Noguchi O, Kuroiwa M, Kogure S, Kohno N, Yoshida K, Zama A, Tamura M
Department of Neurosurgery, Kiryu Kohsei General Hospital, Gunma, Japan.
No Shinkei Geka. 1999 Feb;27(2):163-9.
We report a neonatal male case of cranial fasciitis in childhood. He was born with a large skull tumor in his left fronto-parietal region. The day after his birth, he was admitted to our hospital. On inspection, the mass was firm, non mobile, and had two humped peaks. The size of the mass was 7 x 5 x 4 cm, and it was located on the left coronal suture and fontanelle. The overlying skin was normal. Skull X-rays revealed osteolytic defect and linear bone remnant skirted the outer rim of the tumor. CT scan showed a slightly high-density large frontal mass with a low density area in the center of it and marked enhancement. MR images revealed a large mass with massive intra- and extracranial extension. Cerebral angiography showed the remarkable vascularization of the tumor. Preoperatively, the branches of the external carotid artery were partly embolized. On surgery, the profusely bleeding, elastic hard, yellowish-white mass arising from the dura was partially removed with the surrounding bone. A small mass remained subdurally. The boundary of the mass and the dura was not well demarcated. After the operation, no neurological deficits were seen. 16 months after the surgery, MRI revealed the unfolded brain with no evidence of tumor. Histological examination showed characteristic proliferation of spindle-shaped fibroblasts embedded in a collagenous stroma. Inflammatory lymphocytes and acidophils were also noted. Electron micrograph revealed a tumor cell rich in rough endoplasmic reticulum and nuclei with numerous indentations. Immunostaining confirmed that these cells were positive for vimentin but negative for smooth muscle actin, GFAP, S-100 protein, and desmin. No recurrence of this tumor has been detected at four years of follow-up.
我们报告一例儿童期新生儿男性颅骨筋膜炎病例。他出生时左额顶区有一个巨大的颅骨肿瘤。出生后第二天，他被收治入院。检查发现肿块质地坚硬，不可移动，有两个驼峰状凸起。肿块大小为7×5×4厘米，位于左冠状缝和囟门处。肿块上方的皮肤正常。颅骨X线片显示溶骨性缺损，肿瘤外缘有线性骨残留。CT扫描显示额叶有一个密度略高的大肿块，中心有一个低密度区，并有明显强化。磁共振成像显示一个巨大肿块，有大量颅内和颅外延伸。脑血管造影显示肿瘤有明显的血管化。术前，对颈外动脉分支进行了部分栓塞。手术中，从硬脑膜长出的大量出血、质地坚韧、黄白色的肿块连同周围骨质被部分切除。硬脑膜下仍残留一小部分肿块。肿块与硬脑膜的边界不清。术后未发现神经功能缺损。术后16个月，磁共振成像显示大脑结构展开，无肿瘤迹象。组织学检查显示，在胶原基质中有特征性的梭形成纤维细胞增生。还可见炎性淋巴细胞和嗜酸性粒细胞。电子显微镜检查显示肿瘤细胞富含粗面内质网，细胞核有许多凹陷。免疫染色证实这些细胞波形蛋白呈阳性，但平滑肌肌动蛋白、胶质纤维酸性蛋白、S-100蛋白和结蛋白呈阴性。随访四年未发现该肿瘤复发。
