Neuroendocrine Carcinoma (Merkel Cell Tumour) and Amphicrine Tumours

Abstract

Merkel cell was named after Friedrich Sigmund Merkel, who in 1875 described a special sensory cell (“tastzell”) in the epidermis of vertebrates. These cells are also present in the hair follicles of mammals and have ultrastructural similarities to APUD cells containing neurosecretory granules. The tumours originating in these cells have been variously labelled as trabecular carcinoma of the skin (Toker 1972), neuroendocrine carcinoma of the skin (Sibley et al. 1980), small cell neuroepithelial tumour of the skin (Silva and Mackay 1980), primary small cell carcinoma of the skin (Taxy et al. 1980) and cutaneous APUDOMA (Wolf-Peters et al. 1980). They occur commonly in the head and neck region and extremities (Frigerio et al. 1982; Raaf et al. 1986) but have also been reported from the face, ear, cheek, leg, buttock and forearm. The vulval tumour reported by Bottles et al. (1984) occurred on the left labium majus of a 73-year-old woman who also had in situ squamous cell carcinoma adjacent to the Merkel cell tumour. She had nodal and bony metastases. The patient reported by Copeland et al. (1985) was 59 years old and presented with a painful lump in the left Bartholin’s gland area. She was treated by left hemivulvectomy and left superficial lymph node dissection followed by radiation. There was local recurrence and pulmonary metastases, and she died 4 months later (Copeland et al. 1985). Husseinzadeh et al. (1988) reported a lesion in a 47-year-old woman who presented with vaginal discharge and right labial and groin swelling for 3 months and was treated by radical vulvectomy and inguinal node dissection followed by post-operative radiation. She died of metastatic disease.