Jeunon Thiago, Sampaio Ana Luisa Bittencourt, Caminha Ricardo C, Reis Carlos Umberto da Cunha, Dib Cassio
Dermatopathology Department, Dermatology Unit, Hospital Federal de Bonsucesso, Brazil.
An Bras Dermatol. 2011 Nov-Dec;86(6):1167-73. doi: 10.1590/s0365-05962011000600016.
Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.
肾小球样血管瘤的特征是在扩大的血管腔内含有盘绕的毛细血管,其结构类似于肾小体。该病症与POEMS综合征(多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变)密切相关。本文报道了两例与POEMS综合征相关的肾小球样血管瘤病例,并对文献进行了综述。病例一为一名63岁女性患者,因腹水、肝脾肿大、行走困难和皮肤血管瘤入院。组织病理学检查确诊为肾小球样血管瘤,并指导了临床检查,结果显示为感觉运动性多发性神经病、L4椎体浆细胞瘤,肿瘤细胞κ链阳性,以及糖尿病。这些发现确诊为POEMS综合征。第二例是一名39岁女性,因水肿、腹水、胸腔积液、肾小球样血管瘤和淋巴结病(卡斯尔曼病)入院。其他发现包括单克隆IgG-λ副蛋白血症、右髂骨和L4椎体的原始细胞病变,以及累及四肢的脱髓鞘感觉运动性神经病。该病例的最终诊断为与卡斯尔曼病相关的POEMS综合征。