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脂肪母细胞瘤:临床特征、治疗和结局。

Lipoblastoma: clinical features, treatment, and outcome.

机构信息

Department of General Surgery, Ripas Hospital, Bandar Seri Begawan, BA1710, Brunei.

出版信息

World J Surg. 2010 Jul;34(7):1517-22. doi: 10.1007/s00268-010-0466-8.

DOI:10.1007/s00268-010-0466-8
PMID:20151124
Abstract

BACKGROUND

Lipoblastoma is a rare, benign, encapsulated tumor arising from embryonic white fat. On histology they typically contain variably differentiated adipocytes, primitive mesenchymal cells, myxoid matrix, and fibrous trabeculae. The tumor occurs primarily in infancy and early childhood. It often occurs in the extremities and trunk, and rarely develops in the head and neck and other sites.

METHODS

Ten cases of histopathologically proven lipoblastoma presenting to our hospital during a 6-year period (2003-2008) were reviewed retrospectively for their clinical presentations, treatment, postoperative outcome, and follow-up.

RESULTS

There were five males and five females ranging in age from 6 months to 20 years. The commonest presentation was a painless rapidly growing mass. Tumors occurred in an extremity (n = 5), head and neck (n = 3), trunk (n = 1), and retroperitoneum (n = 1). Preoperative diagnosis was accurate in only one case. The largest tumor measuring 25-cm x 20-cm x 7-cm and weighing 1.9 kg was excised from the retroperitoneum. All patients underwent complete surgical excision. Patient follow-up period ranging from 9 to 76 months showed no recurrences and no metastases.

CONCLUSIONS

Lipoblastoma behaves benignly, occurs in both superficial and deep sites, and occasionally attains large size. Complete surgical excision is the treatment of choice and long-term follow-up is required because there is a reported tendency for these tumors to recur.

摘要

背景

脂肪瘤是一种罕见的良性、包膜肿瘤,来源于胚胎白色脂肪。组织学上,它们通常包含不同程度分化的脂肪细胞、原始间充质细胞、黏液样基质和纤维小梁。肿瘤主要发生在婴儿和幼儿期。它常发生在四肢和躯干,很少发生在头颈部和其他部位。

方法

回顾性分析了我院 6 年间(2003-2008 年)经病理证实的 10 例脂肪瘤患者的临床资料,包括临床表现、治疗、术后结果和随访情况。

结果

男性 5 例,女性 5 例,年龄 6 个月至 20 岁。最常见的表现为无痛性快速生长的肿块。肿瘤发生于四肢(n=5)、头颈部(n=3)、躯干(n=1)和腹膜后(n=1)。术前诊断准确者仅 1 例。最大肿瘤位于腹膜后,大小为 25cm×20cm×7cm,重量为 1.9kg,完整切除。所有患者均行完全手术切除。随访 9-76 个月,无复发和转移。

结论

脂肪瘤呈良性表现,可发生于表浅和深部部位,偶尔可达巨大体积。完整手术切除是首选治疗方法,需要长期随访,因为这些肿瘤有复发的倾向。

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