Scientific letters
Perivascular epithelioid cell neoplasm (PEComa) harboring TFE3 gene rearrangements in a patient with Lynch syndrome
Pecoma con reordenamiento del gen TFE3 en paciente con síndrome de Lynch
Ana B. Larquéa,
, Gerard Frigolaa, Natalia Castrejóna, Sherley Díaz-Mercedesa, Eva Musulén Paletb, Carolina Martínez Ciarpaglinic, Stefania Landolfid, Antonio M. Lacye,f, Francesc Balaguerf,g, Miriam Cuatrecasasa,f
Corresponding author
a Hospital Clinic de Barcelona, Pathology Department, Barcelona, Spain
b Hospital Universitari General de Catalunya, Pathology Department, Sant Cugat del Vallés, Spain
c Hospital Clínico Universitario de Valencia, Pathology Department, Valencia, Spain
d Hospital Vall d’Hebrón, Pathology Department, Barcelona, Spain
e Hospital Clinic de Barcelona, Department of Gastrointestinal Surgery, Barcelona, Spain
f Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
g Hospital Clinic de Barcelona, Gastroenterology Department, Barcelona, Spain
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"apellidos" => "Lacy" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">e</span>" "identificador" => "aff0025" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">f</span>" "identificador" => "aff0030" ] ] ] 8 => array:3 [ "nombre" => "Francesc" "apellidos" => "Balaguer" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">f</span>" "identificador" => "aff0030" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">g</span>" "identificador" => "aff0035" ] ] ] 9 => array:3 [ "nombre" => "Miriam" "apellidos" => "Cuatrecasas" "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">f</span>" "identificador" => "aff0030" ] ] ] ] "afiliaciones" => array:7 [ 0 => array:3 [ "entidad" => "Hospital Clinic de Barcelona, Pathology Department, Barcelona, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Hospital Universitari General de Catalunya, Pathology Department, Sant Cugat del Vallés, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Hospital Clínico Universitario de Valencia, Pathology Department, Valencia, Spain" "etiqueta" => "c" "identificador" => "aff0015" ] 3 => array:3 [ "entidad" => "Hospital Vall d’Hebrón, Pathology Department, Barcelona, Spain" "etiqueta" => "d" "identificador" => "aff0020" ] 4 => array:3 [ "entidad" => "Hospital Clinic de Barcelona, Department of Gastrointestinal Surgery, Barcelona, Spain" "etiqueta" => "e" "identificador" => "aff0025" ] 5 => array:3 [ "entidad" => "Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain" "etiqueta" => "f" "identificador" => "aff0030" ] 6 => array:3 [ "entidad" => "Hospital Clinic de Barcelona, Gastroenterology Department, Barcelona, Spain" "etiqueta" => "g" "identificador" => "aff0035" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Pecoma con reordenamiento del gen TFE3 en paciente con síndrome de Lynch" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2569 "Ancho" => 1967 "Tamanyo" => 1061067 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Axial MRI showing the pelvic nodule (arrow). (B) Gross image of the tumor after formalin fixation showing a white-gray mass. (C) TFE3 rearrangement-associated PEComa displaying nested or sheet-like architecture. (D) At a higher power, the tumor was composed of epithelioid cells with abundant clear to granular cytoplasm separated by delicate fibrovascular septa. (E) Immunohistochemical stain for TFE3 shows diffuse and strong nuclear staining by tumor cells. (F) HMB-45 was also expressed strongly. (G) The neoplasm shows scattered positive cells for MELAN A. (H) TFE3 FISH image indicating TFE3 gene rearrangement with widely separated red and green signals (TFE3 Dual Color Break Apart Probe).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Lynch syndrome (LS) is caused by germline pathogenic variants in the mismatch repair (<span class="elsevierStyleItalic">MMR</span>) genes.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> LS carriers have a high risk of developing various types of tumors, especially colorectal and endometrial cancers.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Although sarcomas are not included in the spectrum of LS tumors, several types of sarcomas have been occasionally reported to occur in individuals with LS.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a> PEComas are rare neoplasms known to be associated with the tuberous sclerosis complex.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> A high number of PEComas have shown inactivation of the <span class="elsevierStyleItalic">TSC1/2</span> genes. Recently, a subset of PEComas harboring <span class="elsevierStyleItalic">TFE3</span> gene fusions has been described.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> These tumors present distinctive features from the convectional PEComas and lack co-existing <span class="elsevierStyleItalic">TSC</span> mutations.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report a case of <span class="elsevierStyleItalic">TFE3</span> rearrangement-associated PEComa in a 38-year-old LS male with a previous rectal mucinous adenocarcinoma at 32 years of age, treated with neoadjuvant radio-chemotherapy followed by surgical resection. The patient fulfilled the modified Bethesda criteria for LS and genetic testing revealed an exon 8 <span class="elsevierStyleItalic">MSH2</span> duplication, which confirmed the diagnosis of LS. He presented two years later with local recurrence, treated with surgery plus adjuvant chemotherapy. Four years later, a pelvic nodule was detected at follow-up, revealing by MRI a 2.5<span class="elsevierStyleHsp" style=""></span>cm round, solid nodule on the right iliac chain suggestive of lymph node recurrence, which was surgically resected (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Grossly, the tumor was a firm, well-defined, 2.5<span class="elsevierStyleHsp" style=""></span>cm solid mass (Fig 1). Histologically, the neoplasm was composed of nests of neoplastic epithelioid cells with abundant clear cytoplasm and round or oval nuclei with small nucleoli, separated by delicate fibrovascular septa, with presence of hemosiderin and melanin pigments. Occasional nuclear pleomorphism was observed, without necrosis or mitotic activity (Fig 1). Immunohistochemistry (IHC) revealed widely positivity of the tumor cells for HMB-45 and TFE3, with scattered positive cells for MELAN A, and actin, while CK AE1/AE3, S-100, Desmin, and PAX-8 were negative. IHC testing for MMR proteins (MLH1, MSH2, MSH6 and PMS2) showed intact nuclear expression (Fig 1). MMR proficiency was also tested with a PCR assay (3130 Genetic Analyzer) and the tumor resulted microsatellite stable (MSS). Fluorescence in situ hybridization confirmed the presence of a <span class="elsevierStyleItalic">TFE3</span> gene rearrangement. The patient was alive 68 months after surgery, with no evidence of recurrence of either tumor.</p><p id="par0020" class="elsevierStylePara elsevierViewall">This case represents, to the best of our knowledge, the first report of a PEComa occurring in a patient with LS. Conversely, there is one case published of a sporadic malignant PEComa that showed MSH2 and MSH6 loss by IHC and a <span class="elsevierStyleItalic">MSH2</span> copy deletion consistent with somatic origin, not diagnostic of LS.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> We did not consider our case to be a LS-related malignancy based on the intact nuclear staining of the tumor cells for all four MMR proteins, and the presence of MSS. In addition, the low incidence of sarcomas, and particularly of PEComas in LS-patients, makes an association with LS very unlikely.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Many different malignancies including sarcomas have been described in LS families. There is cumulative evidence that patients with LS might also develop non-Lynch syndrome tumors, either sporadically or because of MMR defect. According to the literature,<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2,3</span></a> the most frequent type of soft tissue sarcomas in LS-patients are leiomyosarcomas, liposarcomas and malignant fibrous histiocytomas. Most tumors showed MMR deficiency by IHC. This data suggest that sarcomas may occasionally be present as part of the LS-related tumor spectrum.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Our case demonstrates a second neoplasm in a lesion suggestive of a rectal carcinoma lymph node recurrence in a patient with LS, what show the importance of performing a complete histopathological diagnosis including MMR testing in any kind of tumor, either synchronic or metachronic to all LS-patients, especially if they are clinically atypical, given its therapeutic and prognostic implications.</p><p id="par0035" class="elsevierStylePara elsevierViewall">PEComas which harbor <span class="elsevierStyleItalic">TFE3</span> gene fusion, present distinctive features from conventional PEComas, including younger age at diagnosis, lack of association with tuberous sclerosis, predominant alveolar architecture, and epithelioid cytology, minimal or no IHC positivity for muscle markers, and strong TFE3 positivity.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Our case fulfills almost all these criteria except for being a male, since PEComas usually occur in females. Due to the epithelioid morphology, we first ruled out a carcinoma, including a potential metastatic renal cell carcinoma. However, various cytokeratins and PAX-8 were negative. The TFE3 IHC in our case was performed using an automated platform. As is known, TFE3 protein is ubiquitously present in low levels in normal cells. As a result, the antibody often shows low specificity, which may be improved performing manual IHC with overnight incubation.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> For this reason, the presence of <span class="elsevierStyleItalic">TFE3</span> translocations should be confirmed with a FISH assay.</p><p id="par0040" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">TFE3</span> rearrangement-associated PEComas share the same clinicopathological spectrum as other <span class="elsevierStyleItalic">TFE3</span> rearrangement-associated tumors such as melanotic <span class="elsevierStyleItalic">Xp11</span> translocation renal cancer and melanotic <span class="elsevierStyleItalic">Xp11</span> neoplasm, suggesting that they all belong to the same entity.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> Several authors have proposed the term of “melanotic <span class="elsevierStyleItalic">Xp11</span> neoplasm” to designate this spectrum of neoplasms.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Conventional PEComas are most often associated with a benign clinical course, but a small subset is malignant.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Information regarding the clinical behavior of PEComas with <span class="elsevierStyleItalic">TFE3</span> gene fusion remains incomplete due to their low incidence. In the largest series reported of “melanotic <span class="elsevierStyleItalic">Xp11</span> neoplasm”, the 5-year overall survival and 5-year disease free survival were 47.6% and 35.7% respectively, which points out at a clinically aggressive neoplasm.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> In our patient, the tumor did not show atypical morphological features and the clinical course of the patient has been uneventful after 68 months follow-up.</p><p id="par0050" class="elsevierStylePara elsevierViewall">In conclusion, we present the first case of a PEComa with a <span class="elsevierStyleItalic">TFE3</span> rearrangement in a patient with LS. This tumor adds to the list of sporadic non-Lynch syndrome-related tumors, and to the data of <span class="elsevierStyleItalic">TFE3</span> rearrangement-associated PEComas.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflict of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflict of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 2569 "Ancho" => 1967 "Tamanyo" => 1061067 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) Axial MRI showing the pelvic nodule (arrow). (B) Gross image of the tumor after formalin fixation showing a white-gray mass. (C) TFE3 rearrangement-associated PEComa displaying nested or sheet-like architecture. (D) At a higher power, the tumor was composed of epithelioid cells with abundant clear to granular cytoplasm separated by delicate fibrovascular septa. (E) Immunohistochemical stain for TFE3 shows diffuse and strong nuclear staining by tumor cells. (F) HMB-45 was also expressed strongly. (G) The neoplasm shows scattered positive cells for MELAN A. (H) TFE3 FISH image indicating TFE3 gene rearrangement with widely separated red and green signals (TFE3 Dual Color Break Apart Probe).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Genetics, natural history, tumor spectrum, and pathology of hereditary nonpolyposis colorectal cancer: an updated review" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "H.T. Lynch" 1 => "T.C. Smyrk" 2 => "P. Watson" 3 => "S.J. Lanspa" 4 => "J.F. Lynch" 5 => "P.M. 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| Year/Month | Html | Total | |
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