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头颈部脂肪母细胞瘤:3例报告及文献复习

Head and neck lipoblastomas: Report of 3 cases and review of the literature.

作者信息

Merzouqi Boutaina, Laachoubi Mohammed, Oukessou Youssef, Mahtar Mohammed

机构信息

ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco.

ENT Department, Face and Neck Surgery, Hospital August, 20'1953, University Hospital Center IBN ROCHD, Casablanca, Morocco.

出版信息

Int J Surg Case Rep. 2021 Jul;84:106050. doi: 10.1016/j.ijscr.2021.106050. Epub 2021 Jun 4.

Abstract

INTRODUCTION

Lipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities and considered as a rare cause of a pediatric head and neck masses. The aim of this study is to shed light on lipoblastomas as a differential diagnosis of rapidly growing soft fatty masses of children in neck and head area.

PATIENTS AND METHODS

A retrospective review of 3 patients with lipoblastoma, underwent Surgical resection (case 1 and 2) by cervical approach. The third patient with a facial lipoblastoma was not operated due to the high risk of facial paralysis. Review of literature, diagnostic methods and genetics of lipomatous tumors are discussed.

RESULTS

Complete surgical excision via a cervical approach demonstrated irregular lobules of immature fat cells separated by a loose, myxoid connective tissue. Histology analysis confirmed the diagnosis of lipoblastoma.

DISCUSSION

Lipoblastoma is a rare childhood tumor, even rarer in head and neck area. The pathogenesis is unknown, though it is believed to arise from altered embryogenesis of human white fat and genetic predisposition, as chromosome 8 abnormalities may be implicated in the development of lipoblastoma. The presumptive diagnosis is performed by imaging. The most important differential diagnosis of lipoblastoma is myxoid liposarcoma. The mainstay of treatment is complete non-mutilating resection of the tumor to avoid recurrence.

CONCLUSION

Lipoblastoma should be suspected in case of heterogeneous fatty tumor in head and neck area, and included as a differential diagnosis of cervical masses in children younger than 3 years. The mainstay of treatment is complete surgical excision with a good prognosis.

摘要

引言

脂肪母细胞瘤是一种罕见的良性肿瘤,起源于胚胎白色脂肪,发生于儿童早期。它通常发生在四肢,被认为是小儿头颈部肿块的罕见病因。本研究的目的是阐明脂肪母细胞瘤可作为儿童头颈部快速生长的柔软脂肪性肿块的鉴别诊断。

患者与方法

对3例脂肪母细胞瘤患者进行回顾性研究,其中病例1和病例2经颈部入路接受了手术切除。第三例面部脂肪母细胞瘤患者因面瘫风险高未进行手术。文中讨论了脂肪母细胞瘤的文献回顾、诊断方法及遗传学。

结果

经颈部入路的完整手术切除显示,不成熟脂肪细胞呈不规则小叶状,被疏松的黏液样结缔组织分隔。组织学分析证实为脂肪母细胞瘤。

讨论

脂肪母细胞瘤是一种罕见的儿童肿瘤,在头颈部更为罕见。其发病机制尚不清楚,不过据信它源于人类白色脂肪胚胎发育异常和遗传易感性,因为8号染色体异常可能与脂肪母细胞瘤的发生有关。通过影像学进行初步诊断。脂肪母细胞瘤最重要的鉴别诊断是黏液样脂肪肉瘤。治疗的主要方法是完整地切除肿瘤且不造成毁容,以避免复发。

结论

头颈部出现异质性脂肪肿瘤时应怀疑脂肪母细胞瘤,并将其作为3岁以下儿童颈部肿块的鉴别诊断之一。治疗的主要方法是完整的手术切除,预后良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c3e6/8219841/04879bc7320c/gr1.jpg

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