Extensive replacement of spinal cord and brainstem by hemangioblastoma in a case of von Hippel-Lindau disease

Amyn Mohammed Rojiani, K. Elliott, K. Dorovini-Zis

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

We report an extremely unusual case of von Hippel-Lindau disease, characterized by almost total replacement of the spinal cord and most of the medulla by capillary hemangioblastoma (CHB). A 34-year-old female presented 20 years earlier with signs of lower limb weakness. She developed progressive quadriplegia despite multiple surgical attempts to remove separate CHBs from the midthoracic and cervical spinal cord. Spinal cord lesions continued to develop as did multiple cerebellar CHBs. She eventually became respirator-dependent and died following severe upper gastrointestinal hemorrhage. At autopsy there was extensive replacement of spinal cord and medulla by CHB as well as multiple solid and cystic cerebellar tumors. Both kidneys were enlarged and largely replaced by renal cell carcinomas and simple cysts. Additionally she had small pancreatic cysts and microscopic pulmonary hamartomas were observed. There was no evidence of retinal lesions clinically or at autopsy. Light microscopic and immunohistochemical studies of the lesions are presented. The early onset of this disease in a female, with such extensive spinal cord involvement, in addition to other CNS and visceral manifestations of von Hippel-Lindau disease, is unusual and has not been previously reported.

Original languageEnglish (US)
Pages (from-to)297-302
Number of pages6
JournalClinical Neuropathology
Volume10
Issue number6
StatePublished - Dec 1 1991
Externally publishedYes

Fingerprint

Hemangioblastoma
von Hippel-Lindau Disease
Brain Stem
Spinal Cord
Autopsy
Cerebellar Neoplasms
Pancreatic Cyst
Quadriplegia
Gastrointestinal Hemorrhage
Hamartoma
Mechanical Ventilators
Renal Cell Carcinoma
Cysts
Lower Extremity
Kidney
Light
Lung

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology

Cite this

Extensive replacement of spinal cord and brainstem by hemangioblastoma in a case of von Hippel-Lindau disease. / Rojiani, Amyn Mohammed; Elliott, K.; Dorovini-Zis, K.

In: Clinical Neuropathology, Vol. 10, No. 6, 01.12.1991, p. 297-302.

Research output: Contribution to journalArticle

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