著明な頭蓋内・外伸展を示した新生児頭蓋筋膜炎の1例

Cranial Fasciitis of a Neonatal Case with Massive Intra- and Extracranial Extension

I．はじめに

　1980年LauerとEnzingerは乳児の頭蓋に好発する良性腫瘍性病変をcranial fasciitis of child-hood（以後CFCと記す）としてnodular fasciitis（以後NFと記す）から分離し，新たな疾患概念として提唱した^14）．これは成人に好発するNFと類似する線維芽細胞増殖が主体でその中に炎症細胞浸潤などを認める組織像を呈するが，頭蓋骨骨膜や頭皮深部筋層より発生し，急速に増大する稀な疾患である．

　今回われわれは出生時より頭部に巨大な腫瘤を認め，硬膜及び硬膜下腔まで伸展するCFCの1例を経験したので文献的考察を加えて報告する．

We report a neonatal male case of cranial fasciitis in childhood. He was born with a large skull tumor inhis left fronto-parietal region. The day after his birth, he was admitted to our hospital. On inspection, themass was firm, non mobile, and had two humped peaks. The size of the mass was 7×5×4cm, and it waslocated on the left coronal suture and fontanelle. The overlying skin was normal. Skull X-rays revealedosteolytic defect and linear bone remnant skirted the outer rim of the tumor. CT scan showed a slightlyhigh-density large frontal mass with a low density area in the center of it and marked enhancement. MRimages revealed a large mass with massive intra- and extracranial extension. Cerebral angiography showedthe remarkable vascularization of the tumor. Preoperatively, the branches of the external carotid arterywere partly embolized. On surgery, the profusely bleeding, elastic hard, yellowish-white mass arising fromthe dura was partially removed with the surrounding bone. A small mass remained subdurally. The bound-ary of the mass and the dura was not well demarcated. After the operation, no neurological deficits wereseen. 16 months after the surgery, MRI revealed the unfolded brain with no evidence of tumor. Histologic-al examination showed characteristic proliferation of spindle-shaped fibroblasts embedded in a collagenousstroma. Inflammatory lymphocytes and acidphils were also noted. Electron micrograph revealed a tumorcell rich in rough endoplasmic reticulum and nuclei with numerous indentations. Immunostaining con-firmed that these cells were positive for vimentin but negative for smooth muscle actin, GFAP, S-100 pro-tein, and desmin. No recurrence of this tumor has been detected at four years of follow-up.