Primary Vulvar Sarcomas

Nirenberg, Alexander; Ag, Ostör; Slavin, James D.; Riley, Christopher B.; Rm, Rome

doi:10.1097/00004347-199501000-00010

Cited by 55 publications

(31 citation statements)

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“…9 Table 1 summarises the previously described cases together with the cases we report. One of the previously reported cases was thought to be consistent with Ewing's sarcoma, although CD99 was negative, 14 casting doubt on the diagnosis. In several other cases CD99 and/or FLI-1 immunostaining was not undertaken.…”

Section: Discussionmentioning

confidence: 93%

“…5 6 EFTs have rarely been described in the vulva and vagina and some of the reported cases have not had molecular or even immunohistochemical-that is, CD99 or FLI-1-confirmation. [7][8][9][10][11][12][13][14][15][16][17][18] In this report, we describe four new cases of EFTs involving the vulva or vagina, all with appropriate immunohistochemical staining patterns and three with molecular confirmation. In doing so, we undertake a review of the previously reported cases of EFTs involving these sites and discuss the differential diagnosis of these neoplasms, which rarely involve the lower part of the female genital tract.…”

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confidence: 99%

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Ewing family of tumours involving the vulva and vagina: report of a series of four cases

McCluggage¹,

Sumathi

Nucci

et al. 2007

J Clin Pathol

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Background: Ewing's sarcoma and peripheral primitive neuroectodermal tumour (pPNET) are now regarded as two morphological ends of a spectrum of neoplasms, characterised by a t(11;22) or other related chromosomal translocation involving the EWS gene on chromosome 22 and referred to as Ewing family of tumours (EFTs). EFTs are extremely rare in the vulva and vagina, a review of the literature revealing only 13 previously reported possible cases, most of which have not had molecular confirmation. In this study, four new cases of EFTs involving the vulva (three cases) or vagina (one case) are reported. Results: The tumours occurred in women aged 19, 20, 30 and 40 years and ranged in size from 3 to 8 cm. Morphologically, all neoplasms had a lobulated architecture and were composed of solid aggregates of cells. In one case, occasional rosettes were formed. In all the tumours, there was diffuse membranous staining with CD99; nuclear positivity with FLI-1 was present in two cases. Three cases were focally positive with the broadspectrum cytokeratin AE1/3, all were diffusely positive with vimentin and all were desmin negative. In two cases, a t(11;22) (q24;q12) (EWSR1-FLI-1) chromosomal translocation was demonstrated by reverse transcriptase-PCR (one case) and fluorescence in situ hybridisation (FISH) (one case), and in another case a rearrangement of the EWSR1 gene on chromosome 22 was demonstrated by FISH. In the other case, a variety of molecular studies did not reveal a translocation involving the EWS gene but this tumour, on the balance of probability, is still considered to represent a neoplasm in the EFTs. Follow-up in two cases revealed that one patient developed pulmonary metastasis and died and another is alive without disease at 12 months. Conclusions: This report expands the published literature regarding EFTs involving the vulva and vagina and stresses the importance of molecular techniques in firmly establishing the diagnosis, especially when these neoplasms arise at unusual sites.

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Section: Discussionmentioning

confidence: 93%

mentioning

confidence: 99%

Ewing family of tumours involving the vulva and vagina: report of a series of four cases

McCluggage¹,

Sumathi

Nucci

et al. 2007

J Clin Pathol

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“…The system we have used 4 categorizes all lesions into 6 categories, which include cutaneous vascular malformations, 7-11 cutaneous lesions characterized by dilation of preexisting vessels (including varices, 12-14 angiokeratomas, 15,16 and acquired lymphatic lesions 5,17,18 ), cutaneous vascular hyperplasia, [19][20][21][22][23] benign tumors of vascular endothelium, [24][25][26] malignant vascular tumors, [27][28][29][30][31][32] and cutaneous neoplasms with a significant vascular component. The system we have used 4 categorizes all lesions into 6 categories, which include cutaneous vascular malformations, 7-11 cutaneous lesions characterized by dilation of preexisting vessels (including varices, 12-14 angiokeratomas, 15,16 and acquired lymphatic lesions 5,17,18 ), cutaneous vascular hyperplasia, [19][20][21][22][23] benign tumors of vascular endothelium, [24][25][26] malignant vascular tumors, [27][28][29][30][31][32] and cutaneous neoplasms with a significant vascular component.…”

Section: Discussionmentioning

confidence: 99%

Vulvar Vascular Tumors

Papalas¹,

Sangüeza²,

Puri

et al. 2013

The American Journal of Dermatopathology

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The subepidermal hormonally sensitive tissue of the vulva is anatomically unique and may give rise to a wide variety of vascular tumors. As a consequence, classifying vulvar vascular lesions has been challenging due both to the wide variety of lesions that may be encountered and the heterogeneity in reporting across several disciplines. The purpose of this study is to present an institutional experience of vulvar vascular lesions. Overall, 85 patients were identified over a 26-year period. Vascular lesions belonging to the following classes included (n, %total) benign vascular tumors (32, 38%), dilatations of preexisting vessels (31, 36%), hyperplasia/reactive (7, 8%), tumors with significant vascular component (11, 13%), malformations (3, 4%), and malignant vascular tumors (1, 1%). Two reaction patterns based on vulvar lymphatic pathology were identified: one is a stromal dominant pattern and the other is a vascular dominant pattern. Vulvar vascular malformations and true vascular malignancies, although rare, may have associated high morbidity. To accurately classify vulvar lymphatic lesions, the pathologist must carefully consider the patient's clinical history taking into account features such as preexisting lymphedema.

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“…Prognosis seems to depend on the size of the lesion, tumor involvement of adjacent tissue, and mitotic activity. Women with lesions greater than 5 cm in diameter, with infiltrating margins, extensive necrosis and with more than five mitotic figures per 10 high power fields, are more likely to have recurrent disease following surgical excision [1,7]. …”

Section: Discussionmentioning

confidence: 99%

“…The most common vulvar sarcomas are leiomyosarcomas, rhabdomyosarcomas, angiosarcomas, neurofibrosarcomas, malignant fibrohistiocytomas, and aggressive angiomyxomas [1]. Among primary vulvar sarcomas, primary extraskeletal mesenchymal chondrosarcomas (EMC) are extremely rare, and have higher rate of misdiagnosis.…”

Section: Introductionmentioning

confidence: 99%

Primary extraskeletal mesenchymal chondrosarcoma of the vulva

et al. 2013

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Extraskeletal chondrosarcoma is rare, making up only 1% of reported chondrosarcoma. We experienced 3 cases of extraskeletal chondrosarcoma, especially in vulva. They were suspected as lipoma of the vulva. The patients had noticed a small but growing mass on their vulva which had been palpated earlier. The masses were excised with a 2 cm resection margin. The final pathological reports confirmed extraskeletal mesenchymal chondrosarcoma (EMC) of the vulva revealing no microscopic lesions on the resection margins. After 24 months of following from the initial diagnosis, the patients remain without evidence of any recurrent. Management of EMC is not well studied due to the rare and variable nature of the disease. However, the surgery, such as we had, is the mainstay of local treatment with studies showing better survival in patients who undergo wide surgical resection. The establishment of adjuvant systemic pharmacotherapy could be expected in the future.

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Primary Vulvar Sarcomas

Cited by 55 publications

References 0 publications

Ewing family of tumours involving the vulva and vagina: report of a series of four cases

Ewing family of tumours involving the vulva and vagina: report of a series of four cases

Vulvar Vascular Tumors

Primary extraskeletal mesenchymal chondrosarcoma of the vulva

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