Limits...
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

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

License
getmorefigures.php?uid=PMC4231619&req=5

Figure 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.

Mentions: In 2005, a 59-year-old male patient who suffered from occipital headache underwent complete surgical excision of a solitary cerebellar mass. Occipital transtentorial approach was done, and pathological diagnosis was confirmed to be hemangioblastoma (Fig. 1). He had no family history of VHL disease and no clinical stigmata to suggest the presence of VHL disease. VHL gene mutation was not detected in his peripheral blood sample. His symptom resolved after surgery, and the magnetic resonance image (MRI) of the brain showed no remnant lesion postoperatively. The patient was followed up with MRI, and no evidence of residual or recurrent lesion was observed until 2010.


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

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

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

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

Figure 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.
Mentions: In 2005, a 59-year-old male patient who suffered from occipital headache underwent complete surgical excision of a solitary cerebellar mass. Occipital transtentorial approach was done, and pathological diagnosis was confirmed to be hemangioblastoma (Fig. 1). He had no family history of VHL disease and no clinical stigmata to suggest the presence of VHL disease. VHL gene mutation was not detected in his peripheral blood sample. His symptom resolved after surgery, and the magnetic resonance image (MRI) of the brain showed no remnant lesion postoperatively. The patient was followed up with MRI, and no evidence of residual or recurrent lesion was observed until 2010.

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