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

Chung SY, Jeun SS, Park JH - Brain Tumor Res Treat (2014)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus

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).
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Figure 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).

Mentions: Whole spine MRI revealed negative finding until 2012, but a intradural extramedullary mass was noted at T6-7 level in 2012 without any symptom (Fig. 3). In 2014, during follow-up of the thoracic lesion, he developed severe back pain at T9 dermatome, which correlated with the lesion. Spine MRI was done and it showed enlargement of the previously noted tumor mass at T6-7 level. Workup for VHL disease was done again, and the peripheral blood was analyzed for the presence of VHL gene mutations by direct sequencing method using ABI 3,730 sequencer. However, VHL gene mutation was not detected and there was no evidence of VHL disease. The patient underwent surgery for thoracic mass, and the tumor was completely removed (Fig. 4). The pathologic diagnosis of the tumor was HB, which is same as the cerebellar lesion resected previously (Fig. 5). The Ki-67 index was 10%. After surgery, his symptom was improved and until this case being reported, his Karnofsky Performance Scale score was 90 and remained symptom-free.


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

Chung SY, Jeun SS, Park JH - Brain Tumor Res Treat (2014)

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).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4231619&req=5

Figure 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).
Mentions: Whole spine MRI revealed negative finding until 2012, but a intradural extramedullary mass was noted at T6-7 level in 2012 without any symptom (Fig. 3). In 2014, during follow-up of the thoracic lesion, he developed severe back pain at T9 dermatome, which correlated with the lesion. Spine MRI was done and it showed enlargement of the previously noted tumor mass at T6-7 level. Workup for VHL disease was done again, and the peripheral blood was analyzed for the presence of VHL gene mutations by direct sequencing method using ABI 3,730 sequencer. However, VHL gene mutation was not detected and there was no evidence of VHL disease. The patient underwent surgery for thoracic mass, and the tumor was completely removed (Fig. 4). The pathologic diagnosis of the tumor was HB, which is same as the cerebellar lesion resected previously (Fig. 5). The Ki-67 index was 10%. After surgery, his symptom was improved and until this case being reported, his Karnofsky Performance Scale score was 90 and remained symptom-free.

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Seoul St. Mary's Hospital, The Catholic University of Korea College of Medicine, Seoul, Korea.

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.

No MeSH data available.


Related in: MedlinePlus