Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

Kelling, Katharina; Noack, F.; Altgassen, C.; Kujath, P.; Bohlmann, Michael K.; Hoellen, Friederike

doi:10.1007/s00404-011-2011-x

Cited by 20 publications

(16 citation statements)

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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%

“…Ectopic migration of neuroblasts from the neural crest during fetal development (9, 64) may be yet another hypothesis in the development of gynecologic PNETs in general, but does not explain the particular propensity for PNETs to develop in the female reproductive tract compared to the urinary or male genital tracts. The predominance of EWSR1 -rearranged Ewing sarcoma/peripheral PNETs found in the cervix (19, 21, 22), vagina (26, 30, 31), and vulva (26, 31, 34, 36–38) suggests a developmental pathway at these sites similar to those arising in the soft tissues.…”

Section: Discussionmentioning

confidence: 99%

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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%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Primitive Neuroectodermal Tumors of the Female Genital Tract

Chiang

Snuderl

Kojiro-Sanada

et al. 2017

American Journal of Surgical Pathology

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“…14 Extraosseous location of EwS and PNETs is unusual and vulvar involvement is extremely rare, with 15 cases reported in the literature to date. 15,16 Association with a number of chromosomal translocations is known, most often t (11; 22) (q24; q12), as well as translocations associated with the EwS gene on chromosome 22, commonly found in Ewing family of tumors (EFTs).…”

Section: Ewing Sarcoma Of the Vulvamentioning

confidence: 99%

“…15 It is aggressive with a highly malignant course and development of early metastasis. 14 Morphologically EwS usually displays a lobulated architecture, composed of solid aggregates of cells, sometimes forming rosettes. 19 Histologically it shows the typical features of EwS: monomorphic population of small round blue cells with cytoplasmic glycogen, which is confirmed by periodic acid-Schiff.…”

Section: Ewing Sarcoma Of the Vulvamentioning

confidence: 99%

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

Chokoeva¹,

Tchernev

Cardoso

et al. 2015

Int J Immunopathol Pharmacol

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Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.

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