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1例颅底内生软骨瘤——全身性软骨瘤病的一种表现(作者译)

[A case of enchondroma of the skull base--a manifestation of generalized chondromatosis (author's transl)].

作者信息

Imagawa K, Toda I, Hayashi M, Asai A, Nomura T

出版信息

No Shinkei Geka. 1977 May;5(5):457-63.

PMID:559959
Abstract

Cartilaginous tumors arising from the skull or intracranial structures are infrequently seen. There have been 112 such cases reported. They are most commonly found in the middle fossa and the parasellar region. They arise from cartilaginous rests in the base of the skull, particularly around the basal synchondroses. Occasional cases of intracranial growths also show lesions in peripheral bones. The authors have encountered a case in which chondroma was a manifestation of generalized chondromatosis. The patient was a 25-year-old male, who complained of diplopia in May 1975. He noted ptosis in his left eyelid which gradually progressed over a 2 months period to complete ptosis. When he was admitted in July 1975 to Nagoya National Hospital, the neurologic signs revealed a palsy of the IIIrd cranial nerve on the left side. The patient has been suffering from generalized enchondromatosis during a period of 20 years. Roentgenograms revealed extensive areas of bone destruction and pathological calcification of the phalanx, ribs, hip bone, femur, tibia, ulna and radius. Roentgenograms of the skull revealed calcification on the left middle fossa. After a left carotid angiography, an osteoplastic craniotomy was performed on August 5, 1975. The tumor was located in the extradural space of the middle cranial fossa. After incision of the capsule, gelatinous semisolid tissue was removed piecemeal. Its total removal proved to be impossible. Histological examination of this tumor revealed enchondroma.

摘要

起源于颅骨或颅内结构的软骨肿瘤较为少见。已有112例此类病例报告。它们最常出现在中颅窝和鞍旁区域。肿瘤起源于颅底的软骨残余,尤其是围绕基底软骨结合处。偶尔也有颅内生长的病例在外周骨中出现病变。作者遇到过一例软骨瘤是全身性软骨瘤病表现的病例。患者为一名25岁男性,1975年5月主诉复视。他注意到左眼睑下垂,在2个月内逐渐加重直至完全下垂。1975年7月他入住名古屋国立医院时,神经系统体征显示左侧动眼神经麻痹。该患者患有全身性内生软骨瘤病已达20年。X线片显示指骨、肋骨、髋骨、股骨、胫骨、尺骨和桡骨有广泛的骨质破坏和病理性钙化区域。颅骨X线片显示左中颅窝有钙化。1975年8月5日进行了左侧颈动脉血管造影后,实施了整复性颅骨切开术。肿瘤位于中颅窝硬膜外间隙。切开包膜后,将胶冻状半固体组织逐块切除。但事实证明无法将其完全切除。对该肿瘤进行组织学检查显示为内生软骨瘤。

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