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成人囊性腹膜后淋巴管瘤

Cystic retroperitoneal lymphangiomas in adults.

作者信息

Hauser H, Mischinger H J, Beham A, Berger A, Cerwenka H, Razmara J, Fruhwirth H, Werkgartner G

机构信息

Department of Surgery, Karl Franzens University, Graz, Austria.

出版信息

Eur J Surg Oncol. 1997 Aug;23(4):322-6. doi: 10.1016/s0748-7983(97)90777-0.

Abstract

Lymphangioma (LA) is a rare benign tumour of the lymphatic tissue, most common in the neck and head, and clinically manifests itself mostly in childhood. Within this group, intra-abdominal and retroperitonal LA are the rarest tumours, especially when occurring in adults. We report four LAs localized in the retroperitoneum of patients aged between 28 and 72 years. One of these tumours infiltrated the transverse mesocolon and greater omentum, others were situated in the left retroperitoneum and retroperitoneally at the duodeno-jejunal flexure, and in the retrosplenal and retropancreatic area. Diagnosis was made by light microscopy supported by immunohistochemistry. In three cases the tumour could be removed by radical surgery and none of these patients had a recurrence (median follow-up time: 4 years). The tumour could not be removed completely from one patient with pre-operative chylascos. Six months after diagnosis of LA this patient died of cardiopulmonary failure due to progressive tumour chylascos. Isolation and ligation of the cystic LA's peduncle as well as ligation of lymph channels can prevent recurrences and chylascos.

摘要

淋巴管瘤(LA)是一种罕见的淋巴组织良性肿瘤,最常见于颈部和头部,临床上多在儿童期出现。在这一类型中,腹腔内和腹膜后LA是最罕见的肿瘤,尤其是在成人中发生时。我们报告了4例位于28至72岁患者腹膜后的LA。其中1例肿瘤侵犯横结肠系膜和大网膜,其他分别位于左腹膜后、十二指肠空肠曲腹膜后以及脾后和胰后区域。诊断通过光镜检查并辅以免疫组化。3例患者的肿瘤可通过根治性手术切除,且这些患者均无复发(中位随访时间:4年)。1例术前有乳糜腹水的患者肿瘤无法完全切除。LA诊断6个月后,该患者因肿瘤性乳糜腹水进展导致心肺功能衰竭死亡。分离并结扎囊性LA的蒂以及结扎淋巴管可预防复发和乳糜腹水。

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