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[Giant primitive neuroendocrine cutaneous carcinoma of the leg]
D Lebas1, O Carpentier, E Martin De Lassalle
1Clinique Dermatologique, Hôpital Claude Huriez, Lille.
Introduction:
Primitive cutaneous neuroendocrine carcinoma or Merkel cell carcinoma is a tumor characterized by rapid evolution. The size at the time of diagnosis is a mean of 2 to 3 centimeters. We report a case of a particular clinical and anatomopathological presentation.
Observation:
A 76 year-old woman had a voluminous tumoral plaque on the left leg with multiple ulcero-necrotic lesions involving the bone structure without palpable, inguinal lymphadenopathy. The histological and immunocytochemistry examination led to the diagnosis of cutaneous neuroendocrine carcinoma. The normality of the chest, abdominal and pelvis investigations confirmed the primitive cutaneous origin. The patient died a few months later without lymph involvement.
Discussion:
To the best of our knowledge, such a presentation of primitive cutaneous neuroendocrine carcinoma has never been reported. The absence of lymphadenopathy despite the tumor volume and extensive skin involvement suggest the hypothesis of a non lymphophilic form of primitive cutaneous neuroendocrine carcinoma. This case report shows that lymphophily depends neither on the tumoral size nor to the degree of differentiation of tumoral cell proliferation, but on biological factors that require specification.
