Department of Pediatric Oncology, Institut Curie, Paris, France.
Pediatr Blood Cancer. 2012 Jul 15;59(1):115-20. doi: 10.1002/pbc.23387. Epub 2011 Oct 28.
Infantile myofibromatosis is characterized by proliferation of benign fibrous tumors arising in skin, subcutaneous tissue, muscle, or bone. Solitary and multicentric forms are described. Few reports are available in the pediatric population.
To improve the knowledge of this rare tumor in infants, the authors present a series of all cases of infantile myofibromatosis treated in their institution over a 9-year period in order to propose treatment guidelines based on their experience and a review of the literature.
The authors report a series of 9 cases, 8 solitary forms and 1 multicentric form with visceral involvement treated from 2000 to 2009. Median age was 10 months (range: 2 days-14 years). Six patients with solitary forms underwent primary surgical resection leading to remission. Only biopsy was performed in 1 case, followed by tumor regression with no recurrence. The last patient with a solitary form was treated by chemotherapy and then surgery allowing remission. The patient with a multicentric form presented complete regression of tumors after 1 year of vinblastine and methotrexate combination chemotherapy.
Infantile myofibromatosis is a rare soft tissue tumor mainly concerning infants. Surgery is the treatment of choice for solitary forms when excision is possible. Close follow-up may be proposed in the case of inoperable sites. In multicentric life-threatening forms, chemotherapy promotes tumor regression and the vinblastine and methotrexate combination is effective with few long-term adverse effects.
婴儿肌纤维瘤病的特征是良性纤维瘤在皮肤、皮下组织、肌肉或骨骼中生长。描述了单发和多中心形式。在儿科人群中，很少有报道。
为了提高对这种在婴儿中罕见的肿瘤的认识，作者介绍了他们所在机构在 9 年期间治疗的所有婴儿肌纤维瘤病病例系列，以便根据他们的经验和文献复习提出治疗指南。
作者报告了一系列 9 例病例，8 例为单发形式，1 例为多中心形式伴内脏受累，治疗时间为 2000 年至 2009 年。中位年龄为 10 个月（范围：2 天-14 岁）。6 例单发形式的患者行原发性手术切除后缓解。1 例仅行活检，随后肿瘤消退无复发。最后 1 例单发形式的患者接受化疗和手术治疗后缓解。1 例多中心形式的患者在接受长春新碱和甲氨蝶呤联合化疗 1 年后肿瘤完全消退。
婴儿肌纤维瘤病是一种罕见的软组织肿瘤，主要发生在婴儿中。当切除可行时，手术是单发形式的首选治疗方法。对于无法手术的部位，可建议密切随访。在危及生命的多中心形式中，化疗可促进肿瘤消退，长春新碱和甲氨蝶呤联合治疗有效，且长期不良反应较少。
