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脂肪母细胞瘤的治疗现状。

Current management of lipoblastoma.

机构信息

Department of Pediatric and Adolescent Surgery, Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Kaplan St 14, 4920235, Petah Tikva, Israel.

Department of Pathology, Rabin Medical Center, Beilinson Hospital, Sackler School of Medicine, Tel Aviv University, Petah Tikva, Israel.

出版信息

Eur J Pediatr. 2018 Feb;177(2):237-241. doi: 10.1007/s00431-017-3059-9. Epub 2017 Dec 14.

Abstract

UNLABELLED

Lipoblastoma is a rare and benign tumor arising from embryonal fat cells. It is generally diagnosed in children younger than 3 years of age and can occur in the extremities or on the trunk. We present our series of 10 children with lipoblastoma treated at Schneider Children's Medical Center of Israel between 2011 and 2016. Six boys and four girls underwent tumor resection at a median age of 2 years and 3 months (range 5 months to 5.6 years). Locations were trunk (6), groin (2), perineum (1), and omentum (1). Follow up ranges from 1 to 5 years. Two patients had a local recurrence and required a second resection 2 years (perineal) and 6 years (trunk) after the first surgery without further recurrence at 1.9 and 2.9 years, respectively.

CONCLUSION

Higher awareness of lipoblastoma enables optimal imaging strategies and resection. Long follow up is required due to local recurrences. The treatment of choice consists of complete, but non mutilating surgical resection. What is Known: • Lipoblastoma is a rare benign tumor of fatty tissue affecting children • Treatment consists of surgical resection What is New: • MRI is the modality of choice for follow up • Ten-year long-term follow up is required due to late recurrence.

摘要

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脂肪母细胞瘤是一种罕见的良性肿瘤,来源于胚胎脂肪细胞。它通常在 3 岁以下的儿童中诊断出来,可以发生在四肢或躯干上。我们报告了以色列施耐德儿童医疗中心在 2011 年至 2016 年间治疗的 10 例脂肪母细胞瘤患儿。6 名男孩和 4 名女孩在中位年龄为 2 岁和 3 个月(5 个月至 5.6 岁)时接受了肿瘤切除术。肿瘤部位为躯干(6 例)、腹股沟(2 例)、会阴(1 例)和大网膜(1 例)。随访时间为 1 至 5 年。2 例患者出现局部复发,分别在第一次手术后 2 年(会阴)和 6 年(躯干)再次接受第二次切除术,无进一步复发,分别在 1.9 年和 2.9 年。

结论

提高对脂肪母细胞瘤的认识可以优化影像学策略和切除术。由于存在局部复发,需要进行长期随访。治疗选择包括完整但非致残性的手术切除。已知:脂肪母细胞瘤是一种罕见的儿童期良性脂肪组织肿瘤。治疗包括手术切除。新发现:MRI 是随访的首选方法。由于晚期复发,需要进行长达 10 年的长期随访。

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