Giant Supratentorial Acutely Hemorrhagic Enterogenous Cyst: Case Report and Literature Review

doi:10.4236/ojmn.2014.41005

Paper Menu >>

Journal Menu >>

Open Journal of Modern Neurosurgery, 2014, 4, 23-25

Published Online January 2014 (http://www.scirp.org/journal/ojmn)

http://dx.doi.org/10.4236/ojmn.2014.41005

OPEN ACCESS OJMN

Giant Supratentoria l Acutely Hemorrhagic E nterogenous

Cyst: Case Report a nd Litera ture R evi ew

Paul E. Kaloostian*, Han Chen, Franklin Westhout, Howard Yonas

University of New Mexico Neurosurgery, Albuquerque, New Mexico, USA

Email: *pka l oos1@jhmi.edu

Received November 12, 2013; revised December 12, 2013; accepted December 20, 2013

cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. In

ABSTRACT

The authors report the first case of a giant supratentorial enterogenous cyst presenting with acute symptomatic

hemorrhage within the cyst. We report the case of a 78-year-old Philipino female who was found to have a large

right fronto-parietal mass after a minor fall. She had a small amount of hemorrhage within the cyst but was sta-

ble for discharge the following day. She was readmitted 5 days later with acute onset severe headache and left-

side weakness. On repeat imaging, her cyst had grown in size and had large acute hemorrhage within it. She was

taken to the operating room for craniotomy and cyst resection. She recovered well post-operatively. This is the

first known case of a giant supratentorial enterogenous cyst presenting with symptomatic enlargement due to

large hemorrhage within the cyst. Enterogenous cysts should be considered on the differential diagnosis of he-

morrhagic supratentorial giant cysts.

KEYWORDS

Hemorrhagic; Enterogenous; Cyst; Giant; Supratentorial

1. Introduction

The authors report the first case of a giant supratentorial

enterogenous cyst presenting with expanding acute he-

morrhage within the cyst.

2. Case Presentation

We report the case of a 78-year-old Philipino female who

was found to have a large 6 × 3 × 7 cm right fron-

to-parietal mass after a minor fall (Figures 1 and 2). She

was found to have a small amount of hemorrhage within

the cyst and was then discharged home. She was read-

mitted 5 days later with severe headache, altered mental

status, and left-sided weakness.

3. Intervention

On repeat imaging, her cyst had grown in size and had

acute hemorrhage within it (Figure 3). She was taken to

the operating room for craniotomy and cyst resection (Fi-

gure 4). Intra-operatively, this mass was full of xantho-

chromic fluid with a large amount of acute clot located

posteriorly. No mural nodule was identified. The cyst

was drained and the lining was removed until we identi-

fied normal looking white matter at all angles. She suf-

fered some minor cardiac insults post-operatively but her

neurological status recovered back to baseline. Pathology

demonstrated multiple stretches of cyst lining which are

largely composed of cuboidal cells, overlying a continu-

ous basement membrane. No cilia or goblet cells were

noted. Cytokeratin CAM 5.2 was positive, with GFAP

negative and S-100 negative. CAMP 5.2 was positive

and PAS was positive (Fig ure 5).

4. Discussion

Enterogenous cysts are extremely rare, comprising only

0.01% of all central nervous system tumors [1]. Only

about 100 known cases have been described in the world

literature [2]. They are noted to originate during the third

or fourth week of embryonic development. Several names

exist for these lesions such as neuroenteric cysts, enteric

*Corresponding a uthor.

P. E. KALOOSTIAN ET AL.

OPEN ACCESS OJMN

Figure 1. CT head show ing giant right fronto-parietal ente-

rogenic cyst with mass effect on the occipital horn.

Figure 2. MRI brain showing giant right fronto-parietal en-

terogenic cyst with mass effect on the occipital horn.

Figure 3. CT head showing spontaneous hemo rr hage w ithi n

the giant cyst with evidence of midline shift.

Figure 4. Post-operative CT head showing complete resolu-

tion of the mass effect and resection of the cyst.

Figure 5. High power view hematoxylin and eosin staining

demonstrating multiple stretches of cyst lining, which are

largely composed of cuboidal cells, overlying a continuous

basement membrane, a feature of Type I enterogenous cysts

epithelial. No cilia or goblet cells were seen.

cysts, endodermic cysts, gastrogenic cysts, and broncho-

genic cysts. Various theories exist to explain their patho-

genesis. Perhaps the most accepted is that the cysts locat-

ed between the diencephalon and mesencephalon revive

from remnants of the Seesel’s Pouch. This diverticulum

arises caudal to Rathke’s pouch and rostrodorsal to oro-

pharyngeal membrane [3]. These lesions are documented

in all age groups, though most commonly in children and

young adults [4]. These cysts are most commonly located

in the lower cervical and upper thoracic segments of the

spinal cord, and tend to occupy the region anterior to the

spinal cord or brainstem [5]. There are four documented

cases of malignant transformation of these cysts [6].

The intracranial locatio n is quite rare. The first reports

of intracranial cysts wer e by Small in 19 62 and Giombini

in 1981 [7,8]. Reports of intracranial enterogenous cysts

note the midline posterior fossa region anterior to the

brainstem to be the most common site, as well as the

fourth ventricular region [9]. Supratentorial enterogenous

cysts are exceedingly rare, with only 20 described cases

P. E. KALOOSTIAN ET AL.

OPEN ACCESS OJMN

[10]. These patients are usually older than patients with

infratentorial cysts and much is not known about their

natural history due to the paucity of cases worldwide.

Giant supratentorial enterogenous cysts have only been

described in 2 cases thus far [11]. Their natural history

has yet to be documented. No reports of spontaneous he-

morrhage within the cyst have been documented thus far.

On patholog y, these cys t walls show immunorea ctivity

for EMA, CEA, and cytokeratin with absent reactivity

for glioneurona l markers, and are lin ed typically by colu-

mnar mucin-rich epithelium resembling enteric or respi-

ratory epithelium [12].

Treatment of choice for these lesions is total surgical

resection of the contents and cyst wall, so as to prevent

reaccumulation. However, if there is extensive scarring

and adherence to the pia mater , it is reco mmend ed to leave

the inner wall behind to avoid injuring the cortex [13].

Prevention of spillage of the contents of the cyst is criti-

cal to preventing dissemination [13].

REFERENCES

[1] S. Matsunaga, K. Fujitsu, S. Yagishita, T. Ichikawa, Y.

Takemoto, Y. Takenda et al., “Endodermal Cyst Ventral

to the Lower Brain Stem,” Neurologia Medico-Chirurgi-

ca, Vol. 46, No. 12, 2006, pp. 614-618.

http://dx.doi.org/10.2176/nmc.46.614

[2] R. R. Sharma, R. R. Ravi, N. T. Gurusinghe, C. Coutinho,

A. K. Mahapatra, J. Sousa et al., “Cranio-Spinal Entero-

genous Cysts: Clinic-Radiological Analysis in a Series of

Ten Cases,” Journal of Clinical Neuroscience, Vol . 8, No.

2, 2001, pp. 133-139.

http://dx.doi.org/10.1054/jocn.2000.0792

[3] C. P. Harris, M. S. Dias, D. L. Brochmeyer, J. J. Town-

send, B. K. Willis and R. I. Apfelbaum, “Neurenteric Cysts

of the Posterior Fo ssa: Recogni tion, Ma nagement , and Em-

bryogenesis,” Neurosurgery, Vol. 29, No. 6, 1991, pp.

893-897.

http://dx.doi.org/10.1227/00006123-199112000-00015

[4] A. Perry, B. W. Scheithauer, B. W. Zaias and H. V. Mi-

nassian, “Aggressive Enterogenous Cyst with Extensive

Craniospinal Spread: Case Report,” Neurosurgery, Vol.

44, No. 2, 1999, pp. 410-404.

http://dx.doi.org/10.1097/00006123-199902000-00098

[5] A. H. Menezes and V. C. T raynelis, “Spinal Neuroenteric

Cyst in the Magnetic Resonance Imaging Era,” Neurosur-

gery, Vol. 58, No. 1, 2006, pp. 97-105.

http://dx.doi.org/10.1227/01.NEU.0000192160.79897.25

[6] M. Gessi, F. Legnani, E. Maderna, C. Casali, C. L. Soelro,

B. Pollo et al., “Mucinous Low Grade Adenocarcinoma

Arising in an Intracranial Enterogenous Cyst: Case Re-

port,” Neurosurgery, Vol. 62, No. 4, 2008, pp. E972-

E973.

http://dx.doi.org/10.1227/01.neu.0000318186.10536.f6

[7] J. S mall , “Pre Axial Enteroge nous Cysts,” Journal of Neu-

rology, Neurosurgery & Psychiatry, Vol. 25, 1962, p.

184.

[8] S. Giombini, S. Lodrini and F. Migliaavacca, “Intracrani-

al Enterogenous Cysts,” Surgical Neurology, Vol. 16, No.

4, 1981, pp. 271-273.

http://dx.doi.org/10.1016/0090-3019(81)90053-7

[9] F. Beijani, D. C. Wright, D. Schessel and L. N. Sekhar,

“Endodermal Cysts of the Posterior Fossa. Report of Three

Cases and Review of the Literature,” Journal of Neuro-

surgery, Vol. 89, No. 2, 1998, pp. 326-335.

http://dx.doi.org/10.3171/jns.1998.89.2.0326

[10] J. M. Garbizu, B. Mateo-Sierra, F. Ruiz-Juretsche and J.

M. Perez-Calvo, “Supratentorial Endodermal Cyst: Case

Report and Literature Review,” Neurochirurgia, Vol. 20,

No. 4, 2009, pp. 111-115.

[11] C. Christo, F. Chretien, P. Brugreies and M. Djindjian,

“Giant Supratentorial Enterogenous Cyst: Report of a Case,

Literature Review, and Discussion of Pathogenesis,” Neu-

rosurgery, Vol. 54, No. 3, 2003, pp. 759-763.

http://dx.doi.org/10.1227/01.NEU.0000109538.07853.7F

[12] F. Scaravilli, H. Lidov, D. J. Spal ton and L. Sym on, “Neu-

roenteric Cyst of the Optic Nerve. Case Report with Im-

munohistochemical Study,” Journal of Neurology and

Neurosurgical Psychiatry, Vol. 55, No. 12, 1992, pp.

1197-1199. http://dx.doi.org/10.1136/jnnp.55.12.1197

[13] S. Bavetta, K. El-Shunnar and P. J. Hamlyn, “Neuroente-

ric Cyst of the Anterior Cranial Fossa,” British Journal of

Neurosurgery, Vol. 10, No. 2, 1996, pp. 225-227.

http://dx.doi.org/10.1080/02688699650040430