Lebas D, Carpentier O, Martin De Lassalle E, Vilain M-O, Piette F
Clinique Dermatologique, Hôpital Claude Huriez, Lille.
Ann Dermatol Venereol. 2004 Jun-Jul;131(6-7 Pt 1):579-82. doi: 10.1016/s0151-9638(04)93671-x.
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.
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.
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.
原发性皮肤神经内分泌癌或默克尔细胞癌是一种以快速发展为特征的肿瘤。诊断时的大小平均为2至3厘米。我们报告一例具有特殊临床和解剖病理学表现的病例。
一名76岁女性，左腿有一个巨大的肿瘤斑块，伴有多个累及骨骼结构的溃疡坏死性病变，腹股沟无可触及的淋巴结肿大。组织学和免疫细胞化学检查确诊为皮肤神经内分泌癌。胸部、腹部和骨盆检查正常证实了原发性皮肤起源。患者几个月后死亡，无淋巴结受累。
据我们所知，原发性皮肤神经内分泌癌的这种表现从未有过报道。尽管肿瘤体积大且皮肤广泛受累，但无淋巴结肿大提示存在非嗜淋巴性原发性皮肤神经内分泌癌的假说。本病例报告表明，嗜淋巴性既不取决于肿瘤大小，也不取决于肿瘤细胞增殖的分化程度，而是取决于需要明确的生物学因素。
