Lasso Betancor C E, Vázquez Rueda F, Vargas Cruz V, Ayala Montoro J, Ruiz Hierro C, Gómez Beltrán O
Servicio de Cirugía Pediátrica, Hospital Universitario Reina Sofía, Córdoba.
Cir Pediatr. 2011 Aug;24(3):184-7.
The infantile myofibromatosis is a rare pathology usually affecting children under 2 years. More than half are congenital, located in head and neck in a 30% of the cases. Myofibroma is a benign mesenchymal tumor that appear an isolated lesion or multiple, with or without visceral involvement. Case 1: A newborn girl that presents lump in scalp suspected of encefalocele. Ultrasound and RM: solid and heterogeneous mass 4 x 2 cm with increased peripheral vascularisation. Case 2: A 7-month-old baby boy with lump in right occipital zone detected at 4 months. Ultrasound and RM: injury 1 x 1.5 cm in head esplenio muscle suggestive of hemangioma.
After surgical total resection, the histological study and immunohistochemical identified the lesion as myofibroma. The evaluation of disease dissemination was normal and the recovery of the patients was favorable. The differential diagnosis of myofibroma must be carried out with other mesenchymal tumors and with non neoplastic injuries characteristics of the region affects. The visceral location and multiple forms worsen the prognosis, so is important do an exhaustive study. Treatment is conservative surgery, but if there is no risk to the child, the possibility of spontaneous regression makes the abstention therapeutic a correct alternative.
婴儿肌纤维瘤病是一种罕见的病理情况,通常影响2岁以下儿童。超过半数为先天性,30%的病例位于头颈部。肌纤维瘤是一种良性间叶性肿瘤,可表现为孤立性病变或多发性病变,可伴有或不伴有内脏受累。病例1:一名新生儿女孩,头皮出现肿块,怀疑为脑膨出。超声和磁共振成像:4×2厘米的实性、不均匀肿块,周边血管增多。病例2:一名7个月大男婴,4个月时发现右枕部有肿块。超声和磁共振成像:头夹肌内1×1.5厘米的损伤,提示为血管瘤。
手术全切后,组织学研究和免疫组化确定病变为肌纤维瘤。疾病播散评估正常,患者恢复良好。肌纤维瘤的鉴别诊断必须与其他间叶性肿瘤以及该区域特有的非肿瘤性损伤进行鉴别。内脏部位和多发形式会使预后变差,因此进行详尽的研究很重要。治疗为保守手术,但如果对患儿无风险,自发消退的可能性使得放弃治疗成为一种正确选择。