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小儿及成人患者的腹腔内和腹膜后淋巴管瘤

Intra-abdominal and retroperitoneal lymphangiomas in pediatric and adult patients.

作者信息

Goh Brian K P, Tan Yu-Meng, Ong Hock-Soo, Chui Chan-Hon, Ooi London L P J, Chow Pierce K H, Tan Carolyn E L, Wong Wai-Keong

机构信息

Department of Surgery, Singapore General Hospital, Singapore.

出版信息

World J Surg. 2005 Jul;29(7):837-40. doi: 10.1007/s00268-005-7794-0.

Abstract

Intra-abdominal and retroperitoneal lymphangiomas are a rare, congenital malformations of the lymphatics, which are found predominantly in children. The aim of this study is to evaluate the clinical features of this tumor, highlighting the differences in adults and pediatric patients. We also evaluate the preoperative diagnosis, radiological features, surgical treatment, and outcome of this rare condition. Between 1990 and 2004, 14 patients who underwent surgical resection of an intra-abdominal lymphangioma were reviewed retrospectively. There were five pediatric patients between fetal age and 17 years of age and nine adults between 31 and 62 years of age. Overall, females outnumbered males in the series, with a male-to-female ratio of 3:4. However, males predominated in the pediatric age group with a male-to-female ratio of 1.5:1. The clinical presentation of children was more acute ranging from 3 days to 2 months. In adults, four patients were asymptomatic, and the remaining five had symptom duration ranging from 2 weeks to a year. The lymphangiomas occurred in the mesentery (n = 4), retroperitoneum (n = 4), omentum (n = 3), pancreas (n = 2), and spleen (n = 1). All the patients underwent total surgical resection with or without organ resection, and there were no recurrences at a median follow-up of 2 years (range; 3 months--13 years). This series demonstrates that abdominal lymphangiomas have a male preponderance and present more acutely in pediatric patients, whereas in adults, female patients predominate and the history is more chronic.

摘要

腹内和腹膜后淋巴管瘤是一种罕见的先天性淋巴管畸形,主要见于儿童。本研究的目的是评估这种肿瘤的临床特征,突出成人和儿科患者的差异。我们还评估了这种罕见疾病的术前诊断、放射学特征、手术治疗及预后。回顾性分析了1990年至2004年间14例行腹内淋巴管瘤手术切除的患者。其中5例为胎儿期至17岁的儿科患者,9例为31至62岁的成人患者。总体而言,该系列中女性多于男性,男女比例为3:4。然而,在儿科年龄组中男性占主导,男女比例为1.5:1。儿童的临床表现更为急性,病程从3天至2个月不等。在成人中,4例无症状,其余5例症状持续时间从2周至1年不等。淋巴管瘤发生于肠系膜(n = 4)、腹膜后(n = 4)、大网膜(n = 3)、胰腺(n = 2)和脾脏(n = 1)。所有患者均接受了全手术切除,有或无器官切除,中位随访2年(范围:3个月至13年)无复发。该系列表明,腹部淋巴管瘤男性居多,儿科患者表现更为急性,而在成人中,女性患者占主导且病史更为慢性。

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