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婴儿纤维性错构瘤:一项意大利多机构研究经验

Fibrous hamartoma of infancy: an Italian multi-institutional experience.

作者信息

Carretto Elena, Dall'Igna Patrizia, Alaggio Rita, Siracusa Fortunato, Granata Claudio, Ferrari Andrea, Cecchetto Giovanni

机构信息

Division of Paediatric Surgery, Department of Paediatrics, University of Padua, Padua, Italy.

出版信息

J Am Acad Dermatol. 2006 May;54(5):800-3. doi: 10.1016/j.jaad.2005.11.1076.

DOI:10.1016/j.jaad.2005.11.1076
PMID:16635660
Abstract

BACKGROUND

Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative.

OBJECTIVE AND METHODS

The clinical features and treatment results of 18 children with FH are described.

RESULTS

Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery.

LIMITATIONS

The results did not reach statistical significance due to difficulties in collecting cases.

CONCLUSIONS

FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent.

摘要

背景

婴儿纤维性错构瘤(FH)是一种良性间叶组织肿瘤,表现为浅表肿块。完整切除可治愈。

目的和方法

描述了18例FH患儿的临床特征及治疗结果。

结果

局部切除是最常见的手术方式。10例患者手术切除彻底,6例有镜下残留病灶;所有患者在诊断后2至49个月均存活,无疾病证据。1例因肉眼残留病灶接受局部再次切除(并因同步发生的韧带样纤维瘤病接受化疗)的患者,诊断后83个月情况良好;最后1例大阴唇病变患者仅接受了活检,情况良好,等待整形手术。

局限性

由于病例收集困难,结果未达到统计学意义。

结论

FH应通过完整切除进行治疗;根据我们的经验,非根治性切除也能实现治愈。由于总体预后良好,应避免采取激进的治疗方法。

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