Primitive Neuroectodermal Tumors of the Female Genital Tract: A Morphologic, Immunohistochemical and Molecular Study of 19 Cases
| Autor: | Chiang, Sarah, Snuderl, Matija, Kojiro-Sanada, Sakiko, Quer, Ariadna, Daya, Dean, Hayashi, Tohru, Bosincu, Luisanna, Ogawa, Fumihiro, Rosenberg, Andrew E., Horn, Lars-Christian, Wang, Lu, Iafrate, John, Oliva, Esther, Universitat Autònoma de Barcelona |
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| Jazyk: | angličtina |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Adult Pathology medicine.medical_specialty Adolescent Genital Neoplasms Female CD99 Biology Article Pathology and Forensic Medicine 03 medical and health sciences Young Adult 0302 clinical medicine medicine Biomarkers Tumor Humans Neuroectodermal Tumors Primitive Peripheral Child In Situ Hybridization Fluorescence Aged Gene Rearrangement Chromogranin A RNA-Binding Proteins Gene rearrangement Middle Aged medicine.disease Prognosis Immunohistochemistry Gene Expression Regulation Neoplastic 030104 developmental biology 030220 oncology & carcinogenesis Primitive neuroectodermal tumor biology.protein Genital neoplasm Surgery Calmodulin-Binding Proteins Female Sarcoma Anatomy Medulloepithelioma RNA-Binding Protein EWS Follow-Up Studies |
| Zdroj: | Dipòsit Digital de Documents de la UAB Universitat Autònoma de Barcelona |
| Popis: | IGTP Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET. The remaining 4 PNETs were composed entirely of undifferentiated small round blue cells and were classified as Ewing sarcoma/peripheral PNET. Eight PNETs were associated with another tumor type, including 5 ovarian mature cystic teratomas, 2 endometrial low-grade endometrioid carcinomas and a uterine carcinosarcoma. By immunohistochemistry, 17 PNETs expressed at least 1 marker of neuronal differentiation, including synaptophysin, NSE, CD56, S100, and chromogranin in 10, 8, 14, 8, and 1 tumors, respectively. GFAP was positive in 4 PNETs, all of which were of central type. Membranous CD99 and nuclear Fli-1 staining was seen in 10 and 16 tumors, respectively, and concurrent expression of both markers was seen in both central and Ewing sarcoma/peripheral PNETs. All tumors expressed vimentin; while keratin cocktail (CAM5.2, AE1/AE3) staining was only focally present in 4 PNETs. Fluorescence in situ hybridization was successful in all cases and confirmed EWSR1 rearrangement in 2 of 4 tumors demonstrating morphologic features of Ewing sarcoma/peripheral PNET and concurrent CD99 and Fli-1 expression. In conclusion, central and Ewing sarcoma/peripheral PNETs may be encountered in the female genital tract with central PNETs being more common. Central PNETs show a spectrum of morphologic features that overlaps with CNS tumors but lack EWSR1 rearrangements. GFAP expression supports a morphologic impression of central PNET and is absent in Ewing sarcoma/peripheral PNET. Ewing sarcoma/peripheral PNETs lack morphologic features of CNS tumors. |
| Databáze: | OpenAIRE |
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