CASE REPORTS

PONTINE ATYPICAL NEUROCYTOMA: CASE REPORT

Bradley M. Swinson, B.S.
Department of Neurosurgery,
University of Florida,
Gainesville, Florida
William A. Friedman, M.D.
Department of Neurosurgery,
University of Florida,
Gainesville, Florida
Anthony T. Yachnis, M.D.
Department of Pathology,
University of Florida,
Gainesville, Florida
Reprint requests:
William A. Friedman, M.D.,
P.O. Box 100265,
Department of Neurosurgery,
University of Florida,
Gainesville, FL 32610.
Email: friedman
@neurosurgery.ufl.edu
OBJECTIVE AND IMPORTANCE: Neurocytomas are typically located within the
supratentorial ventricular system. Extraventricular neurocytomas are very rare, and this
is only the second reported case of a pontine neurocytoma. We discuss the clinical
presentation, histology, and treatment of these rare tumors.
CLINICAL PRESENTATION: A 58-year-old man presented with a 4-month history of
headache and unilateral facial and distal extremity paresthesia. Magnetic resonance
imaging (MRI) scans demonstrated a 2.6 ⫻ 2.2-cm ring-enhancing cystic mass in the
right pons.
INTERVENTION: MRI-guided stereotactic biopsy yielded a diagnosis of atypical neu-
rocytoma. Because of the location and malignant histological features of the tumor, the
patient was initially treated with external beam radiation therapy. Several months later,
MRI scans demonstrated tumor progression. The patient then underwent three rounds
of temozolomide chemotherapy, during and after which his symptoms worsened.
Aggressive subtotal resection of the tumor was achieved via a right suboccipital
craniectomy.
CONCLUSION: Twenty-eight months postoperatively, the patient is symptom free,
and MRI scans demonstrate no evidence of residual or recurrent tumor.
KEY WORDS: Brainstem, Neurocytoma

Received, August 19, 2005. Neurosurgery 58:990-991, 2006 DOI: 10.1227/01.NEU.0000210213.12847.1E www.neurosurgery-online.com
Accepted, January 3, 2006.

C
entral neurocytomas (CN) are rare neo-
plasms that are usually located in the
supratentorial ventricular system in
the region of the foramen of Monro. They are
most commonly diagnosed in the second and
third decades of life, without sex preference,
presenting with signs and symptoms of ob-
structive hydrocephalus. CNs are composed
of isomorphous, small, round cells with “salt
and pepper” chromatin and perinuclear halos
resembling oligodendrocytes, but showing
immunohistochemical and ultrastructural ev-
idence of neuronal differentiation (22, 23).
They generally demonstrate benign biological
behavior and have been shown to be amena-
ble to surgical excision with favorable long-
term outcome, and accordingly have been
designated World Health Organization
(WHO) Grade I (23, 26, 39, 42). Since the first
description of a CN by Hassoun et al. (22) in
1982, more than 500 cases have been reported
in the literature, and it has been estimated that
CNs account for 0.25 to 0.5% of all brain tu-
mors (23, 39). More recently, immunohisto-
chemical studies have revealed a subset of
CNs, identifiable by a high MIB-1/Ki-67 label-
ing index (LI) or vascular proliferation and
termed atypical CN, which may exhibit more
aggressive biological behavior and be associ-
ated with less favorable outcome (41, 49). Rare
instances of CN-like tumors arising outside
the supratentorial ventricular system have
been reported, and such tumors have been
designated extraventricular neurocytomas
(EVNs). We describe the second reported case
of a pontine neurocytoma.
CASE REPORT
A 58-year-old man was referred to the Neu-
rosurgical Specialties Clinic in July 2001 with a
4-month history of intermittent, left-sided fa-
cial and perioral paresthesia, which pro-
gressed to include the left hand and the left
foot, and a 4-month history of occasional mild
headaches extending from the occiput to the
frontal portion of the head. Physical and neu-
rological examination revealed no abnormali-
ties.

E990 | VOLUME 58 | NUMBER 5 | MAY 2006 www.neurosurgery-online.com


Magnetic resonance imaging (MRI) scans performed at an
outpatient imaging center 6 weeks before the patient’s presen-
tation to our clinic demonstrated a cystic mass lesion in the
right pons extending superiorly to the right mesencephalon
and inferiorly to the right middle cerebellar peduncle. The
mass measured approximately 2.6 cm transversal, 2.2 cm in
the anterior-posterior plane, and 2.5 cm in the superior-
inferior plane and exerted some mass effect upon the anterior
aspect of the fourth ventricle. The mass was hypointense on a
T1-weighted series, hyperintense on a T2-weighted series,
and, after intravenous administration of gadolinium contrast,
demonstrated rim-like enhancement with small zones of nod-
ular enhancement anteriorly and posterosuperiorly. MRI
scans obtained before stereotactic biopsy (Fig. 1) also demon-
strated the above findings. Computed tomographic scans also
performed in preparation for MRI-guided stereotactic biopsy
demonstrated postcontrast enhancement and calcification in
the medial component of the mass.
An MRI-guided stereotactic biopsy of the mass was performed
without complication using a right transcerebellar trajectory via a
twist drill hole in the right suboccipital area. Histological examina-
tion revealed a rather uniform population of small cells with pe-
rinuclear halos, hyperchromatic nuclei with finely speckled ⬙salt and
pepper⬙ chromatin, and small nucleoli (Fig. 2A). These cells were
arranged in sheets and often formed rosettes surrounding islands of
fibrillary neuropil-like material. A second population of slightly
larger cells with clear cytoplasm and more vesicular nuclei with
prominent nucleoli were also present, arranged in small groups and
diffusely scattered throughout the lesion. Rare ganglion cells were
observed and appeared to be part of the tumor rather than resident
neurons (Fig. 2B). A loose myxoid stroma separated tumor cells in
many areas. Occasional mitotic figures were present, and there was
evidence of focal vascular endothelial proliferation (Fig. 2A), but no
apparent necrosis. The MIB-1 tumor LI was 6 to 8% (Fig. 2C).
Immunohistochemical studies revealed that all components of the
tumor were strongly immu-
noreactive for synaptophysin
(Fig. 2D). A subpopulation of
smaller cells and the ganglion
cells were immunoreactive for
neurofilament. Only the gan-
glion cells were immunoreac-
tive for the neuronal marker
NeuN. Course cell processes
of reactive appearing astro-
cytes were strongly immuno-
reactive for glial fibrillary
acidic protein (GFAP). How-
ever, the neoplastic cells were
GFAP-negative. The final
pathological diagnosis was
atypical CN.
Because of the location of FIGURE 1. Transaxial T1-weighted
the tumor and the potential MRI scan with contrast showing a 2.6 ⫻
for malignant biological be- 2.2 cm ring-enhancing cystic mass in right
havior on the basis of MIB-1 pons.
NEUROSURGERY
PONTINE ATYPICAL NEUROCYTOMA
FIGURE 2. A, neurocytic tumor with uniform population of cells with
round, hyperchromatic nuclei and perinuclear “halos.” This area of tumor
also shows vascular endothelial proliferation (hematoxylin-eosin [H&E];
original magnification, ⫻1000). B, rare ganglion cells (right) were present
(H&E; original magnification, ⫻1000). C, immunohistochemical stain for
Ki67 (MIB-1) showing several labeled tumor cell nuclei. Labeling index
was 6 to 8% (original magnification, ⫻1000). D, immunohistochemical
stain for synaptophysin showing diffuse, strong reactivity in tumor cells
(original magnification, ⫻1000).
LI, the patient was initially treated with external beam radiation
therapy (RT). He received 54 Gy in 27 fractions to a local field
with shrinking fields over approximately 6 weeks, after which he
reported that all of his symptoms had abated, and a restaging
MRI scan demonstrated a moderate decrease in tumor size from
2.6 ⫻ 2.2 ⫻ 2.5 cm to 2.0 ⫻ 2.0 ⫻ 2.0 cm. However, approximately
5 months postradiotherapy, the patient began to experience in-
termittent, left-sided facial and perioral paresthesia and occa-
sional headaches, and MRI scans demonstrated tumor progres-
sion to a size of 2.7 ⫻ 2.3 ⫻ 2.6 cm with an increased central cystic
component and increased enhancement in the wall of the mass.
Physical and neurological examination again revealed no abnor-
malities; the patient reported experiencing left-sided facial and
perioral paresthesia. However, he was objectively found to have
intact pain and touch sensation bilaterally.
The patient was then started on temozolomide (Temodar,
Schering-Plough Corp., Kenilworth, NJ) chemotherapy. The
first round (175 mg/m2/d, Days 1–5 of 28) was tolerated
extremely well. However, during follow-up, the patient re-
ported progression of the paresthesia in the left foot to include
the entirety of the left lower extremity distal to the knee and a
decreased ability to walk long distances or run. Neurological
examination revealed no cranial nerve deficits. However, mar-
ginally decreased sensation in the distal left upper and lower
extremities was appreciated. The patient then underwent a
second round of temozolomide chemotherapy (200 mg/m2/d,
Days 1–5 of 28), which was again tolerated extremely well,
after which he reported moderate disequilibrium on upward
gaze in addition to the symptoms already described. Physical
VOLUME 58 | NUMBER 5 | MAY 2006 | E990
SWINSON ET AL.
examination revealed slight nystagmus on upward gaze, and
neurological examination revealed decreased sensation in the
left upper extremity extending from the shoulder distally and
involving all dermatomes, as well as decreased sensation in
the left lower extremity distal to the knee and involving all
dermatomes. The patient then underwent a third and final
round of temozolomide chemotherapy (200 mg/m2/d, Days
1–5 of 28), after which there was no change in his performance
status, symptoms, or physical and neurological examination
findings. Follow-up MRI scans demonstrated minimal tumor
progression, and the patient chose to observe and be reimaged
in 2 to 3 months.
Two and one-half months later, the patient again presented
to our clinic complaining of worsening left-sided facial and
perioral paresthesia, worsening paresthesia of the left extrem-
ities, increasing disequilibrium brought on by certain posi-
tions and movements, and decreased hearing acuity in the left
ear. Neurological examination revealed decreased pin sensa-
tion affecting the left side of the face and the entirety of the left
extremities, as well as limited proprioception on the left. MRI
scans performed at that time (Fig. 3) demonstrated an increase
in tumor size from 2.7 ⫻ 2.3 to 2.9 ⫻ 2.4 cm in the transaxial
plane, increased thickening of the anteromedial wall of the
tumor, slight mass effect on the sylvian aqueduct, and exten-
sion of the mass superiorly into the mesencephalon.
The patient underwent a right suboccipital craniectomy
with drainage of the cyst and aggressive subtotal resection
FIGURE 3. Transaxial (A and B)
and coronal (C) fluid attenuated
inversion recovery MRI scans
showing an increase in the size of
the cystic mass in the right pons
and its extension superiorly into
the right mesencephalon.
E990 | VOLUME 58 | NUMBER 5 | MAY 2006
(STR) of the solid neoplasm. The pons was found to be grossly
expanded and discolored. An incision was made superior to
the trigeminal nerve root exit zone, and xanthochromatic fluid
exited the pons under pressure. Shaggy neoplastic material
lining the cyst was identified and aggressively resected. There
were no perioperative or postoperative complications, al-
though the patient did experience temporary paresthesia in
both upper extremities immediately after surgery. The re-
sected neoplastic tissue was histologically similar to the orig-
inal biopsy specimen, but showed radiation changes; the MIB-
1/Ki-67 LI was 0 to 2%, significantly lower than the 6 to 8% LI
of the original tumor.
The patient’s neurological symptoms resolved after surgery
and have not returned in the 28 months since. Postoperative
MRI sequences (Fig. 4) demonstrate a small focus of enceph-
alomalacia in the right pons with no evidence of residual or
recurrent tumor.
DISCUSSION
Central Neurocytoma
CN was first described by Hassoun et al. (22) in 1982 in an
ultrastructural study of two cases and, in 1993, was recognized
as a distinct pathological entity and classified as Grade I in the
WHO classification of tumors of the central nervous system
(26). It has been estimated that CN may account for only 0.25
to 0.5% of all brain tumors, and the literature contains approx-
imately 500 reported cases (23, 39). They are most commonly
diagnosed in the third decade of life. In a retrospective review
of 127 published cases, Hassoun et al. (23) reported an average
age at diagnosis of 29 years, but CNs may occur at any age and
without sex preference. CNs are characteristically located in
the supratentorial ventricular system in the region of the fo-
ramen of Monro, attached to the septum pellucidum, the walls
of the lateral ventricles, the fornices, the corpus callosum, the
roof of the third ventricle, or the choroid plexus (23, 59).
Consistent with their location, CNs typically present with
signs and symptoms of increased intracranial pressure in-
duced by obstructive hydrocephalus, including headache,
FIGURE 4. Transaxial (A) and coronal (B) proton density MRI scans
with contrast obtained 28 months postoperatively showing a small focus of
encephalomalacia with no evidence of tumor.
www.neurosurgery-online.com

nausea and emesis, and papilledema. Visual disturbances in-
cluding blurred vision, diplopia, decreased visual acuity, in-
termittent loss of vision, and photophobia, as well as mental
disturbances including altered consciousness, memory loss,
disorientation, and mild dementia are not uncommon (23).
Although the clinical history is usually quite short, Hassoun et
al. (23) reported an average of 3.2 months, it may be longer,
and some CNs have been identified incidentally (54). Com-
puted tomographic scans usually show a well-circumscribed,
isodense to hyperdense lesion with areas of calcification,
which moderately and heterogeneously enhances postcontrast
(23, 61). MRI scans typically demonstrate a lesion that is
hypointense to isointense on T1-weighted series and hyperin-
tense on T2-weighted series, proton density sequences, and
fluid attenuated inversion recovery sequences. CNs typically
demonstrate irregular enhancement on postgadolinium series
and may have a cystic component (23, 56, 61). Magnetic res-
onance spectroscopy typically demonstrates an increased cho-
line peak and decreased creatinine and N-acetylaspartate
(NAA) peaks in addition to a characteristic peak at 3.55 ppm,
which may be caused by inositol or glycine and although not
always present may be a unique marker for neurocytoma.
NAA is a neuronal marker, and a decreased NAA signal
generally indicates a loss of intact neurons; its counterintuitive
diminished presence in these neuronal tumors may indicate
that the neurocytes in CNs are so immature that very little
NAA is produced (25).
Extraventricular Neurocytoma
CN-like tumors have been reported in a variety of locations
outside the supratentorial ventricular system, including the
frontal lobe (5, 16, 19–21, 35, 37), temporal lobe (5, 20, 35, 37,
53), parietal lobe (2, 5, 6, 20, 29, 47), occipital lobe (5, 20, 29, 45),
insular cortex (36), insular-operculum (25), amygdala (38),
sphenoid wing (6), thalamus (5, 46), hypothalamus (5, 20),
cerebellum (4, 13), pons (48), spinal cord (2, 10, 30, 32, 50, 52),
cauda equina (51), retina (33), and two sites outside the central
nervous system (17, 24). These unique neoplasms have been
termed EVNs and are mentioned in the 2000 WHO classifica-
tion of tumors of the nervous system, but remain unclassified
(9). Presenting symptoms obviously vary depending on the
location of the tumor. EVNs of the cerebral hemispheres,
which seem to be the most common site for EVN tumorigen-
esis, most often present with seizures (7, 21, 35, 37, 47, 53). The
appearance of EVNs on neuroimaging studies is similar to that
of CNs, as are magnetic resonance spectroscopy spectra (5, 34).
Although EVNs of the posterior fossa, such as the one we
report here, are exceedingly rare, there is a small, but growing,
body of literature concerning a neurocytoma-like tumor that
arises specifically in the posterior fossa. This rare neoplasm
has in the past been referred to by a variety of names including
⬙lipomatous medulloblastoma,⬙ ⬙lipidized medulloblastoma,⬙
⬙medullocytoma,⬙ ⬙neurolipocytoma,⬙ ⬙lipomatous ganglion-
eurocytoma,⬙ ⬙lipomatous glioneurocytoma,⬙ and ⬙lipidized
mature neuroectodermal tumor,⬙ and, in the 2000 WHO clas-
NEUROSURGERY
PONTINE ATYPICAL NEUROCYTOMA
sification of tumors of the nervous system, was officially rec-
ognized as a distinct pathological entity, classified as Grade
I/II and termed the cerebellar liponeurocytoma (1, 8, 27). To
date, the literature contains nearly 30 reported cases of cere-
bellar liponeurocytoma, which is characterized by lipidized
neurocytes resembling mature adipocytes in a neurocytoma-
like background (the histology of CN and EVN is discussed
below) with some glial differentiation (1, 8, 27). This new
entity has been mentioned because of its resemblance to EVN
and its location in the posterior fossa. However, because the
neoplasm with which we are concerned here was located in
the pons rather than the cerebellum and because it did not
contain lipidized neurocytes or differentiated glial cells, cere-
bellar liponeurocytomas will not be described further.
The only previous report of a pontine EVN was made by
Soontornniyomkij and Schelper (48) in 1996. They described
the case of an 18-year-old male presenting with an acute onset
of diplopia and a right trochlear nerve palsy, who was found
to have a 1.2 cm solid mass in the right pons in the region of
the superior cerebellar peduncle, which was totally resected
via a right suboccipital craniotomy approach. The patient did
not receive RT or chemotherapy at any time. His postoperative
course was complicated by acute pyogenic meningitis and
subsequent communicating hydrocephalus, necessitating
treatment with antibiotics and placement of a right ventricu-
loperitoneal shunt, but he was alive and well with no evidence
of recurrence 14 months postoperatively (48).
Biological Behavior
CNs and EVNs usually demonstrate relatively benign bio-
logical behavior and are generally amenable to surgical resec-
tion with favorable long-term outcome (20, 23, 37, 39, 42, 43).
However, there have been reports of aggressive behavior by
neurocytomas, including malignant histological and clinical
features (12, 44, 49, 60), tumor progression (11, 12, 14, 31, 44,
56, 58), recurrence after gross total resection (GTR) (3, 14, 31,
44, 49), and craniospinal dissemination (3, 12, 14), as well as
reports of unfavorable outcomes (12, 31, 43, 49, 56, 57). The
most commonly used method for assessing the proliferative
potential of a neurocytoma is immunolabeling with MIB-1, a
monoclonal antibody directed against the nuclear antigen Ki-
67. MIB-1 immunohistochemistry allows direct assessment of
the size of a tumor’s growth fraction because Ki-67 is ex-
pressed by nuclei in all phases of the cell cycle (G1, S, G2, and
M), but not by cells in the G0 phase. Typically, both CNs and
EVNs have low (⬍2%) MIB-1 LIs. However, this is not always
the case (20, 23, 31, 41, 49). An analysis of 41 neoplasms from
36 patients diagnosed with CN carried out by Soylemezoglu et
al. (49) showed a significant correlation between proliferative
potential as measured by MIB-1 LI and clinical behavior. Their
data demonstrated a significantly longer relapse-free survival
time for patients whose tumors had low (⬍2%) MIB-1 LIs
compared with those whose tumors had high (ⱖ2%) MIB-1
LIs. They also found a highly significant correlation between
high MIB-1 LI and the presence of vascular proliferation. They
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SWINSON ET AL.
suggested the term ⬙atypical neurocytoma⬙ for those tumors
exhibiting a MIB-1 LI 2% or greater or vascular proliferation
and thus potentially at greater risk for aggressive biological
behavior and less favorable outcome. Their data also showed
that atypical neurocytomas may account for as much as 40% of
all neurocytomas (49). A similar study by Rades et al. (41), this
time a retrospective analysis of 131 patients, showed a signif-
icant difference in local control and survival rates between
patients whose tumors exhibited low (⬍3%) versus high
(ⱖ3%) MIB-1 LIs. Although these atypical CNs and EVNs may
demonstrate more aggressive behavior and warrant more ag-
gressive treatment, it is unclear whether neurocytomas exhib-
iting anaplastic features, but low MIB-1 LIs warrant more
aggressive treatment, and some reports indicate that they may
not (43, 44).
Histology and Immunohistochemistry
Histologically, CNs consist of areas of moderately dense,
isomorphous, small, round cells (neurocytes) surrounded by a
fine fibrillary matrix. The neurocytes are arranged in diffuse
sheets and also form pseudorosettes surrounding small is-
lands of fibrillary neuropil-like material. Homer-Wright ro-
settes and perivascular rosettes may also be present. The nu-
clei of these neurocytes are round or oval with finely speckled
⬙salt and pepper⬙ chromatin and a sometimes prominent nu-
cleolus. Perinuclear halos are present, giving the neoplasm a
honeycomb appearance similar to that of an oligodendrogli-
oma. For this reason, CN was often misdiagnosed as such
before its original description by Hassoun et al. (22, 23). Scat-
tered ganglion cells may be present. Mitoses, nuclear atypia,
necrosis, and vascular endothelial proliferation are relatively
rare but do occur (22, 23, 49). Synaptophysin, a glycoprotein
associated with the synaptic vesicle membrane, is the most
reliable marker for the diagnosis of CN because the neurocytes
demonstrate strong immunoreactivity for it (23, 56). They may
also demonstrate immunoreactivity for the neuronal marker
NeuN (15). Neurocytes are typically not immunoreactive for
GFAP, although focal cellular reactivity is sometimes present,
and some GFAP staining in the fibrillary matrix, probably
caused by trapped astrocytic processes, is not uncommon (6,
23). Ultrastructural studies will reveal neuritic processes, syn-
aptic vesicles, and synapses (22, 23). EVNs share these same
basic histological features, but exhibit a wider morphological
spectrum (9). Ganglion cell differentiation, such as that seen in
our case, is much more common in EVNs than in CNs, and, in
a study of 35 EVNs by Brat et al. (5), 66% contained ganglion
cells. Immunohistochemical features of glial differentiation are
also much more common in EVNs than CNs, and in that same
study, focal GFAP immunoreactivity in tumor cells with neu-
rocytic features was noted in almost half of the cases (5).
Although neurocytoma was once widely thought to be a pure
neuronal neoplasm, such findings have led to the hypothesis
that neurocytomas arise from an undifferentiated precursor
cell with the potential for both neuronal and glial differentia-
tion (56).
E990 | VOLUME 58 | NUMBER 5 | MAY 2006
TREATMENT
The treatment of choice for all neurocytomas, central as well
as extraventricular, typical as well as atypical, is GTR (5, 22,
23, 39, 40). For most CNs, tumor resection can be performed
through a transcortical-transventricular approach or an
interhemispheric-transcallosal-transventricular approach (43).
For EVNs, the surgical approach will obviously vary depend-
ing on tumor location; we used a suboccipital craniectomy for
surgical access to the pontine EVN described herein. When
GTR is not possible, STR plus postoperative RT is indicated (5,
6, 31, 39, 40, 49).
After GTR or STR plus RT, recurrence-free intervals of
several years are common (23, 39, 40, 42, 43). Schild et al. (42)
retrospectively reviewed 32 cases of CN and reported actuar-
ial 5-year local control and survival rates of 79 and 81%.
Furthermore, they reported 5-year local control and survival
rates of 100 and 90% for patients who underwent GTR and
5-year local control and survival rates of 100 and 88% for
patients who underwent STR plus RT (42). A similar retro-
spective review of 310 cases of CN was conducted by Rades
and Fehlauer (39). They reported 3-year local control rates of
95% after GTR and 89% after STR plus RT, an insignificant
difference. They also reported 5-year survival rates of 99%
after GTR and 90% after STR plus RT, a significant difference.
In cases with documented tumor progression, they found a
median time to progression of 36 months after GTR and 34
months after STR plus RT (39). Rades et al. (40) retrospectively
reviewed 85 cases of atypical neurocytoma and reported
3-year local control and survival rates of 73 and 93% after GTR
and 85 and 87% after STR plus RT, as well as 5-year local
control and survival rates of 57 and 93% after GTR and 70 and
78% after STR plus RT. Furthermore, they reported 3-year
local control and survival rates of 53 and 57% and 5-year local
control and survival rates of 18 and 22% for 21 cases of
atypical EVN. They concluded that there was no significant
difference in local control or survival between atypical CNs
and atypical EVNs (40). In a study of 35 EVNs, Brat et al. (5)
reported that nearly one-third recurred within a short period
of time. More specifically, although none of the 14 totally
resected tumors recurred within the follow-up period, 10 of
the remaining tumors recurred after STR with or without
accompanying RT. Furthermore, they reported that only one
of 10 atypical EVNs could be totally resected and that five of
the remaining nine atypical EVNs recurred after STR (5).
Radiation Therapy
Large retrospective studies have shown that postoperative
RT does not improve local control or survival in patients who
have undergone GTR, but does seem to significantly improve
local control and survival in patients who have undergone
STR (39, 40, 42). Also, RT is often administered in cases of
recurrent disease, and at least one report of a marked fall in
MIB-1 LI after RT suggests that it may be considered in the
treatment of neurocytomas with elevated proliferation poten-
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tial (6, 18). In this case, RT was administered as initial treat-
ment because the pontine neurocytoma could not be surgically
resected without significant risk of permanent neurological
deficit and because it demonstrated elevated proliferation po-
tential with a MIB-1 LI of 6 to 8%. The treatment resulted in
moderate tumor regression and resolution of symptoms for a
period of approximately 5 months, after which the tumor
continued to progress. The MIB-1 LI was significantly reduced
from 6 to 8% at biopsy to 0 to 2% at surgery 18 months later.
The authors are aware of only one other report of neurocy-
toma treated with RT immediately after stereotactic biopsy.
This report was made by Kulkarni et al. (28), who retrospec-
tively reviewed eight patients with CN who were treated in
this manner and received no other therapy. They reported
that, although one patient developed disseminated intracra-
nial disease 15 months post-treatment, another survived for 5
years post-treatment, and six of the eight were symptom-free
with good local control at a mean follow-up period of 78
months. Because neurocytomas typically exhibit benign bio-
logical behavior and are often amenable to GTR with very
favorable results, RT is generally reserved for adjuvant ther-
apy after STR, treatment of residual or recurrent disease, and
in a few rare cases, treatment of inoperable neurocytomas.
Radiosurgery has also been used as a radiation treatment
option for residual disease (55).
Chemotherapy
Because GTR and STR plus postoperative RT are generally
so effective, chemotherapy has rarely been used in the treat-
ment of neurocytoma, and its value is unclear. In fact, the
literature contains only 14 reported cases in which chemother-
apy was a component of the treatment for neurocytoma (3, 11,
14, 30, 42, 44, 58). Each of these cases involved CN; to the best
of the authors’ knowledge, this is the first reported case of an
EVN treated with chemotherapy. Furthermore, in each of the
previously reported cases, chemotherapy was administered
either as a component of initial therapy also involving surgical
resection (11, 30, 42) or as salvage therapy after postoperative
tumor progression (3, 14, 44, 58). To the best of the authors’
knowledge, this is the first reported case in which chemother-
apy was administered before surgical resection. Chemothera-
peutic agents used in these previously reported cases include
cytoxan (30), cisplatin (3, 14, 30, 42), carboplatin (11, 44),
etoposide (3, 11, 14), ifosfamide (11), cyclophosphamide (3,
14), carmustine (42), lomustine (42), vincristine (42, 58), pro-
carbazine (58), prednisone (42), and CCNU (58) in various
combinations. In this case, the chemotherapeutic agent temo-
zolomide (Temodar, Schering-Plough Corp., Kenilworth, NJ)
was selected. However, after three rounds of chemotherapy
(175 mg/m2/d, Days 1–5 of 28 for round 1, 200 mg/m2/d,
Days 1–5 of 28 for rounds 2 and 3), the patient’s symptoms had
worsened, and MRI scans demonstrated no reduction in tu-
mor size. Of the 14 previously reported cases, one showed
similarly poor response, and the patient died of tumor pro-
gression within several months (44), four showed significant
NEUROSURGERY
PONTINE ATYPICAL NEUROCYTOMA
tumor regression lasting 15 to 32 months (3, 58), seven re-
ported positive outcomes that could not be attributed to the
effects of chemotherapy alone because of concomitant surgical
resection or RT (11, 30, 42), and, in two cases, no data on tumor
response or follow-up were given (14). The value of chemo-
therapy in the treatment of neurocytoma remains unclear;
however, it may offer an alternative when surgical resection is
not possible and RT is inappropriate or has already been
administered, as was the case here.
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COMMENTS
T he authors present a somewhat unusual, perhaps unique, case of
an atypical central neurocytoma in a 58-year-old man. The patient
was treated first with stereotactic biopsy, then with radiation therapy
and temozolomide chemotherapy, and finally with direct surgery via
retrosigmoid approach with subtotal removal. The patient is doing
well after 28 months. Extraventricular neurocytic neoplasms are very
rare, predisposed toward children and young adults, and almost
never exhibit malignant cytological features. The present case is,
therefore, unusual from many points of view, making it even more
interesting. Making this case even more unusual, in my point of view,
is the algorithm treatment adopted (biopsy, radio therapy, chemother-
apy, and direct surgery). But, what is important in the end is the result
obtained, which, in this case, seems extremely favorable with the
patient symptom-free and with clean magnetic resonance imaging
scans, even with a short follow-up period. We cannot say yet that the
patient is cured. In cases similar to this, with focal, well-circumscribed
pontine gliomas on the magnetic resonance imaging scan and, more
importantly, when the tumor is close to the surface of the brainstem,
we prefer to start the management with direct aggressive surgery with
the aim of possible total removal (1, 2). This attitude immediately
www.neurosurgery-online.com

eliminates the need for stereotactic biopsy, which, in this site, is not
free of complications, and allows for later decisions on whether ra-
diotherapy or chemotherapy is needed.
Albino Bricolo
Verona, Italy
1. Bricolo A: Surgical management of intrinsic brainstem gliomas. Op Tech
Neurosurg 3:137–154, 2000.
2. Bricolo A, Turazzi S: Surgery for gliomas and other mass lesions of the
brainstem, in Symon L, Calliauw L, Cohadon F, Dolenc VV, Antunes JL,
Nornes H, Pickard JD, Reulen JJ, Strong AJ, de Tribolet N (eds): Advances and
Technical Standards in Neurosurgery. New York, Springer, 22:261–341, 1995.
N eurocytomas are rare tumors when they occur intraventricularly
and are even more rare in extraventricular locations, as in this
case. Those with low proliferation indices are potentially curable with
surgery alone. This article is one of several, however, that report on
tumors with a more aggressive histology and clinical course. None-
theless, definitive surgery seems to have led to prolonged remission,
if not cure, with the initial high proliferative rate not withstanding.
It is important for neurosurgeons and their pathologists to consider
NEUROSURGERY
PONTINE ATYPICAL NEUROCYTOMA
neurocytoma when biopsying both intra- and extraventricular lesions.
This is particularly true when there is a histological resemblance to
oligodendroglioma, as their respective management strategies, espe-
cially the use of chemotherapy, may be quite different between the
two entities.
Gene H. Barnett
Cleveland, Ohio
T his is an interesting case report of a patient with an atypical neuro-
cytoma that is notable for its unusual location in the pons. Because
this is a rare tumor, it is difficult to draw conclusions about its biological
behavior. The high Ki67-MIB-1 proliferative index may be coincidental or
may somehow be associated with its unusual location.
The unusual clinical features made it difficult to develop a treat-
ment plan for this tumor on initial presentation. Curiously, the tumor
was resistant to radiation and chemotherapy, yet responded well to an
aggressive resection. It will be interesting to see whether long-term
follow-up reveals an aggressive tumor consistent with its predicted
behavior based on Ki67-MIB-1 labeling.
Jeffrey N. Bruce
New York, New York
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