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儿童脂肪母细胞瘤:12例病例回顾

Lipoblastoma in children: Review of 12 cases.

作者信息

Susam-Sen Hilal, Yalcin Bilgehan, Kutluk Tezer, Cahit Tanyel Feridun, Haliloglu Mithat, Orhan Diclehan, Aydin Burca, Kurucu Nilgun, Varan Ali, Akyuz Canan

机构信息

Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Department of Pediatric Surgery, Hacettepe University Faculty of Medicine, Ankara, Turkey.

出版信息

Pediatr Int. 2017 May;59(5):545-550. doi: 10.1111/ped.13239. Epub 2017 Apr 7.

Abstract

BACKGROUND

Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization, and signs of compression of adjacent organs may be seen.

METHODS

A total of 12 children with pathologically proven lipoblastoma from 2000 to 2014 were reviewed retrospectively for their clinical features, treatment and follow up.

RESULTS

There were seven boys and five girls between 7.5 months and 7.5 years of age. The most common symptom was painless rapid-growing mass. The tumors were located in the neck, the abdomen, the groin, pelvis, axilla, glutea, labium majus, thigh and trunk. The largest tumor was in a retroperitoneal location and was 13 × 10 cm in size. Complete resection was performed in 10 patients. All of these patients were followed without any evidence of recurrence. Subtotal excision was performed in two patients. At a median follow up of 38 months (range, 1.8 months-10 years), all patients were disease free.

CONCLUSIONS

Lipoblastoma should be considered in the differential diagnosis of rapidly growing mass in patients <3 years of age. Total excision is the treatment of choice with preservation of vital organs. The prognosis is excellent despite large tumor size and local invasion. Recurrence rate is high in incompletely resected tumors. Regular follow up is important for early detection of recurrence.

摘要

背景

脂肪母细胞瘤是一种罕见的婴幼儿及儿童早期良性间叶组织肿瘤。症状因肿瘤部位而异,可能出现邻近器官受压的体征。

方法

回顾性分析2000年至2014年间12例经病理证实为脂肪母细胞瘤患儿的临床特征、治疗及随访情况。

结果

患儿年龄7.5个月至7.5岁,男7例,女5例。最常见的症状是无痛性快速生长的肿块。肿瘤位于颈部、腹部、腹股沟、骨盆、腋窝、臀部、大阴唇、大腿及躯干。最大的肿瘤位于腹膜后,大小为13×10cm。10例患者行完整切除,所有这些患者随访均无复发迹象。2例患者行次全切除。中位随访38个月(范围1.8个月至10年),所有患者均无疾病。

结论

对于3岁以下快速生长肿块的患者,鉴别诊断时应考虑脂肪母细胞瘤。首选治疗方法是在保留重要器官的情况下完整切除。尽管肿瘤体积大且有局部侵犯,但预后良好。不完全切除的肿瘤复发率高。定期随访对于早期发现复发很重要。

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