Merkel cell carcinoma metastasis and dermatofibrosarcoma protuberans presenting as a collision tumour: a case report and review of the literature
Merkel cell carcinoma metastasis and dermatofibrosarcoma protuberans presenting as a collision tumour: a case report and review of the literature
Journal of Medical Case Reports 2009, 3:7493daigl
Published: August 2009
Published: August 2009
Introduction:
Merkel cell carcinoma and dermatofibrosarcoma protuberans are two very rare neoplasms. The simultaneous occurrence of two different tumour entities at the same anatomical site, collision tumours, is a rare phenomenon.
Case presentation:
We present a rare case of a 74-year-old woman with a previous history of a recurrent dermatofibrosarcoma protuberans presenting with a metastatic Merkel cell carcinoma. Further investigation revealed a collision tumour of a metastatic lesion of the Merkel cell carcinoma within a tumour relapse of a dermatofibrosarcoma protuberans.
Conclusion:
Synchronous occurrence of two different tumour entities is extremely rare and has not been described for Merkel cell carcinoma and dermatofibrosarcoma. Merkel cell carcinoma, a tumour of the elderly or immunocompromised patients, leads to early metastasis and can be expected to be the limiting factor for prognoses.
Introduction
Primary neuroendocrine tumour of the skin, also known as Merkel cell carcinoma (MCC), was first described by Toker in 1972. It is a rare but aggressive tumour entity of sun-exposed skin of the elderly in the age range of 60 to 90 years. The origin of this tumour is thought to be a line of differentiation according to Merkel cells. Under homeostatic conditions, the Merkel cells, discovered by Friedrich Merkel in 1875, are normal constituents of the basal layer of the epidermis and hair follicles. A higher density of Merkel cells is found in the glabrous epithelium of the digits, lips and oral cavity and hair bearing skin. They represent slow acting mechano-receptors with close contact to unmyelinated nervous fibres in the superficial dermis. Whether these cells are of a neuroendocrine or epidermal-ectodermal nature is still unclear. MCC, with a female predominance, 1 has a predilection for the head and neck region, a potential for early metastasis and is rarely encountered on the extremities and trunk. Conditions leading to impaired immunity, such as immunosuppression therapy 2, human immunodeficiency virus infection and chronic lymphocytic leukaemia have been associated with a higher incidence of MCC.
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