Spinal leptomeningeal hemangioblastomatosis occurring without craniospinal surgery in von Hippel-Lindau disease.

Journal: BMJ Case Reports
Treatment Used: Craniospinal Radiotherapy
Number of Patients: 1
Published:
MediFind Summary

Summary: This case report describes a patient with Von Hippel-Lindau (VHL) disease with spinal leptomeningeal hemangioblastomatosis treated with craniospinal radiotherapy.

Conclusion: A patient with Von Hippel-Lindau disease with spinal leptomeningeal hemangioblastomatosis treated with craniospinal radiotherapy stabilized.

Abstract

Hemangioblastomatosis represents an unusual and malignant leptomeningeal dissemination of hemangioblastoma (HB). It has been reported in patients with sporadic HB or von Hippel-Lindau (VHL) disease. Hemangioblastomatosis had been reported following resection of a primary HB lesion in all cases except one patient with a sporadic HB. We present a patient with VHL with several HBs at the brainstem, cerebellum, pituitary stalk and retina who developed spinal hemangioblastomatosis without previous craniospinal surgery. A whole spine MRI showed the spinal dissemination from the primary lesions. The patient received craniospinal radiotherapy due to the extensive spinal leptomeningeal dissemination and multiple HBs. MRI performed 12 months after the radiotherapy showed stability of the lesions.

Authors
Orlando De Jesus, Julio Rosado Philippi

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