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一例伴有广泛成熟的巨大儿童肠系膜脂肪母细胞瘤

A Giant Childhood Mesenteric Lipoblastoma With Extensive Maturation.

作者信息

Squillaro Anthony I, Chow Monica D, Arias Fernando, Sadimin Evita T, Lee Yi-Horng

机构信息

Department of Surgery, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United States.

Department of Pathology, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United States.

出版信息

Front Pediatr. 2020 Jul 24;8:404. doi: 10.3389/fped.2020.00404. eCollection 2020.

Abstract

Abdominal lipoblastomas are uncommon soft tissue tumors in children and rarely arise from the mesentery. Due to intraabdominal location and slow growth, these masses can go unnoticed for long periods of time and often found on surgical exploration. We present a case of a 12-year-old male with years of abdominal distension accompanied by new onset early satiety that was found to have an intra-abdominal mass. He underwent an exploratory laparotomy revealing a large 33 x 27 x 15 cm rubbery mesenteric mass displacing the entire intra-abdominal contents, connected by a single vascular pedicle and encasing a loop of small intestine. The mass was resected and the patient did well without signs of recurrence. Histology confirmed the presence of mature adipocytes but on further cytogenetic analysis, a translocation between chromosomes 2 and 8 at the 12q arm was detected, which is often associated with lipoblastomas. This case represents the one of the largest mesenteric lipoblastomas that matured extensively to lipoma-like histology at the time of surgical resection.

摘要

腹部脂肪母细胞瘤是儿童罕见的软组织肿瘤,很少起源于肠系膜。由于位于腹腔内且生长缓慢,这些肿块可能长时间未被发现,常在手术探查时才被发现。我们报告一例12岁男性,有多年腹胀伴新发早饱症状,发现腹腔内有肿块。他接受了剖腹探查术,发现一个33×27×15 cm的巨大橡胶样肠系膜肿块,推移了整个腹腔内容物,通过单一血管蒂相连,并包绕一段小肠。肿块被切除,患者恢复良好,无复发迹象。组织学证实存在成熟脂肪细胞,但进一步的细胞遗传学分析检测到12q臂上2号和8号染色体之间的易位,这常与脂肪母细胞瘤相关。该病例是手术切除时广泛成熟为脂肪瘤样组织学的最大肠系膜脂肪母细胞瘤之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d85/7393439/de0976afaa33/fped-08-00404-g0001.jpg

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