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家族性脾脏表皮样囊肿:两例报告。

Familial epidermoid cysts of the spleen: report of two cases.

作者信息

Kubo Masatoshi, Yamane Masaomi, Miyatani Katsuya, Udaka Tetsunobu, Mizuta Minoru, Shirakawa Kazutoyo

机构信息

Department of Surgery, Mitoyo General Hospital, 708 Himehama, Toyohama-cho, Kagawa 769-1695, Japan.

出版信息

Surg Today. 2006;36(9):853-6. doi: 10.1007/s00595-006-3244-3.

Abstract

The familial occurrence of epidermoid cysts of the spleen is rare, with only six cases having ever been reported, to our knowledge. We recently diagnosed epidermoid cysts of the spleen in a mother and son. First, a 15-year-old boy was admitted to our hospital for management of blunt abdominal trauma. Computed tomography (CT) showed a ruptured large splenic cyst with an intraabdominal hematoma. We performed a splenectomy, and histopathological examination confirmed the existence of an epidermoid cyst of the spleen. About 2 years and 6 months later, the family physician found that the patient's 41-year-old mother had a large splenic cyst, and she was referred to our hospital for further investigation. CT showed a 10 x 8 cm cyst occupying most of the spleen. The patient underwent splenectomy, and a pathological diagnosis of an epidermoid cyst of the spleen was confirmed. Although the etiology of epidermoid cysts of the spleen is unclear, this familial occurrence may support the hypothesis of congenital malformation as a result of genetic change.

摘要

据我们所知,脾脏表皮样囊肿的家族性发病较为罕见,仅有6例报告。我们最近诊断出一对母子患有脾脏表皮样囊肿。首先,一名15岁男孩因钝性腹部外伤入院治疗。计算机断层扫描(CT)显示一个破裂的巨大脾囊肿伴腹腔内血肿。我们进行了脾切除术,组织病理学检查证实存在脾脏表皮样囊肿。大约2年6个月后,家庭医生发现患者41岁的母亲有一个巨大的脾囊肿,她被转诊至我院进一步检查。CT显示一个10×8cm的囊肿占据脾脏的大部分。患者接受了脾切除术,病理诊断为脾脏表皮样囊肿。虽然脾脏表皮样囊肿的病因尚不清楚,但这种家族性发病可能支持因基因改变导致先天性畸形的假说。

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