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JOURNAL OF NEUROLOGY AND NEUROSCIENCE
ISSN 2171-6625
2015
Vol. 6 No. 3:26
1
© Under License of Creative Commons Attribution 3.0 License | This article is available in: www.jneuro.com
Case Report
DOI: 10.21767/2171-6625.100026
Charuta Gavankar
1
,
Ryan A Grant
1*
,
Robert Fulbright
2
,
Anita Huttner
3
and
Jennifer Moliterno
1
1 Department of Neurosurgery, Yale
University School of Medicine, New
Haven, CT, USA
2 Department of Radiology, Yale University
School of Medicine, New Haven, CT, USA
3 Department of Pathology, Yale University
School of Medicine, New Haven, CT, USA
Corresponding author: Ryan Grant
ryan.grant@yale.edu
Department of Neurosurgery, Yale School
of Medicine, 333 Cedar Street, TMP4, New
Haven, CT 06510, USA.
Tel:203.785.2805
Fax: 203.785.6916
Citation: Gavankar C, Grant RA, Fulbright R,
et al. Mixed Tumor with Subependymoma
and Ependymoma Features: A Case Report
and Review of the Literature. J Neurol
Neurosci. 2016, 6:3.
Introducton
Subependymomas are rare, slow-growing, benign ependymal
neoplasms histologically characterized as grade I tumors by
the World Health Organizaton (WHO) [1]. First described as
pathologically distnct enttes in 1945, subependymomas have a
predilecton for the fourth (50-60%) and lateral (30-40%) ventricles
[2,3]. Characteristcally indolent and asymptomatc, most go
unreported; however, some studies estmate subependymomas
to represent 0.2%–0.7% of all intracranial tumors and
approximately 8% of ependymal tumors [4,5]. Rare clinical
manifestatons of subependymomas include hydrocephalus
secondary to cerebrospinal fuid (CSF) obstructon and less
frequently mass efect [6,7]. More commonly, subependymomas
are clinically silent and treated with expectant management untl
hydrocephalus or mass efect warrants surgical resecton [6].
On the other hand, ependymomas are WHO grade II tumors of
neuroectodermal origin that consttute between 2%–6% of adult
intracranial tumors and 6% - 10% of pediatric brain tumors [8-
11]. Ependymomas arise within the ventricular system of the
central neuraxis and are most commonly infratentorial (60%),
showing a predilecton for the foor of the fourth ventricle [12].
Given the increased risk of recurrence and drop metastasis
associated with these tumors, complete surgical resecton
followed by postoperatve radiaton therapy represents the
standard of care for ependymomas [13-17]. The signifcant
diference in ependymoma and subependymoma behavior
and thus management makes accurate preoperatve diagnosis
especially crucial. Considering both tumors arise in similar
locatons, magnetc resonance (MR) imaging characteristcs can
help diferentate the two diagnoses and guide management.
Mixed Tumor with Subependymoma and
Ependymoma Features: A Case Report and
Review of the Literature
Abstract
Background: Subependymomas are slow-growing, benign ependymal neoplasms
histologically characterized as grade I tumors. On the other hand, ependymomas
are higher-grade tumors of neuroectodermal origin that share a predilecton for
the fourth ventricle. Given that these tumors can occur simultaneously as mixed
lesions, features that raise clinical suspicion of a combined pathology warrant
surgical resecton for defnitve diagnosis and potental cure.
Case report: A 61-year-old ambidextrous male presented with headache and an MRI
demonstratng a lesion involving the fourth ventricle. Imaging demonstrated faint
contrast enhancement and growth through the foramen of Luschka, rendering the
possibility of an ependymoma or a mixed entty. The patent underwent gross total
resecton, with pathology confrming a mixed subependymoma-ependymoma. He
was discharged neurologically intact and underwent external beam radiotherapy.
Conclusions: Mixed subependymoma-ependymomas are exceedingly rare tumors
ofen misdiagnosed as pure subependymomas. In the setng of a mixed pathology,
however, the lesion warrants neurosurgical interventon and requires the more
aggressive management that is standard for ependymomas. While the exact origin
of these mixed neoplasms remains unknown, further investgaton using molecular
methods will likely help elucidate the pathogenesis and mechanisms that underlie
combined subependymoma-ependymoma tumors.
Keywords: Mixed subependymoma ependymoma; Subependymoma;
Ependymoma
Received: August 25, 2015; Accepted: November 10, 2015; Published: November
14, 2015