Epithelioid Sarcoma of the Bartholin's Gland Primarily Diagnosed as Vulvar Carcinoma

Konefka, T; Senkus, Elżbieta; Emerich, Janusz; Dudziak, Mirosław

doi:10.1006/gyno.1994.1231

Cited by 24 publications

(15 citation statements)

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“…Excision should include 2 cm from the tumor margin. On the other hand, some authors prefer radical vulvectomy to local excision [9,[12][13][14][15]. Because of the aggressive biology, poor outcome, and resistance to radiation and chemotherapy of ES, radical resection including inguinal lymph node dissection is suggested as appropriate treatment, except for early stage disease.…”

Section: Discussionmentioning

confidence: 97%

“…In vulvar ES, chemotherapy has also been disappointing. The published patient reports have indicated that pulmonary metastasis has a very dismal prognosis, and chemotherapy adds little benefit to the treatment of the patients with metastatic disease [9,13,15]. Among human tumors, the c-kit proto-oncogene is frequent in gastrointestinal stromal tumors (GISTs); however; c-kit immunostaining has been found in some tumors other than GIST [19].…”

Section: Discussionmentioning

confidence: 97%

“…In contrast to extremity, vulvar ES has been an aggressive tumor that metastasizes earlier [6,7,9,12,13,15]. Although local recurrence of ES is common, survival is related to distant metastasis.…”

Section: Discussionmentioning

confidence: 98%

“…Epithelioid sarcoma (ES) is extremely rare. Since Piver et al first reported a case with ES in the vulva [5], 20 cases of vulvar ES have been published in the English literature [6][7][8][9][10][11][12][13][14][15]. One was diagnosed during pregnancy [13].…”

Section: Introductionmentioning

confidence: 98%

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Primary epithelioid sarcoma of the vulva in pregnancy

2007

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Primary epithelioid sarcoma (ES) of the vulva is an extremely rare tumor, and to date, only 20 cases have been reported. We report the second case of vulvar ES presenting in pregnancy. A 25-year-old woman, at 6 weeks of gestation, was diagnosed as having vulvar sarcoma and was referred to our hospital. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA), and wimentin. The tumor cells did not stain for S-100, SMA, HMB-45, desmin, LCA, CD-34, or estrogen and progesterone receptors. The tumor showed diffuse and strong staining for CD-117 (c-kit). After the curettage, she underwent local wide excision and left inguinal lymph node dissection and received external-beam radiation therapy. At 16 months of follow-up the patient was well without any disease recurrence. The appropriate treatment of vulvar ES is not clear, and the role of chemotherapy is disappointing; however, it might be worth investigating the role of Imatinib in c-kit overexpressing vulvar ES.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 98%

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Primary epithelioid sarcoma of the vulva in pregnancy

2007

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“…1 Primary epithelioid sarcoma (ES) of the vulva is an extremely rare tumor and only 20 cases have been reported in the English literature. 2–18 The majority of these cases were described before immunohistochemistry (IHC) was validated for use in routine surgical laboratories and the diagnosis was based solely on morphology and electron microscopy, in which the findings are usually not very specific for ES.…”

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confidence: 99%

Epithelioid Sarcoma of the Vulva in a 17-year-old Pregnant Woman

Rai¹,

Odunsi

Kesterson

et al. 2009

Applied Immunohistochemistry &Amp; Molecular Morphology

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Epithelioid sarcoma of the vulva is an extremely rare neoplasm with aggressive behavior and poor outcome. Herein, we report a case of vulvar epithelioid sarcoma presenting in a 17-year-old very early in her pregnancy. The patient presented with an asymptomatic nodule of the right labia majora of 1-year duration. Computerized tomographic scans showed enlarged inguinal lymph nodes and numerous lung nodules. Positron emission tomography was performed and revealed no suspicious lesions for metastatic disease. The patient underwent local excision of her vulvar lesion. On the basis of morphology and extensive immunohistochemistry, the lesion was classified as epithelioid sarcoma. The patient was referred to radiation therapy and upon evaluation, she was found to be in her sixth week of gestation. The patient continued with her pregnancy and underwent a lymph node dissection, which was positive. We review the literature of only the well-documented cases extensively studied by immunohistochemical analysis. We summarize the clinical presentation, clinical impression, treatment modalities, and outcomes of these cases. On account of the rarity of this disease in the vulva, there is no clear consensus on treatment modalities, but it seems that early aggressive surgical resection is the treatment of choice, with the role of adjuvant therapy to be determined.

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