Merkel Cell Carcinoma of the Vulva

García-Jiménez, Ángel; Gonzalez-Bosquet, Jesus; Esteller, Manel; Castellví-Vives, J; Palones, J M Martínez; Xercavins, Jordi

doi:10.1006/gyno.1996.4575

Cited by 31 publications

(17 citation statements)

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“…Local tumor + overlying discolored purplish skin. bilateral inguinal LN metastasesInitial: Vulvectomy + bilateral inguinal and Left pelvic Lymphadenectomy 2 months: RadiotherapyResidual pelvic nodes post treatment.2 months: para aortic LN.5 months: Alive with residual disease.Gil et al 1997 [19]74Right labium majus 9 cm3–4 month history of local tumorInitial: Wide Local excision13 months: free of diseaseFawzi et al 1997 [20]78Right vulvar mass 5.5 x 4 cm1 month history of perineal itching and discomfort. Pulmonary LN metastases.Initial: Radical vulvectomy + bilateral inguinal LN dissection20 days postoperative: break down of right groin site and subsequent death due to bleeding.…”

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confidence: 99%

Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review

Nguyen

Tahseen

Vaudreuil

et al. 2017

gynaecol oncol res pract

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BackgroundMerkel cell carcinoma is a rare and aggressive neoplasm originating from mechanoreceptor Merkel cells of the stratum basale of the epidermis. Cases affecting the vulva are exceedingly rare, with the currently available literature primarily in case report form.BodySystematic review of the PubMed database returned 17 cases of Merkel cell carcinoma affecting the vulva. Patients presented at a mean age of 59.6 years with a firm, mobile vulvar mass. Symptoms of pain, erythema, pruritus, edema, and ulceration have been reported. Tumor histology is consistent with that of neuroendocrine tumors and typical Merkel cell carcinomas. Neuroendocrine and cytokeratin immunostains are frequently utilized in histopathological workup. Surgical management was the unanimous first-line therapy with adjuvant radiation in most cases. Recurrence occurred in 70.6% of patients at a mean follow-up of 6.3 months. Mortality was at 47.0% at a mean of 7.8 months after initial operation.ConclusionMerkel cell carcinoma affecting the vulva is an extremely rare and highly aggressive neoplasm. The present review of published cases serves to comprehensively describe the clinical course and treatment approaches for vulvar Merkel cell carcinoma.

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confidence: 99%

Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review

Nguyen

Tahseen

Vaudreuil

et al. 2017

gynaecol oncol res pract

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“…Tumor locations are buttocks (43%), extremities (36%), head (7%), unknown (14%) [11]. Because of its rarity, it is not known whether this neoplasm behaves differently in the vulvar location from at other sites [12]. Less than twenty cases of vulvar Merkel carcinomas are reported [12–14].…”

Section: Discussionmentioning

confidence: 99%

“…Because of its rarity, it is not known whether this neoplasm behaves differently in the vulvar location from at other sites [12]. Less than twenty cases of vulvar Merkel carcinomas are reported [12–14]. Furthermore, a few cases of Merkel cell carcinoma of the Bartholin's gland are reported in the bibliography [15].…”

Section: Discussionmentioning

confidence: 99%

Vulvar Merkel Carcinoma: A Case Report

Iavazzo

Terzı

Arapantoni-Dadioti

et al. 2011

Case Reports in Medicine

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This is a new case of Merkel cell carcinoma of the vulva. It is a rare neuroendocrine carcinoma with an aggressive behavior. Because of its rarity in this location, it is not clear whether it behaves differently from the usual neuroendocrine carcinomas of the skin. A case of a 63-year-old patient with vulvar Merkel carcinoma is presented. The clinical presentation, microscopic and immunohistochemical features, and treatment are discussed.

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“…12 The behavior of Merkel cell tumors seems to be exceptionally aggressive when they appear in unusual locations such as the buttocks, scrotal sac, and vulva, with systemic metastases present either at presentation or soon thereafter. [1][2][3][4][5] The diagnosis of this tumor in our patient was based on histologic identification of the biopsy specimen and subsequent confirmation by immunohistochemistry and electron microscopy. Although Merkel cell carcinomas are a type of small-cell neuroendocrine carcinoma, they are primarily cutaneous and are distinguishable from neuroendocrine tumors found in the gastrointestinal tract.…”

Section: Discussionmentioning

confidence: 99%

“…Merkel cell tumors are rare neuroendocrine small-cell tumors of skin that typically arise on sun-exposed areas and seem to have a more aggressive nature when found in atypical locations. [1][2][3][4][5] Biopsy is the only method of distinguishing and diagnosing these tumors. Although neuroendocrine tumors of the rectum have been described, we report what we believe is the first recorded case of a Merkel cell tumor arising in the anal canal.…”

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confidence: 99%

Merkel Cell (Neuroendocrine) Carcinoma of the Anal Canal

Paterson

Musselman²,

Chorneyko³

et al. 2003

Diseases of the Colon &Amp; Rectum

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Merkel cell tumors are rare neuroendocrine tumors typically found on sun-exposed areas such as extremities. We describe the case of a 42-year-old female with a Merkel cell tumor arising in the anal canal. The tumor was initially thought to represent a hemorrhoid arising during pregnancy and was excised locally after confirmation of extensive metastatic disease. The patient died 13 months after diagnosis with extensive metastatic disease involving the liver. In our search of the world literature there are several reports of neuroendocrine tumors in the rectum; however, no cases of documented Merkel cell tumors arising in the anal canal have been reported. These tumors seem to behave in a very aggressive manner when found in other atypical areas. The presentation symptoms were perianal discomfort and bleeding. Local excision may be the only surgical treatment necessary to control symptoms, given the propensity to early metastases and short life expectancy.

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Merkel Cell Carcinoma of the Vulva

Cited by 31 publications

References 0 publications

Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review

Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review

Vulvar Merkel Carcinoma: A Case Report

Merkel Cell (Neuroendocrine) Carcinoma of the Anal Canal

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