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回肠血管外皮细胞瘤与冯雷克林霍增氏病

Ileal haemangiopericytoma and von Recklinghausen's disease.

作者信息

Neilly P J, Cooper G G, O'Hara M D, McGrady B J

机构信息

Department of Surgery & Pathology, Royal Victoria Hospital, Belfast.

出版信息

Br J Clin Pract. 1992 Autumn;46(3):212-3.

PMID:1286028
Abstract

Haemangiopericytoma is an uncommon tumour of vascular pericytes which usually arises in deep subcutaneous tissues or the retroperitoneum. There are few reports in the English literature of haemangiopericytoma arising in the ileum. Von Recklinghausen's disease, a condition of autosomal dominant inheritance with a prevalence of 1 in 3000, is often associated with tumours of neural origin. Tumours of non-neural origin also occur in Von Recklinghausen's disease but haemangiopericytoma has not been described. We report on a patient with Von Recklinghausen's disease presenting with massive haemorrhage from an ileal haemangiopericytoma.

摘要

血管外皮细胞瘤是一种罕见的血管周细胞瘤,通常发生于深部皮下组织或腹膜后。英文文献中关于回肠发生血管外皮细胞瘤的报道很少。冯·雷克林豪森病是一种常染色体显性遗传病,患病率为1/3000,常与神经源性肿瘤相关。非神经源性肿瘤也可发生于冯·雷克林豪森病,但尚未见有血管外皮细胞瘤的描述。我们报告1例患有冯·雷克林豪森病的患者,其回肠血管外皮细胞瘤出现大量出血。

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