Neuroendocrine tumor of vulva: A case report and review of literature

Abstract

Neuroendocrine tumor (Merkel cell carcinoma-MCC) of the vulva is a very rare entity with less than 15 cases reported in the English literature. It is known for its aggressive behaviour and propensity for early dissemination. The actual cell of origin and etiology of this disease is controversial. In absence of any definite guidelines for management (due to its rarity), extrapolation of data from extra-vulvar MCC seems logical. We present a case of vulvar neuroendocrine tumor who presented at a locally advanced stage.

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

“…Pulmonary LN metastases.Initial: Radical vulvectomy + bilateral inguinal LN dissection20 days postoperative: break down of right groin site and subsequent death due to bleeding. No autopsy.Hierro et al 2000 [18]79Left labium minus 2.5 cmLocal tumorInitial: local excision.2 months: Radiotherapy2 months local recurrence and regional LN metastases.10 months: DeathNuciforo et al 2004 [17]62Right labia majora 20 mmLocal painful tumor.Initial: local excision.3 months: Radical vulvectomy + Radiotherapy.3 months: bilateral inguinal LN metastases.11 months: abdominal and mediastinal LN.19 months: Alive with Several abdominal and thoracic metastases.Khoury et al 2005 [16]49Right vulvar mass 2 cmSpontaneously ruptured Bartholin’s gland abscess with small induration at the site.Initial: Drained abscess + wide local excision + bilateral LN dissection + Radiation therapy24 months: Alive with no evidence of recurrence.Pawar et al 2005 [15]35Left labium majus 4 x 6 cmOne week history of painful swelling of the vulva + purulent discharge + LN massInitial: Drained abscess + antibiotics + partial excisionNo follow up, patient planned to receive radiotherapy in her home country.Mohit et al 2009 [14]50Left labia majora 3–4 cm3 month history of palpable mass.Initial: local excision2 months: Radiotherapy2 months, 3 weeks: radical vulvectomy9 months: Chemotherapy2 months: Recurrent mass 10 x 12 cm w/spontaneously bleeding ulcerations9 months: left hip pain10 months: no evidence of metastases11 months: death due to Pulmonary embolism secondary to DVT of LLE.Sheikh et al 2010 [13]63Right labium majus 5 x 7 cmPost menopausal bleeding with fungating primary lesion.Initial: wide local excision.2 months: local + distant recurrence with multiple firm inguinal LN bilaterally + death before follow up treatmentIavazzo et al 2011 [12]63Left Labium 9 cm6 month history of pruritus treated w/corticosteroid cream. 5 cm inguinal LN metastases.Initial: radical vulvectomy + radiotherapyNo follow upWiner et al 2012 [11]69Right inguinal 3–4 cmPatient noted Inguinal lesion.Initial: Surgical excisionFuture plans for adjuvant chemotherapy + radiotherapyNo follow up …”

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

“…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).…”

“…Neuroendocrine carcinoma (Merkel cell carcinoma) of the vulva is an extremely rare entity with fewer than 20 cases reported in the English literature to date. [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.…”

Combined squamous cell carcinoma and Merkel cell carcinoma of the vulva: Role of human papillomavirus and Merkel cell polyomavirus

“… 1 Although some cutaneous MCCs were reported to be associated with squamous cell carcinomas (SCCs), such combined cases accounted for only a minor portion and the viral status appeared to be different from pure MCC. 1 , 2 , 3 , 4 Rarely, primary MCCs occur on the female vulva, 5 with or without combined SCCs, 6 , 7 the latter is often human papillomavirus (HPV) related.…”