Brain Tumor Res Treat.  2014 Oct;2(2):96-101. 10.14791/btrt.2014.2.2.96.

Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease

Affiliations
  • 1Department of Neurosurgery, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea. jhyun@catholic.ac.kr

Abstract

Hemangioblastoma (HB) of the central nervous system may occur sporadically or in association with von Hippel-Lindau (VHL) disease. Disseminated HB means malignant spread of the original primary HB without local recurrence at surgically resected site. It has been rarely reported previously, and rarer especially without VHL gene mutation. We report a case of disseminated HB without VHL disease. A 59-year-old man underwent a surgery for total removal of a cerebellar HB. From five years after the surgery, multiple dissemination of HB was identified intracranially and he subsequently underwent cyberknife radiosurgery. The lesions got smaller temporarily, but they soon grew larger. Nine years after the initial surgery for cerebellar HB, he showed severe back pain. His magnetic resonance image of spine revealed intradural extramedullary mass at T6-7 level. Complete surgical removal of the mass was performed and the pathological diagnosis was identical to the previous one. He had no evidence of VHL disease. And there was no recurrence of the tumor at the site of the original operation. The exact mechanism of dissemination is unknown, but the surgeon should be cautious of tumor cell spillage during surgery and prudently consider the decision to perform ventriculo-peritoneal shunt. In addition, continuous follow-up for recurrence or dissemination is necessary for patients even who underwent complete removal of cerebellar HB.

Keyword

Hemangioblastoma; Von Hippel-Lindau disease; Central nervous system

MeSH Terms

Back Pain
Central Nervous System*
Diagnosis
Hemangioblastoma*
Humans
Middle Aged
Radiosurgery
Recurrence
Spine
Ventriculoperitoneal Shunt
von Hippel-Lindau Disease*

Figure

  • Fig. 1 Pre and postoperative magnetic resonance images (MRIs). A: Preoperative gadolinium-enhanced T1-weighted MRI shows strongly enhancing mass involving the cerebellar vermis. B: Postoperative gadolinium-enhanced T1-weighted MRI. Tumor mass is completely removed and postoperative changes are observed.

  • Fig. 2 Gadolinium-enhanced T1-weighted magnetic resonance images of brain. A: Highly enhanced mass abutting the medial margin of the tentorium cerebelli was first noted in 2010. B: The size of tumor was reduced in 2012 (after cyberknife radiosurgery). C: But in 2014, previously noted mass showed enlargement, and newly developed mass was noted near ambient cistern. D: Similar mass was observed at posterior falx in 2012. E: It became smaller after cyberknife radiosurgery. F: Other disseminated lesions were observed near midbrain in 2014. A, B, and C: There was no evidence of recurrence at initially resected site.

  • Fig. 3 T1-weighted magnetic resonance images (MRIs) of thoracic spine obtained after gadolinium infusion. A: Small intradural extramedullary mass was observed at T6-7 level in 2012. B: Enlargement of the tumor size was noted in 2013. C: Preoperative image; MRI was taken when the patient showed neurological symptom in 2014, and the mass grew bigger. D: Postoperative image; the tumor was totally removed.

  • Fig. 4 Intraoperative photographs at the T6-7 vertebral level. A: After opening the dura mater, we found highly vascularized tumor mass attached to the dorsal surface of spinal cord. B: Tumor was removed completely in en-bloc fashion after coagulating the surface of the tumor. C: Spinal cord was not injured, and the tumor was totally removed.

  • Fig. 5 Histologic finding of tumor specimen stained with hematoxylin and eosin. Histopathological examination revealed endothelial, pericytic cells and stromal cells. The stromal cells was vacuolated and had larger nuclei with an eosinophilic foamy cytoplasm (×200).


Cited by  1 articles

Central Nervous System Dissemination of Solitary Sporadic Supratentorial Hemangioblastoma: A Case Report and Literature Review
Seong-Chan Jung, In-Young Kim, Shin Jung, Tae-Young Jung, Kyung-Sub Moon, Yeong-Jin Kim, Sue-Jee Park, Kyung-Hwa Lee
Brain Tumor Res Treat. 2024;12(1):80-86.    doi: 10.14791/btrt.2023.0047.


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