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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.
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PMID:[A case of enchondroma of the skull base--a manifestation of generalized chondromatosis (author's transl)]. 55 59

The patient was a 52-year-old woman who was initially admitted to our hospital with a recent history of diplopia, ptosis and visual impairment on the right, adding to 6 years history of the right orbital pain and temporal headache. She had gradually developed pain and fatigue of the right knee since childhood and had been suffered from multiple subcutaneous tumor in the both hands and the right foot from youth onward. On neurological examination, she has had right exphthalmos and 3rd and 6th cranial nerve palsies on the right. Skull X-ray and CT scan showed parasellar calcified mass on the right. In chromosome examination, the inversion of the No. 1 chromosome was disclosed by the Q- and C-band dyeing of her leucocytes and skin culture. Cavernous hemangioma of her skin lesion and enchondroma of the skeletal lesion were diagnosed by X-ray examination and histological specimen, indicating so-called Maffucci's syndrome. One year and a half later, she was readmitted to our institution, because of progressive visual loss on the right and left hemiparesis. Malignant change of the parasellar lesion was suspected by the clinical course and neuroradiological findings and was confirmed histologically by the second peration. During postoperative course, intratumoral hemorrhage had occurred twice and the patient died, and postmortem examination confirmed her clinical affair. This is the case which was clarified malignant transformation of enchondroma in the parasellar region, and is the first case verified the chromosome abnormalities in Maffucci's syndrome.
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PMID:[Maffucci's syndrome with intracranial manifestation and chromosome abnormalities--a case report]. 370 42