ArticleThe American journal of surgical pathology2017
Primitive Neuroectodermal Tumors of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Study of 19 Cases.
Article in The American journal of surgical pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 71 citations in OpenAlex.
- Primary pelvic extraskeletal Ewing sarcoma: a case report and comprehensive literature review of molecularly confirmed cases arising within the female genital tract.Gynecologic oncology reports · 2026Article
- Ovarian central nervous system-type tumors: integrated neuropathological and methylation-based classification reveals site-related features.Acta neuropathologica communications · 2026Article
- Primary Endometrium Primitive Neuroectodermal Tumor with Diagnostic and Treatment Challenges: A Case Report.International journal of women's health · 2026Article
- High-grade gliomas derived from an ovarian mature teratoma: clonal dynamics and genetic insights.International cancer conference journal · 2025Article
- Case report and review of the literature regarding management of primary uterine primitive neuroectodermal tumors (PNET).Gynecologic oncology reports · 2025Article
- Ewing sarcoma of the uterus: A case report.Case reports in women's health · 2024Article
- Consensus and controversy in the management of paediatric and adult patients with ovarian immature teratoma: the Malignant Germ Cell International Consortium perspective.EClinicalMedicine · 2024Review
- Bilateral primary ovarian Ewing sarcoma recurring as left submandibular lymphadenopathy diagnosed on cytology.Autopsy & case reports · 2024Article
- Clinicodemographic characteristics of extraosseous ewing sarcoma: A comparative meta-analysis of pediatric and adult patients.Journal of orthopaedics · 2023Review
- Targeted treatment of refractory primitive neuroectodermal tumor arising from an immature teratoma with crizotinib leading to a sustained response.Clinical case reports · 2023Article
- Extraosseous Ewing Sarcoma in Children: A Systematic Review and Meta-Analysis of Clinicodemographic Characteristics.Children (Basel, Switzerland) · 2022Review
- Desmoplastic Small Round Cell Tumor of the Uterus: A Report of Molecularly Confirmed Case with EWSR1-WT1 Fusion.Diagnostics (Basel, Switzerland) · 2022Article
- Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2021Article
- Importance of studying primitive neuroectodermal tumors and extraosseous Ewings sarcoma of the vagina and vulva.Oncology letters · 2021Review
- Primary Thyroid NUT Carcinoma With High PD-L1 Expression and Novel MassiveFrontiers in oncology · 2021Article
- Primary adult primitive neuroectodermal tumor of the bladder: A case report and literature review.Medicine · 2020Article
- Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature.World journal of clinical cases · 2019Article
- Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature.Orphanet journal of rare diseases · 2019Review
- Uterine corpus tumor with neuroectodermal differentiation and frequent ganglion-like cells in a postmenopausal woman.Gynecologic oncology reports · 2018Article
- Clinical management of a unique case of PNET of the uterus during pregnancy, and review of the literature.Medicine · 2018Review
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Authors and funding
13 authors at 9 institutions in 6 countries.
Funding
Abstract
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, whereas 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.
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