.L.U." 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array:20 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Clinical report</span>" "titulo" => "Perivascular epitheliod cell tumors: Study of three gynecological cases" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "83" "paginaFinal" => "87" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Inmaculada Nicolás, Pere Fusté, Adela Saco, Jaume Ordi, Aureli Torné" "autores" => array:5 [ 0 => array:4 [ "nombre" => "Inmaculada" "apellidos" => "Nicolás" "email" => array:1 [ 0 => "innipe@hotmail.com" ] "referencia" => array:3 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] 2 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "Pere" "apellidos" => "Fusté" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "Adela" "apellidos" => "Saco" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 3 => array:3 [ "nombre" => "Jaume" "apellidos" => "Ordi" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 4 => array:3 [ "nombre" => "Aureli" "apellidos" => "Torné" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Institute Clinic of Gynecology, Obstetrics, and Neonatology, Hospital Clínic – Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Department of Pathology, Hospital Clínic, University of Barcelona, Barcelona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Institut de Salut Global de Barcelona (ISGlobal), Barcelona, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumores de células epitelioides perivasculares: estudio de 3 casos ginecológicos" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1558 "Ancho" => 2507 "Tamanyo" => 454989 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Gynecological PEComas. (a) Uterine. MRI: Solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass (blue arrow) with necrotic areas (white star). (b) Uterine. PET: Hypermetabolic uterine mass (black arterisk) (SUVmax 22). (c) Vaginal. MRI: Isointense vaginal lesion in T1, with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm (white arterisk). (d) Retroperitoneal. MRI: Cystic multiloculated mass with a solid area which extends through the retroperitoneum (left para-aortic side) from the renal area to the left inguinal region (white arrow). (e) Retroperitoneal. PET: Retroperitoneal lesion with low metabolic activity (white arrow) (SUVmax 1.9). (f) Pulmonar lymphangioleomyomatosis CT: Multiple fine wall cystic images in lung parenchyma of different sizes greater than 14<span class="elsevierStyleHsp" style=""></span>mm (small white arrows).</p>" ] ] ] "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 (PEComa) are rare mesenchymal tumors composed of histologically distinctive perivascular epithelioid cells (PEC).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> They are rare in the field of gynecology, heterogeneous clinically, which makes them difficult for gynecologists to consider as a possible diagnostic option.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report three cases (uterine and vaginal PEComa in two menopausal patients and a retroperitoneal PEComa in a premenopausal patient) that led to differential diagnosis with other more common gynecological tumors.</p><p id="par0015" class="elsevierStylePara elsevierViewall">In 2002 PEComas were classified by the WHO as tumors involving soft tissues (stomach, intestines, lungs, female reproductive organs and genitourinary organs). They are more frequent in women (ratio over 7:1) and are occasionally associated with the tuberous sclerosis complex (TSC).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> The histological characteristics of these tumors are: co-expression of melanocytic and muscle markers (smooth muscle actin, HMB45, Melan-A, S-100), epithelioid to spindle cellular shapes with ample clear to eosinophilic cytoplasm and arrangement around blood vessels.<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% of the cases, and the uterus is the most commonly reported site.<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, 3 women with gynecological PEComas were diagnosed at the Gynecology Unit (Hospital Clinic Barcelona). They had given their informed consent to store and dispose of the biological material obtained during their care process, and to use the clinical information for research purposes. We retrospectively reviewed their clinical records, the follow-up and the last status.</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: PEComa of the uterus</span><p id="par0030" class="elsevierStylePara elsevierViewall">A 68-year-old woman, 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. Clinical examination highlighted a pelvic mass of approximately 15<span class="elsevierStyleHsp" style=""></span>cm. 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 (Doppler score 3/4). Abdominopelvic magnetic resonance imaging [MRI] showed a solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass with necrotic areas inside with significantly restricted diffusion (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>a). Positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-<span class="elsevierStyleSmallCaps">d</span>-glucose integrated with computed tomography (<span class="elsevierStyleSup">18</span>F-FDG-PET/CT) showed a large hypermetabolic uterine mass, with no signs of loco-regional or distant extension (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>b). Upon suspicion of a possible malignant uterine-ovarian neoplasm, tumor markers were requested. CA-125, CA19-9, CEA, and HE4 levels were within normal limits. The endometrial biopsy was negative for malignancy. Hysterectomy and double salpingo-oophorectomy were performed by laparotomy. The provisional perioperative diagnosis was PEComa, without being able to rule out other malignancies. Omentectomy and pelvic nodal debulking due to intraoperative suspicion of lymphadenopathies was added.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The definitive histological study showed a large multinodular mass, with areas of yellowish coloration, measuring 16<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>10<span class="elsevierStyleHsp" style=""></span>cm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>5<span class="elsevierStyleHsp" style=""></span>cm compatible with malignant uterine PEComa (size >5<span class="elsevierStyleHsp" style=""></span>cm, infiltrative growth pattern, necrosis and vascular invasion) (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The patient did not receive adjuvant treatment and remained disease free 9 months after surgery.</p><elsevierMultimedia ident="tbl0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0085">Case 2. PEComa of the vagina</span><p id="par0040" class="elsevierStylePara elsevierViewall">A 72-year-old woman, was referred for discomfort, vaginal pressure and difficulty in emptying the bladder. Clinical examination revealed a brownish vaginal tumor of 3<span class="elsevierStyleHsp" style=""></span>cm, with a solid multinodular appearance, pedunculated and inserted in the middle third of the lateral wall of the vagina. MRI showed a well-defined tumor, being iso-hyperintense in T1 and T2, without restricted diffusion, with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm and without extramural extension (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>c). Cyfra 21.1, CEA and SCC levels were within normal limits. A biopsy was performed, obtaining a histological diagnosis of PEComa. Wide local vaginal excision with free margins was performed.</p><p id="par0045" class="elsevierStylePara elsevierViewall">The clinical and histological criteria were compatible with a benign PEComa (size 3<span class="elsevierStyleHsp" style=""></span>cm, no infiltrative growth pattern, no necrosis or vascular invasion) (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The patient was disease free at 5 months postoperatively.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0090">Case 3. PEComa of the retroperitoneum</span><p id="par0050" class="elsevierStylePara elsevierViewall">A 42-year-old, nulliparous woman presented for a second opinion because of a large retroperitoneal mass suspected of recurrence of PEComa.</p><p id="par0055" class="elsevierStylePara elsevierViewall">The patient referred a history of: bilateral ovarian cystectomy for endometriosis at 20 years of age, myomectomy and right cystectomy for endometriosis at age 26, and myomectomy and uterine adenomatoid tumor excision at 36 years of age.</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. Resection by laparoscopy of the retroperitoneal lesion was performed with a diagnosis of PEComa. The paraffin block of the tissue from the previous biopsy was reanalyzed in our hospital, and the diagnosis of PEComa was confirmed (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><p id="par0065" class="elsevierStylePara elsevierViewall">Over a 6-year follow-up the patient showed no evidence of recurrence. Thereafter, a control MRI showed a cystic multiloculated mass with a solid area extending through the retroperitoneum (left para-aortic side) from the renal area to the inguinal region, suggestive of relapse of the basal disease, for which the patient requested a second opinion (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>d).</p><p id="par0070" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleSup">18</span>F-FDG PET/CT showed a multicystic retroperitoneal mass, with a metabolism similar to that of the adjacent tissues (SUVmax 1.9), suggesting recurrence of PEComa with low metabolic activity. No pathological deposits were identified in the remaining body sites studied (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>e). 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, being suggestive of lymphangioleomyomatosis (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>f).</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. 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, with good tolerance. After 8 months of follow-up, the retroperitoneal lesions disappeared, and the lung lesions remained stable. The patient is undergoing surveillance and remains stable at 21 months of follow-up post relapse.</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, PEComas are rare tumors which are difficult to diagnose. These tumors may present with a benign and sometimes malignant behavior, leading to suspicion of malignant oncological entities.</p><p id="par0085" class="elsevierStylePara elsevierViewall">Some clinical, radiological and immunohistochemical characteristics are shared by these tumors, although there is no pathognomonic pattern for PEComas. Cases associated with tuberous sclerosis are rarely reported, and none of our patients were diagnosed with this.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Initially, PEComas were considered to be benign tumors, but with the progressive description of cases a small subset of PEComas were reported to present local recurrences or distant metastases. Therefore, Folpe et al. designed a classification based on several prognostic factors and categorized PEComas into three prognostic groups (benign, uncertain malignant potential or malignant tumors) according to the following six risk factors: (1) tumor size ≥5<span class="elsevierStyleHsp" style=""></span>cm, (2) infiltrative growth pattern, (3) high grade nuclear cellularity, (4) mitotic rate >1/50 high power fields, (5) necrosis or (6) vascular invasion.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> In some cases, there is no consensus regarding treatment, and it is not individualized according to prognostic factors. Surgical treatment is the standard procedure. There is no protocol of adjuvant treatment, and there is little information available regarding chemotherapy and radiotherapy in PEComa.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Regarding other adjuvant treatments, some recent results with the use of mTOR inhibitors seem to be promising in refractory or malignant cases. PEComas as well as LAM and AML show constitutive activation of the mTOR pathway (mammalian target of rapamycin complex 1) derived from genetic alterations of the TSC1 or TSC2 locus.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Cases treated with mTOR inhibitors are scarce but encouraging. There are small series of cases in the literature reporting response rates of 50–100%, with patients being treated with mTOR inhibitors after surgery. Some cases with contraindications for primary surgical treatment demonstrated a reduction of tumor size with the use of mTOR inhibitors, thereafter making surgery possible.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2–5</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Review of 25 cases in the literature, demonstrates the heterogeneity of the cases reported to date (different locations, treated with chemotherapy before treatment with mTOR inhibitors, receiving neoadjuvant treatment, without previous surgery) (<a class="elsevierStyleCrossRef" href="#tbl0010">Table 2</a>). In our evaluation, we defined complete response as radiological disappearance of the lesions, while partial response was considered as a decrease in lesion size and progression to worsening before 12 months. Therefore, response was achieved in 76.9% (20/26) of the cases, being complete in 30.8% (8/26) and partial in 46.1% (12/26), and 23.1% (6/26) of the cases showed progression.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">2,3,5–10</span></a></p><elsevierMultimedia ident="tbl0010"></elsevierMultimedia><p id="par0105" class="elsevierStylePara elsevierViewall">Here, we presented three cases of PEComa with different clinicopathological features (<a class="elsevierStyleCrossRef" href="#tbl0005">Table 1</a> and <a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The histological and immunohistochemical study was essential for the diagnosis in our patients. PEComas can co-express melanocytic and muscle immunohistochemical markers, but not all the tumors have to express the same marker.</p><p id="par0110" class="elsevierStylePara elsevierViewall">In our report only the preoperative study of the case of vaginal PEComa was conclusive, given the possibility of histological study prior to surgery, but in the remaining two cases post-surgical study was required. The perioperative diagnostic approach carried out in our center in the case of uterine PEComa is of note, taking into account the infrequent nature of this neoplasm. The complexity of these unusual gynecological tumors justifies the referral of these patients to oncological reference centers.</p><p id="par0115" class="elsevierStylePara elsevierViewall">Radiological studies and the clinical manifestations of PEComas are not specific. Some authors have reported cases and have defended the role of <span class="elsevierStyleSup">18</span>F-FDG-PET/CT in differentiating malignant and benign PEComas, with cases of malignant PEComas showing intense FDG uptake, and wide SUVmax ranges ranging from 3.2 to 72. However, most benign PEComas exhibited low or negative FDG uptake with a SUVmax less than 2.<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, we observed a retroperitoneal lesion with low uptake, which may indicate a benign PEComa. However, the clinical behavior was similar to that of a malignant PEComa with a large retroperitoneal relapse and pulmonary lymphangiomyomatosis. Nonetheless, an <span class="elsevierStyleSup">18</span>F-FDG-PET/CT study was not performed in the initial diagnosis, and therefore, the initial SUVmax was not known. In this case, the <span class="elsevierStyleSup">18</span>F-FDG-PET/CT study was performed in the assessment of a relapsed retroperitoneal mass, and there are no reports regarding the value of <span class="elsevierStyleSup">18</span>F-FDG-PET/CT in cases of recurrence.</p><p id="par0125" class="elsevierStylePara elsevierViewall">Taking into account the lack of literature regarding the diagnosis and treatment of PEComas, further studies are needed in order to develop diagnostic and therapeutic protocols in these patients.</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0100">Author contributions</span><p id="par0130" class="elsevierStylePara elsevierViewall">IN, PF and AT selected the patients and wrote the article. AS and JO analyzed the histopathology of the tumors. All the authors approved the final article.</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.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:12 [ 0 => array:3 [ "identificador" => "xres1333690" "titulo" => "Abstract" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Antecedents" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Discussion" ] ] ] 1 => array:2 [ "identificador" => "xpalclavsec1228626" "titulo" => "Keywords" ] 2 => array:3 [ "identificador" => "xres1333689" "titulo" => "Resumen" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Antecedentes" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Discusión" ] ] ] 3 => array:2 [ "identificador" => "xpalclavsec1228627" "titulo" => "Palabras clave" ] 4 => array:2 [ "identificador" => "sec0005" "titulo" => "Introduction" ] 5 => array:2 [ "identificador" => "sec0010" "titulo" => "Materials and methods" ] 6 => array:3 [ "identificador" => "sec0015" "titulo" => "Results" "secciones" => array:3 [ 0 => array:2 [ "identificador" => "sec0020" "titulo" => "Case 1: PEComa of the uterus" ] 1 => array:2 [ "identificador" => "sec0025" "titulo" => "Case 2. PEComa of the vagina" ] 2 => array:2 [ "identificador" => "sec0030" "titulo" => "Case 3. PEComa of the retroperitoneum" ] ] ] 7 => array:2 [ "identificador" => "sec0035" "titulo" => "Discussion" ] 8 => array:2 [ "identificador" => "sec0040" "titulo" => "Author contributions" ] 9 => array:2 [ "identificador" => "sec0045" "titulo" => "Conflict of interest" ] 10 => array:2 [ "identificador" => "xack460880" "titulo" => "Acknowledgement" ] 11 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-10-23" "fechaAceptado" => "2019-01-10" "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec1228626" "palabras" => array:3 [ 0 => "PEComa" 1 => "Perivascular epithelioid cell tumor" 2 => "Mtor inhibitor" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec1228627" "palabras" => array:3 [ 0 => "PEComa" 1 => "Tumor de célula epitelioide perivascular" 2 => "Inhibidor mTOR" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:3 [ "titulo" => "Abstract" "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Antecedents</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Perivascular epitheliod cell tumor (PEComa) is a rare mesenchymal tumor. They are rare in the field of gynecology, which makes them difficult to consider as a possible diagnostic. We aim to contribute with our experience to ease clinical practice to others gynecologists.</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; uterine, vaginal and retroperitoneal PEComas.</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, have required surgical treatment, and are free of disease at 9 and 5 months respectively. The retroperitoneal PEComa has recurred at 72 months of follow-up in form of retroperitoneal mass and pulmonary lymphangioleomyomatosis, continues treatment with sirolimus with good tolerance and partial response.</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, the literature consists of case reports and mini-reviews. Some authors have categorized the PEComas based on prognostic factors, but there is no agreement regarding the follow-up and treatment. <span class="elsevierStyleSup">18</span>F-FDG-PET/CT can help characterize these lesions.</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The surgery is the standard. In recurrent or malignant cases, there is a lack of evidence regarding chemotherapy and radiotherapy. New therapies with inhibitory m-TOR open a hopeful therapeutic window.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0005" "titulo" => "Antecedents" ] 1 => array:2 [ "identificador" => "abst0010" "titulo" => "Patients and methods" ] 2 => array:2 [ "identificador" => "abst0015" "titulo" => "Results" ] 3 => array:2 [ "identificador" => "abst0020" "titulo" => "Discussion" ] ] ] "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 célula epitelioide perivascular (PEComa) es un tumor mesenquimal raro. Son entidades ginecológicas extrañas, lo que dificulta su consideración diagnóstica. Contribuimos con nuestra experiencia para facilitar la práctica clínica a otros ginecólogos.</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Pacientes y métodos</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Aportamos 3 casos ginecológicos a la literatura: PEComas uterino, vaginal y retroperitoneal.</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 quirúrgico y están libres de enfermedad a los 9 y 5 meses, respectivamente. El PEComa retroperitoneal recidivó a los 72 meses de seguimiento en forma de masa retroperitoneal y linfangioleiomiomatosis pulmonar, continuando tratamiento con sirolimus, con buena tolerancia y respuesta parcial.</p></span> <span id="abst0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Discusión</span><p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Dada la escasez de casos, la literatura consiste en casos clínicos y mini revisiones. Algunos autores han categorizado los PEComas en base a factores pronósticos, pero no hay acuerdo respecto al seguimiento y tratamiento. La <span class="elsevierStyleSup">18</span>F-FDG-PET/TC puede ayudar a caracterizar estas lesiones. La cirugía es el tratamiento estándar. En casos de recurrencia o malignidad, hay falta de evidencia respecto a la quimioterapia y radioterapia. Nuevas terapias con inhibidores mTOR abren una ventana terapéutica esperanzadora.</p></span>" "secciones" => array:4 [ 0 => array:2 [ "identificador" => "abst0025" "titulo" => "Antecedentes" ] 1 => array:2 [ "identificador" => "abst0030" "titulo" => "Pacientes y métodos" ] 2 => array:2 [ "identificador" => "abst0035" "titulo" => "Resultados" ] 3 => array:2 [ "identificador" => "abst0040" "titulo" => "Discusión" ] ] ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1558 "Ancho" => 2507 "Tamanyo" => 454989 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0050" class="elsevierStyleSimplePara elsevierViewall">Gynecological PEComas. (a) Uterine. MRI: Solid 18<span class="elsevierStyleHsp" style=""></span>cm uterine mass (blue arrow) with necrotic areas (white star). (b) Uterine. PET: Hypermetabolic uterine mass (black arterisk) (SUVmax 22). (c) Vaginal. MRI: Isointense vaginal lesion in T1, with a diameter of 24<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>21<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>33<span class="elsevierStyleHsp" style=""></span>mm (white arterisk). (d) Retroperitoneal. MRI: Cystic multiloculated mass with a solid area which extends through the retroperitoneum (left para-aortic side) from the renal area to the left inguinal region (white arrow). (e) Retroperitoneal. PET: Retroperitoneal lesion with low metabolic activity (white arrow) (SUVmax 1.9). (f) Pulmonar lymphangioleomyomatosis CT: Multiple fine wall cystic images in lung parenchyma of different sizes greater than 14<span class="elsevierStyleHsp" style=""></span>mm (small white arrows).</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1123 "Ancho" => 3163 "Tamanyo" => 889277 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0055" class="elsevierStyleSimplePara elsevierViewall">Pathology and immunohistochemistry analysis of PEComas.</p>" ] ] 2 => array:8 [ "identificador" => "tbl0005" "etiqueta" => "Table 1" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at1" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0065" class="elsevierStyleSimplePara elsevierViewall">TSC, tuberous sclerosis complex; ANED, alive with no evidence of disease; AWD, alive with disease; DFS, disease free survival.</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"> \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 \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 \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 \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 (years) \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 \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 \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 \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 \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 \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 \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 \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 \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 \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 \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 \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 (mm) \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 \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 \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 \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 \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.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/10 CGA \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/10 CGA \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 \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 \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: HMB45 and CD10Negativity: CK7, CK20, CK AE1-AE3, S100, Melan-A, calretinin, inhibin A, CD99, CD68 and smooth muscle actinKi 67: 15% \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: HMB-45, desmin and smooth muscle actinNegativity: CK7, CK20, S-100 and Melan-AKi 67: 5% \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: HMB-45 and smooth muscle actin. Estrogen receptors 30% and progesterone receptors 60%Negativity: Melan-A, S-100Ki-67: 1% \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 \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, double adnexectomy, omentectomy and pelvic lymphadenectomy<a class="elsevierStyleCrossRef" href="#tblfn0005"><span class="elsevierStyleSup">a</span></a> \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 \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 \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 \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 \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 \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 \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 \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 \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 \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 – sirolimus (ongoing) \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 \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 \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 \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 \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2285844.png" ] ] ] "notaPie" => array:1 [ 0 => array:3 [ "identificador" => "tblfn0005" "etiqueta" => "a" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Omentum and lymph nodes were negative for malignancy.</p>" ] ] ] "descripcion" => array:1 [ "en" => "<p id="spar0060" class="elsevierStyleSimplePara elsevierViewall">Clinicopathologic characteristics of the reported PEComa cases.</p>" ] ] 3 => array:8 [ "identificador" => "tbl0010" "etiqueta" => "Table 2" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at2" "detalle" => "Table " "rol" => "short" ] ] "tabla" => array:3 [ "leyenda" => "<p id="spar0075" class="elsevierStyleSimplePara elsevierViewall">NA, not available.</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"> \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 \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 \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 \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 \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. (2016) \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 \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 \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 \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 \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. (2016) \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 \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 \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 \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 \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 (2014) \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 \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 \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 \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 \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. (2014)<a class="elsevierStyleCrossRef" href="#tblfn0010"><span class="elsevierStyleSup">a</span></a> \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 \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 \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 \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 \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. (2014) \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 \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 \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 \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 \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. 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