Inenaga C, Morii K, Tamura T, Tanaka R, Takahashi H
Department of Pathology, Brain Research Institute, Niigata University, Japan.
Acta Neurochir (Wien). 2003 Jul;145(7):593-7; discussion 597. doi: 10.1007/s00701-003-0059-5.
It is known that, although rare, mesenchymal chondrosarcoma can originate intracranially. However, no such malignant tumour has been described in the sellar region.
We report a case of mesenchymal chondrosarcoma in a 21-year-old man who presented with double vision, right blepharoptosis and facial pain. Upon initial admission, no endocrinological abnormalities were found, and computed tomography and magnetic resonance imaging revealed a mass with calcification in the sella and right cavernous sinus.
For this malignant tumour, three surgical resections, two sessions of gamma-knife radiosurgery, one session of fractional irradiation, and one cycle of chemotherapy were performed, resulting in only brief arrest of the tumour growth. Pathologically, the tumour consisted of undifferentiated small cells of high cellularity, and islands of hyaline cartilage. The undifferentiated small cells showed immunoreactivity for vimentin and ultrastructural features suggesting a mesenchymal origin. Lacunar cells in the islands were immunopositive for S-100 protein and vimentin.
Although malignant tumours in the sellar region are rare, they should be considered in the differential diagnosis of various sellar tumours typified by non-functioning pituitary adenoma, and mesenchymal chondrosarcoma is one possible candidate.
众所周知，间叶性软骨肉瘤虽然罕见，但可起源于颅内。然而，蝶鞍区尚未见此类恶性肿瘤的报道。
我们报告一例21岁男性间叶性软骨肉瘤患者，该患者出现复视、右侧上睑下垂和面部疼痛。初次入院时，未发现内分泌异常，计算机断层扫描和磁共振成像显示蝶鞍和右侧海绵窦有一钙化肿块。
针对该恶性肿瘤，进行了三次手术切除、两次伽玛刀放射外科治疗、一次分次照射和一个周期的化疗，结果仅使肿瘤生长短暂停滞。病理检查显示，肿瘤由高细胞密度的未分化小细胞和透明软骨岛组成。未分化小细胞对波形蛋白呈免疫反应阳性，超微结构特征提示为间叶起源。软骨岛内的陷窝细胞对S-100蛋白和波形蛋白呈免疫阳性。
尽管蝶鞍区恶性肿瘤罕见，但在以无功能垂体腺瘤为代表的各种蝶鞍肿瘤的鉴别诊断中应考虑到，间叶性软骨肉瘤是其中一种可能的类型。
