ABSTRACT
Ganglioglioma (GG) is an uncommon primary lesion of the central nervous system that
is typically located supratentorially. There are only a few reports of GG arising
from the cerebellum. To the best of our knowledge this is the first case of a cerebellar
GG with supratentorial extension and a longstanding history before its recognition.
In fact, this 29-year-old male presented with an 11-year history of intermittent headaches.
A cranial computerized tomography (CT) performed at the onset of his complaints failed
to reveal the tumor. After a particularly longstanding cephalalgic episode, the patient
underwent a new CT scan that was also negative. However, magnetic resonance (MR) imaging
of the brain revealed a space-occupying lesion in the right cerebellar hemisphere
with extension to the level of the superior colliculi and pineal recess. The tumor
was partially removed through a midline suboccipital craniotomy and supracerebellar
approach. Pathological examination of the tumor showed composition of atypical ganglion
cells and astrocytes, indicating the diagnosis of cerebellar GG. At last follow-up,
24 months after surgery, the patient reported a marked improvement of his clinical
condition with significant reduction of intensity and frequency of the headache. The
present report illustrates how cerebellar GG may remain undetectable by CT and may
therefore present with a longstanding history and nonspecific signs and symptoms.
MR investigation can lead to the proper diagnosis. Even after partial removal the
prognosis remains good and remission of the symptoms may be achieved. In this article,
we review the literature and summarize the current understanding of infratentorial
GGs.
KEYWORDS
Ganglioglioma - posterior cranial fossa - cerebellum - brainstem - neuronal tumors
- history
REFERENCES
- 1
Zentner J, Wolf H K, Ostertun B et al..
Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients.
J Neurol Neurosurg Psychiatry.
1994;
57
1497-1502
- 2
Demierre B, Stichnoth F A, Hori A, Spoerri O.
Intracerebral ganglioglioma.
J Neurosurg.
1986;
65
177-182
- 3
Koeller K K, Henry J M.
Superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of
Pathology.
Radiographics.
2001;
21
1533-1556
- 4
Garcia C A, McGarry P A, Collada M.
Ganglioglioma of the brain stem: case report.
J Neurosurg.
1984;
60
431-434
- 5
Probst A, Ulrich J, Zdrojewski B, Hirt H R.
Cerebellar ganglioglioma in a child.
J Neuropathol Exp Neurol.
1979;
38
57-71
- 6
Mork S J, Berg-Jensen M, Haugen A.
Cerebellar ganglioglioma: case report.
J Neurosurg.
1979;
51
562-564
- 7
Fukuoka T, Tamiya T, Yamanaka A et al..
A case of cerebellar ganglioglioma in an infant: immunohistochemical study. [in Japanese].
No To Shinkei.
1985;
37
1101-1107
- 8
Dhillon R S.
Posterior fossa ganglioglioma: an unusual cause of hearing loss.
J Laryngol Otol.
1987;
101
714-717
- 9
Harada K, Sigemori M, Kobayashi S, Matsuo H, Kuramoto S, Aoyagi K.
Cerebellar ganglioglioma: a case report. [in Japanese].
No Shinkei Geka.
1988;
16(suppl)
607-612
- 10
Turgut M, Ozcan O E.
Cerebellar ganglioglioma: case report.
J Neurosurg Sci.
1990;
34
151-153
- 11
Kimura M, Suzuki M.
Cerebellar ganglioglioma: a case report. [in Japanese].
No Shinkei Geka.
1990;
18
861-865
- 12
Bills D C, Hanieh A.
Hemifacial spasm in an infant due to fourth ventricular ganglioglioma: case report.
J Neurosurg.
1991;
75
134-137
- 13
Nishizawa S, Yokoyama T, Ryu H et al..
Cerebellar ganglioglioma-case report.
Neurol Med Chir (Tokyo).
1991;
31
777-781
- 14
Hirose T, Kannuki S, Nishida K, Matsumoto K, Sano T, Hizawa K.
Anaplastic ganglioglioma of the brain stem demonstrating active neurosecretory features
of neoplastic neuronal cells.
Acta Neuropathol (Berl).
1992;
83
365-370
- 15
Lindboe C F, Cappelen J, Kepes J J.
Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report.
Neurosurgery.
1992;
31
353-355
- 16
Geyer J R, Schofield D, Berger M, Milstein J.
Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma.
J Neurooncol.
1992;
14
237-241
- 17
Chang T, Teng M M, Lirng J F.
Posterior cranial fossa tumours in childhood.
Neuroradiology.
1993;
35
274-278
- 18
Handa H, Yamagami T, Furata M.
An adult patient with cerebellar ganglioglioma.
J Neurooncol.
1994;
18
183-189
- 19
Osanai S, Iida Y, Nomura T et al..
A case of unilateral brain-stem tumor and impaired ventilatory response. [in Japanese].
Nihon Kyobu Shikkan Gakkai Zasshi.
1994;
32
990-995
- 20
Blatt G L, Ahuja A, Miller L L, Ostrow P T, Soloniuk D S.
Cerebellomedullary gangliglioma: CT and MR findings.
AJNR Am J Neuroradiol.
1995;
16
790-792
- 21
Wang Z, Sutton L N, Cnaan A et al..
Proton MR spectroscopy of pediatric cerebellar tumors.
AJNR Am J Neuroradiol.
1995;
16
1821-1833
- 22
Harvey A S, Jayakar P, Duchowny M et al..
Hemifacial seizures and cerebellar ganglioglioma: an epilepsy syndrome of infancy
with seizures of cerebellar origin.
Ann Neurol.
1996;
40
91-98
- 23
Jay V, Greenberg M.
Unusual cerebellar ganglioglioma with marked cytologic atypia.
Pediatr Pathol Lab Med.
1997;
17
105-114
- 24
McLone D G, Stieg P E, Scott R M, Barnett F, Barnes P D, Folkerth R.
Cerebellar epilepsy.
Neurosurgery.
1998;
42
1106-1111
- 25
Vinchon M, Soto-Ares G, Ruchoux M M, Dhellemmes P.
Cerebellar gliomas in children with NF1: pathology and surgery.
Childs Nerv Syst.
2000;
16
417-420
- 26
Evans A J, Fayaz I, Cusimano M D, Laperriere N, Bilbao J M.
Combined pleomorphic xanthoastrocytoma-ganglioglioma of the cerebellum.
Arch Pathol Lab Med.
2000;
124
1707-1709
- 27
Kwon J W, Kim I O, Cheon J E et al..
Cerebellopontine angle ganglioglioma: MR findings.
AJNR Am J Neuroradiol.
2001;
22
1377-1379
- 28
Chae J H, Kim S K, Wang K C, Kim K J, Hwang Y S, Cho B K.
Hemifacial seizure of cerebellar ganglioglioma origin: seizure control by tumor resection.
Epilepsia.
2001;
42
1204-1207
- 29
Lagares A, Gomez P A, Lobato R D, Ricoy J R, Ramos A, de la Lama A.
Ganglioglioma of the brainstem: report of three cases and review of the literature.
Surg Neurol.
2001;
56
315-324
- 30
Kinoshita Y, Kiya K, Satoh H et al..
Cerebellar ganglioglioma associated with a huge cyst: case report. [in Japanese].
No Shinkei Geka.
2002;
30
503-507
- 31
Mesiwala A H, Kuratani J D, Avellino A M, Roberts T S, Sotero M A, Ellenbogen R G.
Focal motor seizures with secondary generalization arising in the cerebellum: case
report and review of the literature.
J Neurosurg.
2002;
97
190-196
- 32
Mink J W, Caruso P A, Pomeroy S L.
Progressive myoclonus in a child with a deep cerebellar mass.
Neurology.
2003;
61
829-831
- 33
Friedman W A, Vries J K, Quisling R G.
Ganglioglioma of the medulla oblongata.
Surg Neurol.
1979;
12
105-108
- 34
Richmond J G, Cunningham D L, Fuste F G, Erickson C C.
Ganglioglioma of the brainstem.
Am J Clin Pathol.
1966;
45
692-696
- 35
Garrido E, Becker L F, Hoffman H J, Hendrick E B, Humphreys R.
Gangliogliomas in children: a clinicopathological study.
Childs Brain.
1978;
4
339-346
- 36
Epstein N, Epstein F, Allen J C, Aleksic S.
Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction:
report of a case.
Neurosurgery.
1982;
10
612-616
- 37
Martin L D, Kaplan A M, Hernried L S, Fisher B J.
Brainstem gangliogliomas.
Pediatr Neurol.
1986;
2
178-182
- 38
Nelson J, Frost J L, Schochet Jr S S.
Sudden, unexpected death in a 5-year-old boy with an unusual primary intracranial
neoplasm: ganglioglioma of the medulla.
Am J Forensic Med Pathol.
1987;
8
148-152
- 39
Mizuno J, Nishio S, Barrow D L, Davis P C, Tindall G T.
Ganglioglioma of the cerebellum: case report.
Neurosurgery.
1987;
21
584-588
- 40
Mickle J P.
Ganglioglioma in children: a review of 32 cases at the University of Florida.
Pediatr Neurosurg.
1992;
18
310-314
- 41
Pollack I F, Hoffman H J, Humphreys R P, Becker L.
The long-term outcome after surgical treatment of dorsally exophytic brain-stem gliomas.
J Neurosurg.
1993;
78
859-863
- 42
Lang F F, Epstein F J, Ransohoff J et al..
Central nervous system gangliogliomas. Part 2: clinical outcome.
J Neurosurg.
1993;
79
867-873
- 43
Nixon K T, Hudgins P A, Davis P C, O'Brien M S, Hudgins R J, Hoffman Jr J C.
Delayed intracranial hemorrhage in children after suboccipital craniectomy.
AJR Am J Roentgenol.
1994;
163
897-900
- 44
Karamitopoulou E, Perentes E, Probst A, Wegmann W.
Ganglioglioma of the brainstem: neurological dysfunction of 16-year duration.
Clin Neuropathol.
1995;
14
162-168
- 45
Yachnis A T.
Neuropathology of pediatric brain tumors.
Semin Pediatr Neurol.
1997;
4
282-291
- 46
Gleckman A M, Smith T W.
Sudden unexpected death from primary posterior fossa tumors.
Am J Forensic Med Pathol.
1998;
19
303-308
- 47
Farmer J P, Montes J L, Freeman C R, Meagher-Villemure K, Bond M C, O'Gorman A M.
Brainstem gliomas: a 10-year institutional review.
Pediatr Neurosurg.
2001;
34
206-214
- 48
Ildan F, Tuna M, Gocer I A, Erman T, Cetinalp E.
Intracerebral ganglioglioma: clinical and radiological study of eleven surgically
treated cases with follow-up.
Neurosurg Rev.
2001;
24
114-118
- 49
Courville C B.
Gangliogliomas: tumor of the central nervous system-review of the literature and report
of 2 cases.
Arch Neurol Psychiatry.
1930;
24
39-91
- 50
Courville C B, Anderson F M.
Neuro-gliogenic tumor of the central nervous system: report of two additional cases
of ganglioglioma of the brain.
Bull Los Angeles Neurol Soc.
1941;
6
154-176
- 51
Sasaki A, Hirato J, Nakazato Y, Tamura M, Kadowaki H.
Recurrent anaplastic ganglioglioma: pathological characterization of tumor cells-case
report.
J Neurosurg.
1996;
84
1055-1059
- 52
Russell D S, Rubinstein L J.
Ganglioglioma: a case with a long history and malignant evolution.
J Neuropathol Exp Neurol.
1962;
21
185-193
- 53
Hayashi Y, Iwato M, Hasegawa M, Tachibana O, von Deimling A, Yamashita J.
Malignant transformation of a gangliocytoma/ganglioglioma into a glioblastoma multiforme:
a molecular genetic analysis-case report.
J Neurosurg.
2001;
95
138-142
- 54
Tien R D, Tuori S L, Pulkingham N, Burger P C.
Gangliogliomas with leptomeningeal and subarachnoid spread: results of CT, MR, and
PET imaging.
AJR Am J Roentgenol.
1992;
159
391-393
- 55
Goncalves-Ferreira A J, Herculano-Carvalho M, Pimentel J.
Stereotactic biopsies of focal brainstem lesions.
Surg Neurol.
2003;
60
311-320
- 56
Johnson Jr J H, Hariharan S, Berman J et al..
Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years.
Pediatr Neurosurg.
1997;
27
203-207
- 57
Athale S, Hallet K K, Jinkins J R.
Ganglioglioma of the trigeminal nerve: MRI.
Neuroradiology.
1999;
41
576-578
- 58
Dorne H L, O'Gorman A M, Melanson D.
Computed tomography of intracranial oligodendrogliomas.
AJNR Am J Neuroradiol.
1986;
7
281-285
- 59
Castillo M, Davis P C, Takei Y, Hoffman Jr J C.
Intracranial ganglioglioma: MR, CT and clinical findings in 18 patients.
AJR Am J Roentgenol.
1990;
154
607-612
- 60
Haddad S F, Moore S A, Menezes A H, Van Gilder J C.
Ganglioglioma: 13 years of experience.
Neurosurgery.
1992;
31
171-178
- 61
Baltuch G H, Farmer J P, Meagher-Villemure K, O'Gorman A M, Montes J L.
Ganglioglioma presenting as a vascular lesion in a 10-year-old boy: case report.
J Neurosurg.
1993;
79
920-923
- 62
Siddique K, Zagardo M, Gujrati M, Olivero W.
Ganglioglioma presenting as a meningioma: case report and review of the literature.
Neurosurgery.
2002;
50
1133-1135
- 63
Kumabe T, Shimizu H, Sonoda Y, Shirane R.
Thallium-201 single-photon emission tomographic and proton magnetic resonance spectroscopic
characteristics of intracranial ganglioglioma: three technical case reports.
Neurosurgery.
1999;
45
183-187
- 64
Uematsu Y, Fujita K, Tanaka Y et al..
Gamma knife radiosurgery for neuroepithelial tumors: radiological and histological
changes.
Neuropathology.
2001;
21
298-306
- 65
Schrottner O, Unger F, Eder H G, Feichtinger M, Pendl G.
Gamma-Knife radiosurgery of mesiotemporal tumour epilepsy observations and long-term
results.
Acta Neurochir Suppl.
2002;
84
49-55
- 66
Kalyan-Raman U P, Olivero W C.
Ganglioglioma: a correlative clinicopathological and radiological study of ten surgically
treated cases with follow-up.
Neurosurgery.
1987;
20
428-433
- 67
Silver J M, Rawlings III C E, Rossitch Jr E, Zeidman S M, Friedman A H.
Ganglioglioma: a clinical study with long-term follow-up.
Surg Neurol.
1991;
35
261-266
- 68
Rumana C S, Valadka A B.
Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas.
Neurosurgery.
1998;
42
1038-1043
Sam Safavi-AbbasiM.D. Ph.D.
c/o Neuroscience Publications, Barrow Neurological Institute
350 W. Thomas Road, Phoenix, AZ 85013
Email: neuropub@chw.edu
Email: s.sabbasi@chw.edu