Clinicopathological Report

Eye-conserving treatment in massive congenitalorbital teratomaKaan Gündüz MD,1 Rengin Aslıhan Kurt MD1 and Aylin Okçu Heper MD2

Departments of 1Opthalmology and 2Pathology, Ankara University Faculty of Medicine, Ankara, Turkey

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ABSTRACT

An 8-month-old healthy girl presented with a leftorbital mass, which orbital magnetic resonanceimaging revealed to be a well-circumscribed, mostlycystic lesion. The patient underwent orbitotomy viainferior fornicial conjunctival approach. Tumourshrinkage was accomplished by aspiration of theintralesional fluid, and the remaining debulked masswas removed by total excisional biopsy. Pathologicalexamination revealed a cystic tumour lined mainlywith keratinized stratified squamous epithelium, inaddition to small foci of mucinous ciliated epitheliumresembling respiratory epithelium. Histopathologicalfindings were consistent with the diagnosis of matureorbital teratoma (hair follicles, adipose tissue, matureglial elements, choroid plexus-like papillary organiza-tions and small foci of cartilage in the cyst wall).Derivatives of all three germ lines were present. At56-month follow up, uncorrected visual acuity in theaffected eye was 6/9. This case demonstrates theimportance of decompressing the tumour before dis-secting it from the periorbital tissues in an eye-conserving approach to orbital teratoma.

Key words: eye conserving treatment, orbital teratoma,orbitotomy, tumour decompression.

INTRODUCTION

Teratomas are composed of a mixture of tissuesthat are foreign to the site of origin. Therefore,they are considered to be choristomatoustumours.1–3 The most common locations for terato-

mas are sacrococcygeal, gonadal, retroperitoneal,mediastinal, and the head and neck region. Terato-mas of the head and neck comprise about 9% of allteratomas.2,4

Orbital teratomas, which are congenital, are rare.They can arise primarily within the orbit, or cranialteratomas may secondarily invade the orbit.1–3 In thispaper, we present a case of primary orbital teratomaand describe our eye-conserving managementstrategy.

CASE REPORT

An 8-month-old girl presented with a rapidlygrowing mass over the past months pushing theeyeball superiorly. She had been born full-term viavaginal delivery. The prenatal and postnatal historieswere unremarkable. External examination showed aleft orbital mass displacing the eye superiorly andanteriorly and producing bluish discoloration on theoverlying skin (Fig. 1a). The left pupil was reactiveto light. Orbital magnetic resonance imagingrevealed a well-circumscribed, mostly cystic leftorbital mass (Fig. 1b,c). On T1-weighted images, thelesion was isointense with respect to the contralat-eral vitreous and hypointense with respect to thecerebral grey matter (Fig. 1b). On T2-weightedimages, the lesion was isointense with respect to thecontralateral vitreous and hyperintense with respectto the cerebral grey matter (Fig. 1c).

The patient underwent orbitotomy via an inferiorfornicial conjunctival approach. To shrink thetumour, we aspirated the intratumoural fluidthrough a 20-G needle. Approximately 16 mL of yel-lowish clear fluid was withdrawn (Fig. 1f). Care was

� Correspondence: Professor Kaan Gündüz, Department of Ophthalmology, Mesa Koza Plaza 24/18, Küpe Sok, GOP 06700, Ankara, Turkey. Email:

eyemd@ada.net.tr

Received 7 October 2008; accepted 22 February 2009.

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© 2009 The AuthorsJournal compilation © 2009 Royal Australian and New Zealand College of Ophthalmologists

taken not to spill the cystic contents into the orbitand rupture the cyst wall. Then, the collapsed masswas carefully dissected off the orbital tissues usingblunt dissection. The mass was adherent to the pos-terior sclera, and a beaver 57 blade was used tocarefully dissect the tumour off the sclera. The lesionwas totally excised (Fig. 1d). There were no intraop-erative complications. The conjunctival incision wasclosed with a running 7/0 vicryl suture.

On gross examination, the bisected tumour had acystic appearance (Fig. 1e). Histopathological exami-nation revealed a cystic tumour with a mixture ofvarious mature tissues within the solid areas of cystwall. Most of the inner surface of the cyst was linedwith keratinized stratified squamous epithelium(Fig. 2a), with small foci of mucinous ciliated epi-

thelium resembling respiratory epithelium (Fig. 2b).Small hair follicles representing skin and its append-ages were seen under the epithelium (Fig. 2a).Adipose tissue, mature glial tissue (Fig. 2a), choroidplexus-like papillary organizations (Fig. 2c), andsmall foci of cartilage (Fig. 2d) were also noted. Nomitotic figures were seen. Histopathological findingswere consistent with the diagnosis of mature orbitalteratoma. Derivatives of all three germ lines werepresent, such as squamous epithelium and hair fol-licles for ectoderm, glial tissue for neural ectoderm,cartilage for mesoderm and respiratory epitheliumfor endoderm.

The patient has now been followed up for56 months with no sign of recurrence. Uncorrectedvisual acuity in the affected eye is 6/9.

Figure 1. (a) Facial photograph shows aleft orbital mass displacing the eye supe-riorly and anteriorly and producing bluishdiscoloration on the overlying skin. (b)T1-weighted axial orbital magnetic reso-nance image reveals a well-circumscribedmostly cystic left orbital mass. The lesionis isointense with respect to the contral-ateral vitreous and hypointense withrespect to the cerebral grey matter. (c)T2-weighted axial orbital magnetic reso-nance image reveals a well-circumscribedmostly cystic left orbital mass. The lesionis isointense with respect to the contralat-eral vitreous and hyperintense withrespect to the cerebral grey matter. (d)Gross photograph of the excised orbitalmass. (e) Cut surface of the orbital tumourshowing a cystic mass. (f ) Aspirated intra-tumoural fluid. The total amount comes to16 ml in 2 separate syringes.

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DISCUSSION

Review of the literature shows a slight preponder-ance (60%) of left-sided orbital teratomas and afemale to male ratio of 2:1.1–3,5 Orbital teratomasusually grow rapidly after birth, causing extensiveproptosis and complications, such as globe rupturesecondary to corneal exposure and melting.1–3,5 Opticatrophy can also develop when the tumour engulfsthe optic nerve.5 This tumour occurs in healthy full-term infants, usually as a unilateral lesion. Only onebilateral case has been reported.1,2 The bony orbit candouble or triple in volume as a result of tumourgrowth, resulting in facial deformities. This rapidgrowth rate may arouse the suspicion of a malignantorbital tumour. However, most orbital teratomas arehistopathologically benign lesions, with only a fewmalignant cases reported.4,6 Rarely, a slowly growingorbital teratoma can appear in adulthood.

Orbital imaging studies of orbital teratomasusually show a well-circumscribed mass with acystic component and foci of calcification.1-3,5 Thetumour is rarely solid.2 Histopathologically, themajority of teratomas are composed of tissues repre-senting at least two, but usually three embryo-nic layers, including ectoderm, mesoderm andendoderm. The term ‘teratoid’ refers to growthsinvolving only two germ layers. If, as in our case, theneoplastic tissue is uniformly mature, resemblingthe adult tissues, the tumour is defined as a matureteratoma. Tumours containing any immature orembryonal tissue with mitotic figures are designatedas immature teratomas,1–6 and they can displaymalignant behaviour with metastasis.2

The predominant germ cell type observed inorbital teratomas is surface ectoderm-producingsquamous epithelial-lined cysts filled with keratinand adnexal structures, such as hair follicles andsweat glands.2 Neuroectodermal tissues includeprimitive neural tubes, choroidal plexus, ganglia andglial elements.2 Our case had choroidal plexus andglial elements. Mesoderm is the second mostcommon germ cell layer, represented by such tissuesas muscle, bone, cartilage and fat.2 Our case hadcartilage and fat as mesodermal components. Endo-derm is the least common component of teratomas,and it may produce gastrointestinal tissue cysts orcysts lined with respiratory-type pseudostratifiedcolumnar epithelium.2 Our case had mucinous cili-ated epithelium resembling respiratory epithelium.The rapid growth observed in the majority of orbitalteratomas can be explained by the accumulation ofglandular secretions within cystic spaces.1–3,5

In the past, orbital teratomas have usually beentreated with exenteration with preservation of theeyelids.5,7 However, if the globe is intact, globe-conserving treatments via various orbitotomyapproaches are indicated.1,2,7 If no eye or optic nerveis present, or when there is extreme proptosisbecause of massive teratoma encasing the globe andfilling the orbital contents, exenteration is necessary.Mature cystic teratomas elsewhere in the body havebeen reported to demonstrate malignant transforma-tion into squamous cell carcinoma many years afterinitial diagnosis.8 However, there has been no evi-dence so far that eye-conserving treatment is associ-ated with malignant degeneration in mature orbitalteratomas.

Figure 2. Photomicrograph demon-strating various sections of the cyst wall.(a) The cyst wall is lined with keratinizingsquamous epithelium (upper left corner).Small hair follicles are seen beneath theepithelium. The lower part of the figuredemonstrates mature glial elements (hae-matoxylin and eosin, original magnifica-tion ¥40). (b) Small foci of mucinousciliated epithelium resembling respiratoryepithelium are noted on the inner surfaceof the cyst wall (haematoxylin and eosin,original magnification ¥200). (c) Choroidalplexus-like organization is seen (haema-toxylin and eosin, original magnification¥40). (d) Small foci of cartilage are noted(haematoxylin and eosin, original magnifi-cation ¥100).

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We emphasize the importance of decompressingthe orbital teratoma before dissecting it from theperiorbital tissues if the tumour has a major cysticcomponent. With this approach, we were able tocompletely excise the tumour without damaging theeye in our patient.

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4. Assalian A, Guy A, Codere F. et al. Congenital orbitalteratoma: a clinicopathological case report includingimmunohistochemical staining. Can J Ophthalmol 1994;29: 30–3.

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6. Garden JW, McManis J. Congenital orbital-intracranialteratoma with subsequent malignancy: case report. Br JOphthalmol 1986; 70: 111–3.

7. Spinelli HM, Crisuolo GR, Tripps M, Buckkley PJ.Massive orbital teratoma in the newborn. Ann Plast Surg1993; 31: 453–8.

8. Griffiths D, Wass J, Look K, Sutton G. Malignant degen-eration of a mature cystic teratoma five decades afterdiscovery. Gynecol Oncol 1995; 59: 427–9.

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