Kotiloğlu E, Göğüş S, Ruacan S, Akyüz C, Büyükpamukçu M, Srialioğlu F
Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.
Turk J Pediatr. 1996 Oct-Dec;38(4):527-32.
A three-month-old boy was admitted for a slowly progressing nodule in his right chin, first recognized at birth and recently accompanied by facial paralysis. It was found to be a soft tissue mass arising in subcutaneous tissue and extending deep into the temporal muscle, causing temporal bone erosion without infiltrating the dura. Initially interpreted as mesenchymal chondrosarcoma, combined chemotherapy (PULSE-VAC) was given. Eighteen months later an additional nodule developed in the paraspinal skin. Evaluation of both lesions showed vimentin and alpha-smooth-muscle-action positivity. As the final diagnosis was multicentric infantile myofibromatosis, the child was followed up without therapy. Thirty months later, multiple osteolytic lesions appeared on the skull and long bones of the extremities. Some lesions remained stable and some regressed during two years of follow-up.
一名三个月大的男婴因右下巴处一个缓慢生长的结节入院，该结节出生时就已发现，最近伴有面部麻痹。发现这是一个起源于皮下组织并深入颞肌的软组织肿块，导致颞骨侵蚀但未侵犯硬脑膜。最初被诊断为间叶性软骨肉瘤，给予联合化疗（PULSE-VAC）。18个月后，脊柱旁皮肤出现了另一个结节。对两个病灶的评估显示波形蛋白和α-平滑肌肌动蛋白呈阳性。最终诊断为多中心婴儿肌纤维瘤病，该患儿未接受治疗，进行随访观察。30个月后，颅骨和四肢长骨出现多处溶骨性病变。在两年的随访期间，一些病灶保持稳定，一些病灶有所消退。
