A consecutive series of 30 cases of extracranial medulloblastoma metastases in adults is analysed. The majority of the patients were males with a 3:1 male/female ratio. Bone was the most frequent site of metastases in adults (77%) and children (78%), followed by lymph nodes (33%) in both children and adults. Lung metastases were more common in adults (17%), but liver metastases occurred more frequently in children (15%). Case report A 33 year old male presented with severe headache and vomiting. On physical examination, he showed bilateral papilloedema and severe ataxia and incoordination. Brain computerised tomography (CT) demonstrated a large mass, 5 cm in diameter, in the vermis and right cerebellar lobe. Suboccipital craniotomy was performed and a large, soft greyish cerebellar tumour, extending into the roof of the fourth ventricle, was totally excised. The tumour was found to be a medulloblastoma. A post-operative CT scan did not reveal any residual tumour. The patient subsequently received radiation therapy: whole brain-3600 RAD, posterior fossa-5200 RAD, total spine-3000 RAD. On discharge, the patient had no neurological deficit and remained symptomless.Twenty one months after corrective surgery, however, low back pain and micturition difficulties appeared, and within two weeks, urinary retention developed, with paraparesis and loss of sphincter control. Myelography showed a partial block at T7 and a complete block at T3, with multiple intradural lesions along the dural sac. CT scan revealed a hypodense area in the roof of the fourth ventricle, containing a discrete area with some contrast enhancement. Bone radio-isotope scan showed increased absorption in thoracic vertebrae TI-TIo, in most ribs, and in the sternum, pelvis and both femurs. Liver radioisotope scan revealed several foci of increased absorption, and a chest radiograph demonstrated multiple pulmonary metastases. Bone marrow biopsy revealed an infiltration of numerous tumour cells diagnosed as medulloblastoma, with large areas of necrosis and fibrosis. The patient received a course of radiation therapy and a course of C-MOPP. However, his condition gradually deteriorated and he died two months later.To the best of our knowledge, although questions of incidence of extracranial metastases of medulloblastoma, location, mode of spread and survival have been discussed in previous reviews, none of them have separately discussed the problem of extracranial metastases of medulloblastoma in adults.
Results and commentsIn 1930 Bailley5 demonstrated that medulloblastomas tended to seed along cerebrospinal fluid (CSF) pathways, and in the same year Wohlvill6 was the first to draw attention to the occurrence of systemic metastasis of a medulloblastoma. Since then, metastases within the central nervous system 80 on 7 May 2018 by guest. Protected by copyright.
