J Korean Neurosurg Soc.  2010 Sep;48(3):263-267. 10.3340/jkns.2010.48.3.263.

Long-Term Follow-Up Clinical Courses of Cerebellar Hemangioblastoma in von Hippel-Lindau Disease : Two Case Reports and a Literature Review

• Lee SH
• Park BJ
• Kim TS
• Lim YJ

• KMID: 2066816
• DOI: http://doi.org/10.3340/jkns.2010.48.3.263

Abstract

Although cerebellar hemangioblastomas are histopathologically benign, they yield a degree of malignant clinical behavior in long-term follow-up. We present two cases of long-term progression of renal cell carcinoma, which had been diagnosed as renal cysts during treatment for cerebellar hemangioblastoma. A 14-year-old male with von Hippel-Lindau disease was admitted for a cerebellar hemangioblastoma with multiple spinal hemangioblastomas and a renal cyst. After primary total resection of the cerebellar hemangioblastoma, the patient required two further surgeries after 111 and 209 months for a recurrent cerebellar hemangioblastoma. Furthermore, he underwent radical nephrectomy as his renal cyst had progressed to renal cell carcinoma 209 months after initial diagnosis. A 26-year-old male presented with multiple cerebellar hemangioblastomas associated with von Hippel-Lindau disease and accompanied by multiple spinal hemangioblastomas and multiple cystic lesions in the liver, kidney, and pancreas. He underwent primary resection of the cerebellar hemangioblastoma in association with craniospinal radiation for multiple intracranial/spinal masses. Unexpectedly, a malignant glioma developed 83 months after discovery of the cerebellar hemangioblastoma. At the same time, renal cell carcinoma, which had developed from an initial renal cyst, was diagnosed, and a radical nephrectomy was performed. In the view of long term clinical course, cerebellar hemangioblastoma associated with von Hipple-Lindau disease may redevelop even after primary total resection. In addition, associated lesions such as renal cysts may also progress to malignancy after the passing of a sufficient length of time.

Keyword

Cerebellar hemangioblastoma; von Hippel-Lindau disease; Renal cyst; Renal cell carcinoma

MeSH Terms

Adolescent
Adult
Carcinoma, Renal Cell
Follow-Up Studies
Glioma
Hemangioblastoma
Humans
Kidney
Liver
Male
Nephrectomy
Pancreas
von Hippel-Lindau Disease