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

Abstract

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.

Advanced neuroendocrine carcinoma (Merkel cell carcinoma) of the vulva: a case report and literature review

“…The age of the patient described in the present case report (34 years old) is far younger than the mean age reported in the systematic review by Nguyen et al 19 of 59.6 (28-79) years old; however, two of the cases in their series were younger than 30 years of age (both 28 years old).…”

“…To date, 18 cases of neuroendocrine carcinoma of the vulva have been described in the English literature, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] as well as 2 cases of neuroendocrine carcinoma of Bartholin's gland. 22,23 A recent systematic review on the subject by Nguyen et al 19 (in 2017) included 17 of these cases (16 cases of neuroendocrine carcinoma of the vulva and 1 case of neuroendocrine carcinoma of Bartholin's gland). Two further cases of neuroendocrine carcinoma of the vulva were documented by Aminimoghaddam et al 1 in 2016 and Correia et al 6 in 2017 ( Table 1).…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Most cases show aggressive behaviour, with an almost universally reported poor outcome. 19 However, isolated reported cases have demonstrated better outcomes with complete surgical excision. 1 In sites other than the vulva, neuroendocrine carcinoma (Merkel cell carcinoma) of the skin is a rare and aggressive tumour predominantly affecting elderly persons, with purported pathogenetic mechanisms including ultraviolet light exposure 20 and polyomavirus infection.…”

Merkel cell carcinoma: literature review

“…The rest 10% reported in the trunk [4]. Non-sun exposed area MCC also has been reported as vulva [12], tongue [13] and gluteal area [14]. In the year 2008, a team from University of Pittsburgh, USA, came with new break through related to the pathogenesis of MCC.…”

Merkel Cells and Merkel Cell Carcinoma of Genitourinary System Tumor with Aggressive Behavior and Poor Prognosis

“…Histologically, MCC has been classified into three distinct subtypes: trabecular, intermediate, small-cell type [33]. Diagnosis is based on typical histology representation on haematoxylin-eosin stained slides together with the results of immunohistochemistry [34]. [16].…”