Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report

Rekhi, Bharat; Qureshi, Sajid; Basak, Ranjan; Desai, Sangeeta; Medhi, Seema; Kurkure, Purna; Menon, Santosh; Maheshwari, Amita; Jambhekar, Nirmala A.

doi:10.1186/1752-1947-4-88

Cited by 21 publications

(19 citation statements)

References 11 publications

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“…None of the PNETs occurring at these sites, including the present case, were found associated with another neoplasm (1, 26–39). While our patient with a vulvar Ewing sarcoma/peripheral PNET was postmenopausal, patients with Ewing sarcoma/peripheral PNETs arising in the cervix, vagina, and vulva tend to be of reproductive age (median, 35, 34, and 21 years, respectively) (26, 30, 31, 34, 36–38). …”

Section: Discussionmentioning

confidence: 76%

“…Interestingly, regardless of whether the tumor is central or peripheral type, patients with ovarian PNETs tend to be of reproductive age (median, 23 years) (1, 3–7, 45) compared to those with uterine PNETs who are typically postmenopausal (median, 57 years) (1, 9–18, 40, 41). Among the 14 PNETs with known EWSR1 rearrangement status reported in the cervix, vagina, and vulva (26, 30, 31, 34, 36–38), approximately 87%, including our single vulvar tumor, are Ewing sarcoma/peripheral PNETs confirmed by FISH or RT-PCR. None of the PNETs occurring at these sites, including the present case, were found associated with another neoplasm (1, 26–39).…”

Section: Discussionmentioning

confidence: 77%

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Primitive Neuroectodermal Tumors of the Female Genital Tract

Chiang

Snuderl

Kojiro-Sanada

et al. 2017

American Journal of Surgical Pathology

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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.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 77%

Primitive Neuroectodermal Tumors of the Female Genital Tract

Chiang

Snuderl

Kojiro-Sanada

et al. 2017

American Journal of Surgical Pathology

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“…[51213] While biopsy is a key in confirming the diagnosis, imaging remains critical in defining the extent of disease, in evaluating response to treatment, and in the detection of recurrent/metastatic disease. 18 F FDG-PET can play an important role in guiding the biopsy of these heterogeneous, often partially necrotic, vascular tumors.…”

Section: Discussionmentioning

confidence: 99%

Vaginal Ewing Sarcoma: An Uncommon Clinical Entity in Pediatric Patients

Cross

Stanescu

Rudzinski

et al. 2017

Journal of Clinical Imaging Science

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Ewing sarcoma, including classical Ewing sarcoma of the bone and primitive neuroectodermal tumors arising in bone or extraosseous primary sites, is a highly aggressive childhood neoplasm. We present two cases of Ewing sarcoma arising from the vagina in young girls. Previously reported cases in literature focused on their pathologic rather than radiographic features. We describe the spectrum of multimodality imaging appearances of Ewing sarcoma at this unusual primary site. Awareness of vaginal Ewing tumors may facilitate prompt diagnosis and lead to a different surgical approach than the more commonly encountered vaginal rhabdomyosarcoma.

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“…In the current study and previous seven cases immunohistochemical stains were used, while previous two cases used molecular analysis [4][5][6][7][8][9][10].…”

Section: Discussionmentioning

confidence: 99%

“…To date, only seven cases of vaginal ES/ PNET have been previously described and they were all diagnosed with no metastatic disease [4][5][6][7][8][9][10]. We present an additional case of vaginal ES/PNET with bilateral inguinal lymph nodes metastasis in a 54-year-old postmenopausal Chinese woman.…”

Section: Introductionmentioning

confidence: 92%