0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Clinicopathologic Report |

Intraocular Neoplastic Cyst From Mucoepidermoid Carcinoma of the Conjunctiva FREE

Kaan Gündüz, MD; Carol L. Shields, MD; Jerry A. Shields, MD; Gary Mercado, MD; Ralph C. Eagle Jr, MD
[+] Author Affiliations

From the Oncology Service (Drs Gündüz, C. L. Shields, J. A. Shields, and Mercado) and Pathology Department (Dr Eagle), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pa.


Section Editor: W. Richard Green, MD

More Author Information
Arch Ophthalmol. 1998;116(11):1521-1523. doi:10.1001/archopht.116.11.1521.
Text Size: A A A
Published online

An 89-year-old woman with recurrent conjunctival mucoepidermoid carcinoma developed intraocular inflammation and an elevated fundus lesion that simulated choroidal detachment in her affected right eye. Intraocular invasion of squamous cell carcinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recurrence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst.

Figures in this Article

Mucoepidermoid carcinoma arises mainly in the salivary glands, but has also been noted in the upper respiratory tract and rarely in other sites.1 In the ocular region, it can affect the eyelid, conjunctiva, caruncle, lacrimal gland, lacrimal sac, and the paranasal sinuses.1 Conjunctival mucoepidermoid carcinoma has a tendency to recur after excision and invade the eye and the orbit.15 We describe a patient with recurrent conjunctival mucoepidermoid carcinoma that demonstrated intraocular invasion and formed a large intraocular cyst lined by neoplastic cells.

An 89-year-old woman developed an exophytic papillary conjunctival lesion in her pseudophakic right eye, affecting the nasal bulbar and limbal conjunctiva between the 2-o'clock and 4-o'clock positions. The lesion was treated with wide excision and cryotherapy. Histopathologic examination revealed conjunctival squamous cell carcinoma with focal mucin production, consistent with mucoepidermoid carcinoma (Figure 1). She had a recurrence 3 months later (Figure 2), and was again treated with wide excision and cryotherapy. A wound leak through scleral dehiscence was discovered during the second surgery but was successfully treated with a patch graft of Tenon fascia and conjunctiva.

Place holder to copy figure label and caption
Figure 1.

Conjunctival biopsy specimen shows conjunctival squamous cell carcinoma with focal mucin production (arrow) (periodic acid–Schiff, original magnification ×50).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Anterior segment view shows the recurrent mucoepidermoid carcinoma at the nasal conjunctiva and limbus (arrows).

Graphic Jump Location

The patient had ocular pain 2 months later. Ophthalmic examination disclosed a red epibulbar mass at the prior site. There was no wound leak or hypotony, but marked intraocular inflammation was found. An elevated fundus lesion resembling choroidal detachment was observed ophthalmoscopically through hazy, inflammed media. The mass was located inferiorly between the 5-o'clock and 7-o'clock positions and measured approximately 10×10 mm in basal diameter. B-scan ultrasonography showed a 9.5-mm-thick, acoustically hollow cystic lesion in the suprauveal space (Figure 3). A neoplastic cyst caused by intraocular invasion of the conjunctival malignant neoplasm was suspected based on these findings. The patient underwent modified enucleation with wide tenonectomy and conjunctivectomy to include the recurrent bulbar tumor.

Place holder to copy figure label and caption
Figure 3.

B-scan ultrasonogram shows a 9.5-mm-thick, acoustically hollow intraocular cyst (arrow).

Graphic Jump Location

Results of a histopathologic examination showed that the conjunctival carcinoma had invaded the interior of the eye through a scleral wound. The tumor had grown posteriorly through a cyclodialysis cleft into the supraciliary and suprachoroidal space, forming a neoplastic cyst (Figure 4). The cyst was lined by tumor cells growing on the inner surface of the sclera and the outer surface of the detached uvea. No mucin production was identified intraocularly or in the tumor on the outer surface of the globe.

Place holder to copy figure label and caption
Figure 4.

View of neoplastic cyst lumen (*) in suprauveal space lined by squamous cell carcinoma (single arrows) (at left). Tumor lining cyst is seen at higher magnification (at right). Tongues of tumor cells infiltrate choroidal stroma. Double arrows indicate retinal pigment epithelium; S, sclera. (hematoxylin-eosin, left, original magnification ×10, right, original magnification ×100).

Graphic Jump Location

The patient developed massive orbital recurrence 1 year after enucleation. An eyelid-splitting orbital exenteration was performed. Histopathologically, the tumor exhibited squamous cell carcinoma but no mucus-producing cells were evident. The patient had no further recurrence and died 2 years later of cardiac disease.

Squamous cell carcinoma of the conjunctiva generally occurs in older individuals with a history of excessive sun exposure. The tumor typically is located in the interpalpebral region near the corneoscleral limbus. Squamous cell carcinoma of the conjunctiva tends to be superficially invasive. Deep intraocular invasion is uncommon.68 Mucoepidermoid carcinoma, a variant of squamous cell carcinoma, is more locally aggressive. In a review of 12 cases of conjunctival mucoepidermoid carcinoma from the English-language literature, 6 cases were found to have intraocular invasion and 4 cases had orbital involvement. No intraocular or orbital involvement was found in the remaining 2 patients.5 Intraocular invasion in conjunctival squamous cell carcinoma may simulate intraocular inflammation.8 Similarly, the intraocular invasion was heralded by marked intraocular inflammation in our patient.

The recommended treatment of conjunctival squamous cell carcinoma and mucoepidermoid carcinoma includes alcohol corneal epitheliectomy, wide surgical excision with tumor-free margins, and cryotherapy of the remaining uninvolved margins.9 Radiation treatment, either with β-irradiation or plaque radiotherapy, has been advocated to reduce the risk of recurrence in conjunctival mucoepidermoid carcinoma.5

Our patient had 2 unusual features. In the reported cases of intraocular invasion by conjunctival mucoepidermoid carcinoma, the tumor generally involved the anterior uvea as a solid mass and there was no evidence of a fundus lesion.15 The occurrence of a neoplastic cyst in the suprauveal space that simulated a choroidal detachment, as in our patient, is exceptionally rare. In addition the intraocular invasion from conjunctival mucoepidermoid carcinoma is generally characterized by the presence of mucus-secreting cells.15 It has been suggested that intraocular enviromental factors may play a role in this type of tissue differentiation. The intraocular invasion in our patient was composed of only squamous epithelial cells without mucin production.

Recurrent conjunctival mucoepidermoid carcinoma generally exhibits fewer mucus-secreting cells than the original tumor.1 The recurrent conjunctival and orbital tumors were composed entirely of squamous carcinoma cells in our patient.

A prior report3 noted that the scleral defect from prior surgery predisposed the eye to the development of intraocular invasion through recurrent conjunctival squamous cell carcinoma. The scleral defect observed during our patient's second conjunctival excision probably also played a role in the development of intraocular invasion. We believe that the defect was related to scleral necrosis from invasive tumor, as the margins were irregular and attenuated. Intraocular inflammatory signs in a patient with prior excision of conjunctival mucoepidermoid carcinoma should raise suspicion of intraocular invasion by the tumor.

Accepted for publication July 8, 1998.

This study was supported by the Pennsylvania Lion's Sight Conservation and Eye Research Foundation, Philadelphia (Drs Gündüz, C. L. Shields, and J. A. Shields), the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J. A. Shields), the Eye Tumor Research Foundation (Dr C. L. Shields), Philadelphia, and the Macula Foundation (Drs Gündüz, C. L. Shields, J. A. Shields), New York, NY.

Reprint requests: Jerry A. Shields, MD, Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.

Rao  RAFont  RL Mucoepidermoid carcinoma of the conjunctiva: a clinicopathologic study of five cases. Cancer. 1976;381699- 1709
Link to Article
Brownstein  S Mucoepidermoid carcinoma of the conjunctiva with intraocular invasion. Ophthalmology. 1981;881226- 1230
Link to Article
Searl  SSKrigstein  HJAlbert  DMGrove  AS  Jr Invasive squamous cell carcinoma with intraocular mucoepidermoid features: conjunctival carcinoma with intraocular invasion and diphasic morphology. Arch Ophthalmol. 1982;100109- 111
Link to Article
Gamel  JWEiferman  RAGuibor  P Mucoepidermoid carcinoma of the conjunctiva. Arch Ophthalmol. 1984;102730- 731
Link to Article
Carrau  RLStillman  ECanaan  RE Mucoepidermoid carcinoma of the conjunctiva. Ophthal Plast Reconstr Surg. 1994;10163- 168
Link to Article
Shields  JAShields  CL Conjunctival squamous cell carcinoma. Shields  JAShields  CLeds.Atlas of Eyelid and Conjunctival Tumors Philadelphia, Pa Lippincott-Raven PublishersIn press
Iliff  WJMarback  RGreen  WR Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol. 1975;93119- 122
Link to Article
Li  WWPettit  THZakka  KA Intraocular invasion by papillary squamous cell carcinoma of the conjunctiva. Am J Ophthalmol. 1980;90697- 701
Shields  JAShields  CLDe Potter  P Surgical approach to conjunctival tumors: the 1994 Lynn B. McMahan lecture. Arch Ophthalmol. 1997;115808- 85
Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.

Conjunctival biopsy specimen shows conjunctival squamous cell carcinoma with focal mucin production (arrow) (periodic acid–Schiff, original magnification ×50).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Anterior segment view shows the recurrent mucoepidermoid carcinoma at the nasal conjunctiva and limbus (arrows).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.

B-scan ultrasonogram shows a 9.5-mm-thick, acoustically hollow intraocular cyst (arrow).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 4.

View of neoplastic cyst lumen (*) in suprauveal space lined by squamous cell carcinoma (single arrows) (at left). Tumor lining cyst is seen at higher magnification (at right). Tongues of tumor cells infiltrate choroidal stroma. Double arrows indicate retinal pigment epithelium; S, sclera. (hematoxylin-eosin, left, original magnification ×10, right, original magnification ×100).

Graphic Jump Location

Tables

References

Rao  RAFont  RL Mucoepidermoid carcinoma of the conjunctiva: a clinicopathologic study of five cases. Cancer. 1976;381699- 1709
Link to Article
Brownstein  S Mucoepidermoid carcinoma of the conjunctiva with intraocular invasion. Ophthalmology. 1981;881226- 1230
Link to Article
Searl  SSKrigstein  HJAlbert  DMGrove  AS  Jr Invasive squamous cell carcinoma with intraocular mucoepidermoid features: conjunctival carcinoma with intraocular invasion and diphasic morphology. Arch Ophthalmol. 1982;100109- 111
Link to Article
Gamel  JWEiferman  RAGuibor  P Mucoepidermoid carcinoma of the conjunctiva. Arch Ophthalmol. 1984;102730- 731
Link to Article
Carrau  RLStillman  ECanaan  RE Mucoepidermoid carcinoma of the conjunctiva. Ophthal Plast Reconstr Surg. 1994;10163- 168
Link to Article
Shields  JAShields  CL Conjunctival squamous cell carcinoma. Shields  JAShields  CLeds.Atlas of Eyelid and Conjunctival Tumors Philadelphia, Pa Lippincott-Raven PublishersIn press
Iliff  WJMarback  RGreen  WR Invasive squamous cell carcinoma of the conjunctiva. Arch Ophthalmol. 1975;93119- 122
Link to Article
Li  WWPettit  THZakka  KA Intraocular invasion by papillary squamous cell carcinoma of the conjunctiva. Am J Ophthalmol. 1980;90697- 701
Shields  JAShields  CLDe Potter  P Surgical approach to conjunctival tumors: the 1994 Lynn B. McMahan lecture. Arch Ophthalmol. 1997;115808- 85
Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 8

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
PubMed Articles