Fine-Needle Aspiration Diagnosisof Enterogenous Cyst of the Orbit:A Case ReportEnrique Ballesteros, M.D.,1 Ellen Greenebaum, M.D.,1,2*and John C. Merriam, M.D.3

Enterogenous cyst is a benign lesion derived from misplacedendodermal epithelium. We report the aspiration cytology findingsof an orbital cyst from a 32-year-old woman. The smears containbenign-appearing cuboidal glandular cells with focal mucinousfeatures. Carcinoembryonic antigen level in the cyst fluid wasmarkedly elevated. These findings are consistent with recurrence ofthe enterogenous cyst initially diagnosed 7 years earlier.Diagn.Cytopathol. 16:450–453, 1997.r 1997 Wiley-Liss, Inc.

Key Words:enterogenous cyst; orbital cyst; fine-needle aspiration;carcinoembryonic antigen

Enterogenous cysts (ECs) are congenital endodermal-derived cysts, thought to be embryonic developmentaldisplacements, and are lined by columnar to cuboidalepithelium.1 ECs of the central nervous system are rare, butwhen they occur they are most often located on the ventralsurface of the spinal cord and rarely intracranially. Wedescribe the cytomorphologic findings of aspirated cellularmaterial from a recurrent EC of the apical orbit. Althoughthere are many reports of FNAof endodermal-derived cysts,this case is unique because of its location in the orbit.2

Case ReportA32-year-old woman initially developed severe left retrobul-bar pain progressing to total left ophthalmoplegia and rapidloss of vision in February 1986.2 Optic neuritis was diag-nosed, and she was treated with oral prednisone. In March1986, computed tomographic (CT) scan and magneticresonance imaging (MRI) demonstrated a mass in the apex

of the left orbit. The patient was hospitalized for intravenouscorticosteroid therapy, and the pain subsided. A needlebiopsy was not diagnostic, and a presumptive diagnosis ofpseudotumor was made. Over the following 2 years thepatient required intermittent prednisone for retrobulbar pain.Repeat CT scan showed a mass between the superior andinferior orbital fissures. In June 1989, tumor extendingthrough the superior orbital fissure was excised via acraniotomy. The lesion could not be removed in toto.Histology revealed a cystic lesion lined by a benign-appearing, nonciliated, periodic acid-Schiff PAS-positive,mucicarmine-negative, columnar epithelium, consistent withan EC (Fig. C-1). She subsequently had two orbital proce-dures for recurrent fluid-filled cyst of the orbital apex.Postoperatively, she remained on a gradually tapering doseof oral prednisone for orbital pain and ophthalmoplegia andwas not completely weaned from prednisone until June1991. Over the ensuing 2 years, the patient was clinicallystable.In 1993 the patient’s best corrected visual acuity was

20/50 in the left eye with constriction of the visual field, andthe eye was limited in downward gaze. In May 1994, shecomplained of tearing, and a CT scan documented recur-rence of the cyst. By November 1994 her acuity had fallen to20/200, and she had frequent severe headaches. Needleaspiration of the cyst was not successful, and steroids wereinjected locally. The patient’s pain resolved, and her visionimproved to 20/402. However, she continued to experienceintermittent needle-like pain behind her left eye. In June1995, she returned with profound painless loss of vision tolight perception inferiorly. With CT guidance and localanesthesia (parabulbar 2% xylocaine and topical propara-caine) a 25-gauge 2.5-inch needle was used to aspirate 1 mlof fluid from the cystic orbital lesion. Twenty milligrams ofdepomedrol and contrast were injected into the residualcystic cavity (Fig. 1). Two years after the last needleaspiration she was pain free, and her visual acuity hadreturned to 20/70 corrected.

1Department of Pathology, Columbia-Presbyterian Medical Center, NewYork, New York

2Department of Clinical Pathology, College of Physicians and Surgeonsof Columbia University, New York, New York

3Department of Ophthalmology, Edward S. Harkness Eye Institute,College of Physicians and Surgeons of Columbia University, New York,New York*Correspondence to: Ellen Greenebaum, M.D., Surgical Pathology,

VC-14-215, College of Physicians and Surgeons of Columbia University,630West 168th Street, New York, NY 10032.Received 26 June 1996; Accepted 27 September 1996

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Materials and MethodsOne milliliter of aspirated cyst fluid was centrifuged for 20minutes at 2,000 rpm, and the sediment was smeared ontotwo frosted slides, which were immediately fixed in 95%alcohol for Papanicolaou staining. After microscopic exami-nation, one of the slides was destained and restained withmucicarmine.Carcinoembryonic antigen (CEA) in cyst fluid superna-

tant and the patient’s serum was quantified by immunoenzy-metric assay (Tandem-E CEA, Hybritech Inc., San Diego,CA).

ResultsThe Papanicolaou-stained cytologic preparations containedsmall clusters and strips of benign-appearing uniform cuboi-dal glandular cells and a few cuboidal to columnar cells withmucinous features; single cells were sparse, and nucleiappeared round and uniform (Fig. C-2). The background oferythrocytes, lymphocytes, histiocytes, and proteinaceousdebris was consistent with cyst contents. The glandularepithelial cells were cytomorphologically similar to the cystlining in the first biopsy obtained by craniotomy.2 Mucicar-mine stained the wispy intracellular and extracellular muci-nous material (Fig. C-3).The CEA level was 18,788 ng/ml in cyst fluid after

20-fold dilution; this is an exceedingly high level regardlessof the absence of standards for this type of fluid. Thepatient’s serum CEA was 1.4 ng/ml. Normal serum levelsmay be up to 3.0 ng/ml.

DiscussionCysts lined by a simple cuboidal and/or columnar epitheliumhave been indiscriminately labeled as endodermal, enterog-enous, neurenteric, foregut, bronchiogenic, respiratory, orteratomatous.1 Although their embryogenesis is not clearlyunderstood, these geographically varied lesions presumablyarise frommisplaced epithelium of the types destined for therespiratory or intestinal tract. Cysts lined by epithelium, aswell as communicating fistulas and sinus tracts, can resultfrom abnormal displacement of neuroectoderm, ectoderm,and endoderm; an enterogenous cyst (EC) is an evaginationof future gut endoderm (for review, see ref. 2).Enterogenous cysts are uncommon. In the central nervous

system ECs are thought to be congenital lesions.1 In 1928,Kubie and Fullon first described two cases of a CNS cystwith an intestinal-type epithelium.3 Although most ECs arespinal, arising on the extramedullary ventral surface of thelower cervical or thoracocervical spinal canal, they arereported to occur anywhere from the level of the posteriorfossa down to the lumbosacral region.2,4–6 Intraspinal ECshave been grouped according to their histologic features.7

Cysts lined by a simple cuboidal or columnar epithelium arelabeled group A, and cysts with additional features such asmucous glands and smooth muscle, or an ependymal lining,are labeled group B and C, respectively. Most intracranialenterogenous cysts would be classified as group A. Intracra-nial enterogenous cysts are extremely rare,2,5,6,8–10 mostbeing located in the posterior fossa.2,9,11A single case has

Fig. 1. Axial computed tomographic scan of contrast-filled cystic lesion at apex of left orbit (white arrow).

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been reported arising in the supratentorial region.9 This caseis the only one in the orbital apex.2

Enterogenous cysts, including those arising intracranially,are difficult to differentiate from other cystic lesions. The

neuroradiologic and clinical features of orbital tumors maybe nonspecific, making it difficult to distinguish betweencongenital, inflammatory, and neoplastic lesions.12 In thiscase a long list of diagnostic possibilities was considered:

Figs. C-1–C-3. Fig. C-1.Cyst lined by single layer of uniform columnar cells with bland basally located nuclei (hematoxylin-eosin; original magnification,3160). Reproduced by permission from Leventer et al., 1994.2 Fig. C-2.Small cluster of benign-appearing cuboidal cells. Note the round, uniform, basallylocated nuclei (Papanicolau stain,3500).Fig. C-3.Mucicarmine stain demonstrates intracellular mucin (Papanicolau stain,3500).

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optic nerve tumor, cystic schwannoma, lymphoma, sarcoid-osis, vascular anomaly, dermoid, epidermoid, and arachnoidcysts, and inflammatory pseudotumor. The cytologic find-ings of columnar and cuboidal epithelial cells, extracellularmucinous material, and a high CEA (18,788 ng/ml) supporta diagnosis of enterogenous cyst.In 1975 Schyberg13 was the first to report use of fine-

needle aspiration (FNA) for the diagnosis of orbital tumors.Although complications of FNA are of great concern,especially in the apical region, FNA of orbital lesions isgenerally a safe diagnostic method.12 The value of cytologyin the diagnosis and management of orbital and periorbitaltumors has been demonstrated in basal-cell carcinoma,14

squamous-cell carcinoma,14,15sebaceous carcinoma,12,15eo-sinophilic granuloma,14,15 meningioma,15 rhabdomyosar-coma,14,15and dermoid cyst.14,15The diagnostic accuracy ofFNAof orbital masses varies from 80 to 100%.12 In our casediagnostic FNA had the additional benefit of allowingtherapeutic injection of steroids.The CEA in the cyst fluid was markedly increased relative

to the normal level in the patient’s serum.Astudy by Fleurenet al.16 showed that ovarian cyst CEA concentrations arehigher than plasma CEA by a factor of 102–103. ElevatedCEA levels have been reported to occur in cyst contents ofboth benign and malignant serous and mucinous neoplasms.Elevated levels are more common with mucinous tumors.17

In the ovary, themucinous cysts with intestinal-type differen-tiation have higher levels of CEA than those with endocervi-cal-type epithelium (Marguerite M. Pinto, personal commu-nication). CEA measurement in ovarian cyst fluid helps todistinguish epithelial from nonepithelial types.17 However,an elevated CEA cannot distinguish between benign andmalignant16,17; 7% to 25% of malignant effusions withelevated fluid levels of CEA may be negative by cytologyand biopsy.18

The tissue biopsy performed in 1989 showed a simpleunilocular cyst lined by benign columnar epithelium. Al-though the benign features of the original biopsy and thelong intermittent clinical course of our patient argue againsta malignancy, only definitive excision with en bloc dissec-tion would determine whether the histomorphologic criteriaof malignancy are present. This procedure could be doneonly by craniotomy and would totally blind the eye. Thepresence of CEA in endodermally derived tissue, whethernon-neoplastic or neoplastic, could theoretically explain theelevated CEA level present in this orbital cyst.

AcknowledgmentsThe authors thank Marguerite M. Pinto, M.D., Larry Bern-stein, M.D., and Susan M. Staugaitis, M.D., Ph.D., forreview of manuscript.

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16. Fleuren GJ, Nap M, Aaldere JG, et al. Explanation of the limitedcorrelation between tumor CA-125 content and serum CA-125 antigenlevels in patients with ovarian tumors. Cancer 1987;60:2437–2442.

17. Pinto MM, Bernstein LH, Brogan DA, Parikh F, Lavy G. Measurementof CA125, carcinoembryonic antigen, and alpha-fetoprotein in ovariancyst fluid. Diagn Cytopathol 1990;6:160–163.

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