[Combined medullary hemangioblastoma and syringomyelia in a patient with von Hippel-Lindau disease: pathological study].
A patient presented with what was clinically diagnosed as a solitary medullary haemangioblastoma. One year later she was operated from a cerebellar haemangioblastoma. The neuro-radiologic findings showed all the characteristics of von Hippel-Lindau's disease, except the retinal haemangioblastoma. The patient died five years later as a result of deglutition problems with aspiration pneumonia. Post-mortem examination showed that the tumor level was in the posterior right side of the dorsal spinal cord. A syringomyelic cavity spread above the tumor up to the medulla - without communicating with the fourth ventricle and beneath the tumor down to the lowest part of the thoracic spinal cord. A large venous pia mater stasis and a thick glial wall of the cavity - mostly below the tumor - suggested a lack of reabsorption of liquids produced by the tumor.
Pou Serradell
A
A
Service de Neurologie, Hôpital N. S. del Mar, Université Autonome, Barcelone, Espagne.
Mares Segura
R
R
Lamarca Ciuro
J L
JL
D000798:Angiomatosis / Q000150:complications*; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology; D005260:Female; D006394:Hemangiosarcoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D008526:Medulla Oblongata; D008875:Middle Aged; D013120:Spinal Cord Neoplasms / Q000150:complications* / Q000473:pathology; D013595:Syringomyelia / Q000150:complications* / Q000473:pathology; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000473:pathology
Hémangioblastome médullaire associé a une syringomyélie dans un cas de maladie de von Hippel-Lindau: étude pathologique.