Brainstem gangliogliomas: a retrospective series

Clinical article

Restricted access

Object

The authors retrospectively analyzed data on brainstem gangliogliomas treated in their department and reviewed the pertinent literature to foster understanding of the preoperative characteristics, management, and clinical outcomes of this disease.

Methods

In 2006, the authors established a database of treated lesions of the posterior fossa. The epidemiology findings, clinical presentations, radiological investigations, pathological diagnoses, management, and prognosis for brainstem gangliogliomas were retrospectively analyzed.

Results

Between 2006 and 2012, 7 patients suffering from brainstem ganglioglioma were treated at the West China Hospital of Sichuan University. The mean age of the patients, mean duration of symptoms prior to diagnosis, and mean duration of follow-up were 28.6 years, 19.4 months, and 38.1 months, respectively. The main presentations were progressive cranial nerve deficits and cerebellar signs. Subtotal resection was achieved in 2 patients, and partial resection in 5. All tumors were pathologically diagnosed as WHO Grade I or II ganglioglioma. Radiotherapy and adjuvant chemotherapy were not administered. After 21–69 months of follow-up, patient symptoms were resolved or stable without aggravation, and MRI showed that the size of residual lesions was unchanged without progression or recurrence.

Conclusions

The diagnosis of brainstem ganglioglioma is of great importance given its favorable prognosis. The authors recommend the maximal safe resection followed by close observation without adjuvant therapy as the optimal treatment for this disease.

Abbreviation used in this paper:GFAP = glial fibrillary acidic protein.

Article Information

Address correspondence to: Xuhui Hui, Ph.D., Department of Neurosurgery, West China Hospital of Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu, 610041, People's Republic of China. email: zhangsi198712@126.com.

Please include this information when citing this paper: published online February 1, 2013; DOI: 10.3171/2013.1.JNS121323.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Magnetic resonance images showing different patterns of enhancement of brainstem gangliogliomas. The tumor in Case 1 (A) enhanced homogeneously with an associated cyst after administration of Gd. The tumor in Case 2 (B) enhanced heterogeneously. The tumors in Cases 4 (C) and 7 (D) did not enhance after administration of Gd.

  • View in gallery

    Case 3. Preoperative MR images showing a lesion in the medulla and middle cerebellar peduncle with hypointensity on a T1-weighted sequence (A) and hyperintensity on a T2-weighted sequence (B). No enhancement is observed after administration of Gd (C). Postoperative (45 months after surgery) MR images (D–F) showing the size of the residual tumor is unchanged.

  • View in gallery

    Case 3. Photomicrographs obtained during pathological examination of tumor tissue. An H & E–stained section (A) showing the coexistence of ganglion cells (arrow) and glial cells. Results of immunohistochemistry testing for neurofilaments (B) and GFAP (C) revealing the glial cells classified as astrocytomas (WHO Grade II). Original magnification ·200 (B) and ·400 (A and C).

References

  • 1

    Akiyama HNakamizo SKawamura ANagashima THasegawa DKosaka Y: [Ganglioglioma of the medulla oblongata: case report and review of the literature.]. No Shinkei Geka 34:125512602006. (Jpn)

  • 2

    Baussard BDi Rocco FGarnett MRBoddaert NLellouch-Tubiana AGrill J: Pediatric infratentorial gangliogliomas: a retrospective series. J Neurosurg 107:4 Suppl2862912007

  • 3

    Cultrera FGuiducci GNasi MTPaioli GFrattarelli M: Twostage treatment of a tectal ganglioglioma: endoscopic third ventriculostomy followed by surgical resection. J Clin Neurosci 13:9639652006

  • 4

    Gleckman AMSmith TW: Sudden unexpected death from primary posterior fossa tumors. Am J Forensic Med Pathol 19:3033081998

  • 5

    Im SHChung CKCho BKWang KCYu IKSong IC: Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery. J Neurooncol 59:1731832002s

  • 6

    Johnson JH JrHariharan SBerman JSutton LNRorke LBMolloy P: Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg 27:2032071997

  • 7

    Lagares AGómez PALobato RDRicoy JRRamos Ade la Lama A: Ganglioglioma of the brainstem: report of three cases and review of the literature. Surg Neurol 56:3153242001

  • 8

    Lang FFEpstein FJRansohoff JAllen JCWisoff JAbbott IR: Central nervous system gangliogliomas. Part 2: Clinical outcome. J Neurosurg 79:8678731993

  • 9

    Löbel UEllison DWShulkin BLPatay Z: Infiltrative cerebellar ganglioglioma: conventional and advanced MRI, proton MR spectroscopic, and FDG PET findings in an 18-month-old child. Clin Radiol 66:1942012011

  • 10

    Luyken CBlümcke IFimmers RUrbach HWiestler ODSchramm J: Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 101:1461552004

  • 11

    Majores Mvon Lehe MFassunke JSchramm JBecker AJSimon M: Tumor recurrence and malignant progression of gangliogliomas. Cancer 113:335533632008

  • 12

    Mpairamidis EAlexiou GAStefanaki KSfakianos GProdromou N: Brainstem ganglioglioma. J Child Neurol 23:148114832008

  • 13

    Pandita ABalasubramaniam APerrin RShannon PGuha A: Malignant and benign ganglioglioma: a pathological and molecular study. Neuro Oncol 9:1241342007

  • 14

    Rades DZwick LLeppert JBonsanto MMTronnier VDunst J: The role of postoperative radiotherapy for the treatment of gangliogliomas. Cancer 116:4324422010

  • 15

    Rumana CSValadka AB: Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas. Neurosurgery 42:103810431998

  • 16

    Rumana CSValadka ABContant CF: Prognostic factors in supratentorial ganglioglioma. Acta Neurochir (Wien) 141:63691999

  • 17

    Safavi-Abbasi SDi Rocco FChantra KFeigl GCEl-Shawarby ASamii A: Posterior cranial fossa gangliogliomas. Skull Base 17:2532642007

  • 18

    Siddique KZagardo MGujrati MOlivero W: Ganglioglioma presenting as a meningioma: case report and review of the literature. Neurosurgery 50:113311362002

  • 19

    Westwood DAMacFarlane MR: Pontomedullary ganglioglioma: a rare tumour in an unusual location. J Clin Neurosci 16:1081102009

  • 20

    Zentner JWolf HKOstertun BHufnagel ACampos MGSolymosi L: Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry 57:149715021994

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 151 151 26
Full Text Views 431 431 1
PDF Downloads 193 193 2
EPUB Downloads 0 0 0

PubMed

Google Scholar