Shuangshoti S, Chayapum P, Suwanwela N, Suwanwela C
Department of Pathology, Chulalongkorn University, Bangkok, Thailand.
Br J Ophthalmol. 1988 Sep;72(9):713-9. doi: 10.1136/bjo.72.9.713.
A case is reported of unilateral proptosis of the left eyeball as a unique clinical presentation of primary angiosarcoma of the skull involving the sphenoid and frontal bones and roof of the orbit on the left side of a 32-year-old man. The intraosseous, extradural non-encapsulated, and friable tumour contained cytoplasmic factor VIII-related antigen. After complete removal of the tumour the patient has been well for 10 months of the follow-up period. This case brings the total number of reported primary angiosarcomas of the cranium to nine. All patients were male and most of them were young. The average age was 24 years. The frontal bone seems to be the site of predilection for cranial angiosarcomas. Surgical extirpation, radiation, or combined surgical removal and radiation have been used for treating angiosarcoma of the skull with variable results. It is suggested that angiosarcoma of the skull has a worse prognosis than angiosarcoma of bones other than the cranium because the patient may die of secondary neoplastic involvement of the brain.
报道了一例32岁男性原发性颅骨血管肉瘤的独特临床表现，即左侧眼球单侧突出，该肿瘤累及左侧蝶骨、额骨及眶顶。骨内、硬膜外、无包膜且质脆的肿瘤含有细胞质VIII因子相关抗原。肿瘤完全切除后，患者在随访的10个月中情况良好。该病例使已报道的颅骨原发性血管肉瘤总数增至9例。所有患者均为男性，且大多数为年轻人。平均年龄为24岁。额骨似乎是颅骨血管肉瘤的好发部位。手术切除、放疗或手术切除联合放疗已用于治疗颅骨血管肉瘤，效果不一。提示颅骨血管肉瘤的预后比颅骨以外骨骼的血管肉瘤更差，因为患者可能死于脑的继发性肿瘤累及。
