Demirhan B, Sökmensüer C, Karakayali H, Güngen Y, Doğan A, Haberal M
Department of Pathology, Baskent University School of Medicine, Anakara, Turkey.
J Clin Pathol. 1997 Jan;50(1):74-6. doi: 10.1136/jcp.50.1.74.
Extramedullary plasmacytoma of the liver is a rare tumour, only two cases of which have been reported so far. A third case arising in a 22 year old woman, who presented with abdominal pain and enlargement of the liver, is described. Ultrasound and a computed tomography scan showed a solitary hepatic mass, 12 cm diameter, involving both lobes of the liver. Serum immunoelectrophoresis revealed an IgG kappa monoclonal gammopathy. Histologically, the tumour was composed of mature plasma cells with mild atypia. The plasma cells infiltrated the liver parenchyma and showed kappa light chain restriction. The monoclonal nature of the tumour was also demonstrated by PCR amplification of the immunoglobulin heavy chain genes. There was no evidence of bone involvement and repeated bone marrow aspirates and biopsy specimens were normal. The patient was treated with eight courses of chemotherapy. One year after diagnosis, the patient is well, the size of the tumour has decreased and the paraproteinaemia has disappeared.
肝脏髓外浆细胞瘤是一种罕见肿瘤,迄今为止仅报道过两例。本文描述了第三例发生在一名22岁女性身上的病例,该患者表现为腹痛和肝脏肿大。超声和计算机断层扫描显示肝脏有一个直径12厘米的孤立肿块,累及肝脏两叶。血清免疫电泳显示IgG κ单克隆丙种球蛋白病。组织学上,肿瘤由具有轻度异型性的成熟浆细胞组成。浆细胞浸润肝实质并显示κ轻链限制。免疫球蛋白重链基因的PCR扩增也证实了肿瘤的单克隆性质。没有骨受累的证据,多次骨髓穿刺和活检标本均正常。该患者接受了八个疗程的化疗。诊断一年后,患者情况良好,肿瘤体积减小,副蛋白血症消失。