Michal M, Lamovec J, Mukensnabl P, Pizinger K
Sikl's Department of Pathology, Medical Faculty, Charles University, Pilsen, Czech Republic.
Pathol Int. 1999 May;49(5):419-25. doi: 10.1046/j.1440-1827.1999.00890.x.
Twelve cases of spiradenocylindromas, which revealed features of both spiradenoma and cylindroma in the same tumor mass, are presented. Nine female patients had multiple neoplasms occurring mostly on the scalp, and two female and one male patient had a solitary cutaneous lesion. Three of the female patients with multiple cutaneous tumors had a familial history of similar cutaneous neoplasms. In one of the patient's family, the multiple cutaneous tumors were known to occur in multiple family members in four consecutive generations. One patient with multiple cutaneous lesions was known to have associated multiple kidney cysts as confirmed by computed tomography. Histologically, spiradenocylindromas are composed of intermixed areas that are either of typical spiradenoma in appearance or of typical cylindroma appearance. Apocrine and trichoepitheliomatous differentiation seen in two cases in the present series points to spiradenomas, as well as cylindromas, having complex hair follicle (folliculo-sebaceous apocrine) rather than eccrine differentiation. The presence of lymphoid tissue was a histological feature in the present series, which was prominent in all the spiradenomatous parts of the tumors and which was scanty or practically absent in all the cylindromatous parts. The selective presence of lymphocytes in spiradenoma and an absence in cylindroma suggest that spiradenomas have the unique property of attracting lymphocytes. The malignant tumors arising in three patients in the present series had the morphology of a poorly differentiated epithelioid neoplasm. Three patients died of the disease and the other patients were either free of disease or alive with disease 1-30 years on follow up.
本文报告了12例汗腺螺旋腺瘤样圆柱瘤,这些肿瘤在同一肿块中呈现出汗腺螺旋腺瘤和圆柱瘤的特征。9名女性患者有多发肿瘤,主要发生在头皮上,2名女性和1名男性患者有单发皮肤病变。3名有多发皮肤肿瘤的女性患者有类似皮肤肿瘤的家族史。在其中一名患者的家族中,四代人中多名家庭成员均有多发皮肤肿瘤。一名有多发皮肤病变的患者经计算机断层扫描证实伴有多发肾囊肿。组织学上,汗腺螺旋腺瘤样圆柱瘤由外观呈典型汗腺螺旋腺瘤或典型圆柱瘤的混合区域组成。本系列中的2例病例中见到的顶泌汗腺和毛发上皮瘤样分化表明,汗腺螺旋腺瘤以及圆柱瘤具有复杂的毛囊(毛囊 - 皮脂腺 - 顶泌汗腺)而非小汗腺分化。淋巴组织的存在是本系列的组织学特征,在肿瘤的所有汗腺螺旋腺瘤样部分中都很突出,而在所有圆柱瘤样部分中则稀少或几乎不存在。汗腺螺旋腺瘤中淋巴细胞的选择性存在以及圆柱瘤中淋巴细胞的缺失表明汗腺螺旋腺瘤具有吸引淋巴细胞的独特特性。本系列中有3名患者发生的恶性肿瘤具有低分化上皮样肿瘤的形态。3名患者死于该病,其他患者在随访1至30年期间无病或带病生存。