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The Giant Grape-Like Enterogenous Cyst Extending from the Upper Cervical Canal to the Ambient Cistern: Case Report and Literature Review

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  • Carrington Neurosciences Institute
  • Institute of General and Endovascular Neurosurgery (IGEN)

Abstract and Figures

Objective and Importance: The authors report the first known case of a giant multiloculated grape-like enterogenous cyst extending from the upper cervical canal to the ambient cistern. Clinical Presentation/Methods: We report the case of a 40-year-old male who had a prior transmastoid craniotomy at an outside facility 14 months prior with an indeter-minate diagnosis, who presented to the University of New Mexico with recurrent headaches and nausea. Scans demon-strated a giant multiloculated cystic lesion in the right cerebellopontine angle that extended superiorly and inferiorly with brainstem compression and hydrocephalus. Intervention/Results: We took the patient to the operating room for a retrosigmoid suboccipital craniectomy for tumor resection. Post-operatively, the patient improved but required ven-triculoperitoneal shunting for continued communicating hydrocephalus. Conclusion: This is the first known case of a giant multiloculated grape-like enterogenous cyst extending simultaneously from the upper cervical canal to the ambi-ent cistern. Enterogenous cysts should be considered on the differential diagnosis of giant grape-like lesions extending from the cervical canal to the prepontine cistern.
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Open Journal of Modern Neurosurgery, 2013, 3, 48-51
http://dx.doi.org/10.4236/ojmn.2013.33011 Published Online July 2013 (http://www.scirp.org/journal/ojmn)
The Giant Grape-Like Enterogenous Cyst Extending from
the Upper Cervical Canal to the Ambient Cistern: Case
Report and Literature Review
Han Chen1, Paul E. Kaloostian2*, Franklin Westhout1, Shah-Naz Khan1
1Department of Neurosurgery, University of New Mexico Albuquerque, USA
2Department of Neurosurgery, The Johns Hopkins University, Baltimore, USA
Email: *pkaloos1@jhmi.edu
Received March 19, 2013; revised April 19, 2013; accepted May 3, 2013
Copyright © 2013 Han Chen et al. This is an open access article distributed under the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Objective and Importance: The authors report the first known case of a giant multiloculated grape-like enterogenous
cyst extending from the upper cervical canal to the ambient cistern. Clinical Presentation/Methods: We report the case
of a 40-year-old male who had a prior transmastoid craniotomy at an outside facility 14 months prior with an indeter-
minate diagnosis, who presented to the University of New Mexico with recurrent headaches and nausea. Scans demon-
strated a giant multiloculated cystic lesion in the right cerebellopontine angle that extended superiorly and inferiorly
with brainstem compression and hydrocephalus. Intervention/Results: We took the patient to the operating room for a
retrosigmoid suboccipital craniectomy for tumor resection. Post-operatively, the patient improved but required ven-
triculoperitoneal shunting for continued communicating hydrocephalus. Conclusion: This is the first known case of a
giant multiloculated grape-like enterogenous cyst extending simultaneously from the upper cervical canal to the ambi-
ent cistern. Enterogenous cysts should be considered on the differential diagnosis of giant grape-like lesions extending
from the cervical canal to the prepontine cistern.
Keywords: Enterogenous; Giant; Cervical; Pre-Pontine; Grape
1. Objective and Importance
The authors report the first known case of a giant multi-
loculated grape-like enterogenous cyst extending from
the upper cervical canal to the ambient cistern.
2. Clinical Presentation
We report the case of a 40-year-old male who had a prior
craniotomy at an outside facility 14 months prior with an
indeterminate diagnosis, who presented to the University
of New Mexico with new onset headaches and nausea.
Scans demonstrated a giant multiloculated cystic lesion
extending from the upper cervical canal, into the cere-
bellopontine angle, and finally up into the ambient cis-
tern. This mass was causing brainstem compression and
obstructive hydrocephalus (Figures 1-6).
3. Intervention
We took the patient to the operating room for a retro-
sigmoid suboccipital craniectomy for tumor resection.
We identified at least 10 separate loculated compart-
ments filled with xanthochromic looking fluid that we
fenes trated and resected. The inferior portion attached to
the upper cervical cord and medulla was not resected.
Pathology demonstrated the presence of abundant mucin
secretion and glandular epithelium. CAM 5.2 highlights
the presence of abundant glandular epithelium. EMA was
also positive highlighting the presence of fibrotic under-
lying leptomeninges. CDX-2 was positive in this glandu-
lar epithelium. PAS positivity highlights the presence of
abundant mucin secretion and glandular epithelium (Fig-
ures 7 and 8). Post-operatively the patient improved
neurologically but required ventriculoperitoneal shunting
for continued communicating hydrocephalus and to al-
low for healing of his leaking wound (Figure 9).
4. Discussion
Enterogenous cysts, also known as endodermic or neu-
roepithelial cyst, are benign congenital lesions that were
first described by Puusepp in 1934 [1]. They arise from
*Corresponding author.
C
opyright © 2013 SciRes. OJMN
H. CHEN ET AL. 49
Figure 1. Post-operative CT scan after initial transmastoid
surgery at outside hospital showing minimal residual cyst
remaining. Diagnosis was not made from pathological
reports at that time.
Figure 2. Patient presents to our facility 14 months later
with recurrent symptoms and giant mass with brainstem
compression and obstructive hydrocephalus.
Figure 3. Diffusion MRI showing no restricted diffusion.
Figure 4. Coronal MRI post contrast showing this giant
grape-like mass with multiple different compartments.
Figure 5. Axial MRI post contrast showing this giant grape-
like mass.
Figure 6. Sagittal MRI post contrast showing this giant
grape-like mass.
Copyright © 2013 SciRes. OJMN
H. CHEN ET AL.
50
Figure 7. Histological analysis demonstrated presence of
abundant mucin secretion and glandular epithelium.
Figure 8. Histological analysis demonstrated presence of
abundant mucin secretion and glandular epithelium.
Figure 9. Post operative MRI showing complete resection
misplaced epithelium of the nasopharynx, re
and resolution of obstructive hydrocephalus.
spiratory tree,
known to contain clear, gelatinous,
m
differential diagnosis
sh
[1] M. Puusepp, “ e, Sousdural de la
ujitsu, S. Yagashita, T. Ichikawa, Y.
or intestinal tract and are characterized by their simple
columnar, ciliated, or goblet cell epithelium [2]. They
develop during the third or fourth week of embryonic
development [2]. Only about 100 of these lesions have
been described in the world literature [3]. These lesions
are found in all age groups, although more commonly
diagnosed in young adulthood [4]. Most of these lesions
have been described to occur in the lower cervical spine
and thoracic spine in an intradural-extramedullary fash-
ion [5]. Intracranial enterogenous cysts are extremely
rare with most of them located in the posterior fossa. The
literature notes locations of cysts are as follows: within
4th ventricle [6], cerebellopontine angle [7], ventral
brainstem [8], cerebellar vermis [9], within the medulla
[10], and at the foramen magnum [11]. Supratentorial
cysts are exceedingly rare with only 20 cases reported
worldwide [12]. No previous reports of simultaneous
upper cervical spine to ambient cistern giant multilocu-
lated lesions have been described. Additionally, this le-
sion was very unusual in that it had at least 10 separate
loculated compartments that were distinct and separate
from one another.
These cysts are
ucoid or xanthochromic fluid [13]. In 1976, Wilkins
and Odon classified these lesions into three groups based
on their histological features. Type 1 cysts have a simple
or pseudo stratified epithelium that can be cuboidal or
columnar with or without cilia. Type 2 cysts have a more
complex epithelium with respiratory or gastrointestinal
tissues. Type 3 cysts are similar to type 2 cysts but also
have glial or ependymal cells [14].
For giant intracranial cysts, the
ould include glial tumors, epidermoid cysts, arachnoid
cysts, dermoid cysts, infectious cysts, colloid cysts, me-
tastatic tumors as well as enterogenous cysts. Treatment
of choice for these lesions is total surgical resection of
the cyst wall and drainage of the cyst contents. If the wall
is not resected, these lesions may recur [15]. In cases
where the wall cannot be completely excised due to dif-
ficulty separating arachnoid pial plane, partial resection
is recommended with connection of the loculations to the
subarachnoid space [16]. When partial resection is per-
formed, symptomatic recurrence is documented at 31 %
at 2 months to 14 years [17].
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