title: [A case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis (author's transl)].
abstract: We presented a case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis. We have not found a hemangioblastoma with these associations, as far as we have seen in the literature. 36-year-old male was admitted with complaints of nausea, vomiting and ataxic gait in June, 1970. On admission, the examination revealed no evidence of increased intracranial pressure except for elevated CSF pressure by lumbar puncture and incoordination. The peripheral blood count disclosed slight erythrocythemia. Vertebral angiography revealed a vascular lesion of 2.0 cm in diameter situated almost in the midline of caudal cerebellum receiving its blood supply from the right posterior inferior cerebellar artery. In addition, a right vertebro-occipital anastomosis was visualized. Plain reoentgenograms showed persistent metopic suture and spina bifida occulta of C 5 - 6. After admission, installation of Ommaya reservoir and decompressive suboccipital craniectomy were performed, and a thyroid papillary adenocarcinoma was totally removed. After discharge, he had been well for two years until a month previously to the second admission, when he commenced to have again headache, nausea, and vomiting with ataxic gait. Vertebral angiography showed the tumor enlarged in size measuring 4.0 X 5.0 cm and the tumor stain was more irregular and less homogenous than 3 years before. Brain scan revealed an increased uptake in the midline of the posterior fossa. After readmission, in April, 1973, he gradually developed dysphagia, disturbance of articulation and inactivity of mentality and died from pneumonia in October, 1974. Autopsy revealed a vascular tumor originated from the medial portion of the right cerebellum and the tumor showed multiple cyst formation in the rostral part in contrast to the caudal solid mass. Histologically the tumor tissue was composed of capillaries supported by fine argyrophilic fibers, large clear interstitial cells containing lipid granules and hemosiderin pigment. Carcinoma of the right lobe of the thyroid was found with metastasis to the bone marrow, lungs and anterior cervical lymphnodes and lymphnodes at the left supraclavicular angle. Bone marrow showed marked erythropoiesis. The case reported here provides an evidence to suggest that there is more than a random relationship between hemangioblastoma, dysraphic state and thyroid carcinoma. The other association, the vertebrooccipital anastomosis may result from the enhanced demand of blood supply by hemangioblastoma but this speculation needs further examination.
mesh_terms: D000015:Abnormalities, Multiple; D000230:Adenocarcinoma / Q000150:complications*; D000328:Adult; D002528:Cerebellar Neoplasms / Q000150:complications*; D005402:Fistula / Q000150:complications; D006394:Hemangiosarcoma / Q000150:complications*; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary; D009778:Occipital Lobe / Q000098:blood supply; D011086:Polycythemia / Q000150:complications; D012886:Skull / Q000002:abnormalities; D016135:Spinal Dysraphism / Q000150:complications; D013964:Thyroid Neoplasms / Q000150:complications*; D014711:Vertebral Artery