An Uncommon Malignant Neoplasm of the Caruncle Report of a Case of Undifferentiated Carcinoma

CLINICOPATHOLOGIC REPORT

SECTION EDITOR: W. RICHARD GREEN, MD An Uncommon Malignant Neoplasm of the Caruncle Report of a Case of Undifferentiated Carcinoma

Dennis S. C. Lam, FRCS, FRCOphth; Ka F. To, FRCPA; Dorothy S. P. Fan, MD; Wah Cheuk, MD

he caruncle is an uncommon site for the occurrence of neoplasm despite its diverse histological composition of conjunctival, lacrimal, and skin tissues. The following case report describes a 66-year-old man who suffered from a primary undifferentiated carcinoma of the left caruncle. He remained well with no evidence of recurrence 24 months Tafter a complete surgical excision. To our knowledge, this could represent the first case of undifferentiated carcinoma of the caruncle to be reported in the English literature. Early detection and complete excision for this type of lesion could lead to a satisfactory clinical outcome. Arch Ophthalmol. 1998;116:374-376

Lesions involving the caruncle are rare; cm. It was not adherent to the orbital bone however, a malignant neoplasm of the car- or the deeper structures. The upper and uncle is even rarer. Luthra et al1 reported the lower canaliculi, including their punc- only 112 caruncular lesions, 4 of which tal openings, were uninvolved. No sur- were malignant neoplasm, in a clinico- rounding satellite lesions were found. Vi- pathological review during a 52-year pe- sual acuity was 20/50 OD and 10/200 OS. riod. Other series have similarly docu- There were bilateral cataracts, with the left mented an overall 4% frequency of cataract being more severe. The patient also malignant neoplasm among caruncular le- had old trachoma with some corneal scar sions.2-4 The more commonly occurring ca- in the left eye. No other ocular abnormali- runcular tumors are nevus and papil- ties or regional adenopathy were found. loma, while sebaceous carcinoma is the Medical history and systemic physical ex- most common malignant tumor.5 Other amination findings were unrevealing. A rare tumors include malignant mela- computed tomographic scan of the orbit noma,1 oncocytoma,2,3 mucoepidermoid revealed a large tumor in the anterome- carcinoma,6 sebaceous carcinoma,7 adeno- dial aspect of the orbit indenting and dis- squamous carcinoma,8 and tumors of mes- placing the globe to the left (Figure 2). enchymal or vascular origin.1 We report There was no bony involvement and the a rare case of primary undifferentiated car- adjacent nasal cavities were clear. Re- cinoma of the caruncle. sults of systemic investigations for dis- seminated malignant neoplasms were nor- REPORT OF A CASE mal. The serum IgA titer for Epstein-Barr virus viral capsid antigen was negative. A A 66-year-old man had a 3-month his- complete excision of the tumor was per- tory of progressive enlargement of a formed under general anesthesia. The sur- reddish nodular lesion over the left gical margins, assessed by frozen section caruncular region (Figure 1). The firm were declared to be free of tumors. No ad- erythematous mass was well circum- juvant chemotherapy or radiotherapy was scribed, measuring about 2.5ϫ1.5ϫ2.0 given. The patient was symptom free and there was no evidence of recurrence or me- From the Eye Unit, Department of Ophthalmology & Visual Sciences (Drs Lam, Fan, tastasis at the latest follow-up, 24 months and Cheuk) and the Department of Anatomical & Cellular Pathology (Dr To), Prince after the operation. of Wales Hospital, the Chinese University of Hong Kong, Shatin, New Territory, Hong Histological examination showed an Kong. infiltrative tumor comprising clusters and

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 Figure 1. Left eye showing a well–circumscribed nodular mass over the left Figure 2. Computed tomographic scan shows a tumor at the medial side of caruncle. the left orbit with no surrounding bony involvement.

Figure 3. Clusters and lobules of undifferentiated carcinoma cells were Figure 4. The carcinoma cells exhibited marked nuclear pleomorphisms, present beneath the stratified squamous epithelium (hematoxylin-eosin, hyperchromasia, distinct nucleoli, and scanty amphophilic cytoplasms original magnification ϫ10). (hematoxylin-eosin, original magnification ϫ200).

lobules of undifferentiated carci- phoid marker (leukocyte common lower canaliculi.10 When fully devel- noma cells lying beneath the strati- antigen), melanocytic marker (S- oped, the caruncle is lined by non- fied squamous epithelium 100 protein), muscle marker (des- keratinized epithelium that is simi- (Figure 3). The tumor cells con- min), and neuroendocrine markers lar to conjunctival epithelium, while sisted of marked nuclear pleomor- (neuron-specific enolase, chro- it harbors all the skin appendageal el- phisms, hyperchromasia, distinct mograin) were all negative. The Ep- ements including hair follicles, seba- nucleoli, and scanty amphophilic cy- stein-Barr virus–encoded RNAs, ceous glands, and sweat glands.11 Ac- toplasms (Figure 4). Frequent mi- which are untranslated RNAs pres- cessory lacrimal glands can also be totic activity and apoptosis were evi- ent in Epstein-Barr virus–infected seen in this area. As a result, car- dent. No squamous or glandular cells, were absent in the specimen.9 uncle may spawn any tumor or cyst differentiation was seen and no cy- The tumor was classified as an un- that occurs in the conjunctiva, skin, toplasmic vacuolation was identi- differentiated carcinoma. or lacrimal gland.12,13 Surprisingly, fied. Histochemical stain (oil- these are all rare, especially malig- red-O) for lipid material in frozen COMMENT nant neoplasm, which might be at- section was negative, excluding tributed to the small anatomic size of the possibility of sebaceous carci- The caruncle has a unique embryo- the caruncle. noma. Immunohistochemical study logical origin, with contribution from To our knowledge, this is the findings for epithelial marker the lower eyelid and its appendages first report of undifferentiated carci- (AP1/AE3) were positive while lym- admixed with the ingrowth from the noma of the caruncle. We regard this

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/29/2021 tumor as a primary carcinoma of car- and radiotherapy has not been prop- 5. Maino JH, Richer SP, McGregor D. Caruncle le- uncle because of the location of the erly evaluated. In our patient, com- sions: the use of histological evaluation in deter- mining a definitive diagnosis. J Am Optom As- tumor, the exclusion of other pri- plete excision with clear surgical soc. 1983;54:521-524. mary tumors in the nearby struc- margins has achieved a 24-month 6. Margo CE, Weitzenkorn DE. Mucoepidermoid car- tures by the computed tomographic symptom-free period with no evi- cinoma of the conjunctiva: report of a case in a scan, and the lack of primary tumor dence of recurrence. The possibil- 36-year-old with paranasal sinus invasion. Oph- thal Surg. 1986;17:151-154. found or developed in other parts of ity of late metastasis is still present. 7. Folberg R, Whitaker DC, Tse DT, Nerad JA. Re- 1 the body during the follow-up pe- Luthra et al have reported a case of current and residual sebaceous carcinoma after riod. There is, however, at least 1 case sebaceous carcinoma of caruncle that Mohs’ excision of the primary lesion Am J Oph- each of undifferentiated carcinomas recurred only 10 years after initial thalmol. 1987;103:817-823. excision. Apart from the usual routes 8. Nylander AG, Atta HR. Adenosquamous carci- reported in the orbit and maxillary noma of the lacrimal caruncle: a case report. Br J sinus in a 20-year review of orbital of metastatic spread, caruncular tu- Ophthalmol. 1986;70:864-866. exenteration.14 In addition, 1 case of mor may shed along the intracana- 9. Chang KL, Chen YY, Shibata D, Weiss LM. De- anaplastic tumor was reported by licular pathway.17 scription of an in situ hybridization methodology Pecorella and Garner3 in their series for detection of Epstein-Barr virus RNA in paraffin- embedded tissues, with a survey of normal and of caruncular tumors during a 59- Accepted for publication November 11, neoplastic tissues. Diagn Mol Pathol. 1992;1:246- year period. No further details or the 1997. 255. exact lineage of the tumor was pro- This study was supported in part 10. Duke-Elder S, Cooke C. Normal and abnormal de- vided. Leung et al15 reported a case by the Mrs Annie Wong Eye Founda- velopment. In: Duke-Elder S, ed. System of Oph- thalmology. St Louis, Mo: Mosby–Year Book Inc; of undifferentiated carcinoma in the tion. 1963;234-235. lacrimal sac with metastasis to the Reprints: Dennis S. C. Lam, FRCS, 11. Spencer WH, Zimmerman LE. Conjunctiva. In: cervical lymph node at presenta- FRCOpth, Department of Ophthalmol- Spencer WH, ed. Ophthalmic Pathology: An At- tion. The tumor was accompanied by ogy & Visual Sciences, Prince of Wales las and Textbook. Philadelphia, Pa: WB Saun- intense lymphoid infiltrate and ders Co; 1985:109-114. Hospital, The Chinese University of 12. Evans WH Tumor of the lacrimal caruncle: a study showed the Epstein-Barr virus, com- Hong Kong, Shatin, New Territory, of 200 collected cases. Arch. Ophthalmol. 1940; patible with lymphoepithelioma- Hong Kong (e-mail: dennislam@ 24:83. like carcinoma.16 The current case cuhk.edu.hk). 13. Reese AB. Epithelial tumors of the lid, conjunc- does not exhibit the histological fea- tiva, cornea, and lacrimal sac. In: Reese AB, ed. tures of lymphoepithelioma-like car- Tumors of the Eye. London, England: Harper & REFERENCES Row Publishers Inc; 1976:59-60. cinoma and is Epstein-Barr virus 14. Levin PS, Dutton JJ. A 20-year series of orbital negative. exenteration. Am J Ophthalmol. 1991;112:496- Given the rarity of carcinomas 1. Luthra CL, Doxanas MT, Green WR. Clinical patho- 501. of the caruncle, little is known about logical review. Surv Ophthalmol. 1978;23:183-195. 15. Leung SY, Chung LP, Ho CM, Yuen ST, Wong MP, 2. Shields CL, Shields JA, White D, Augsburger JJ. Kwong WK. An Epstein-Barr virus positive undif- their natural history and the plan of Types and frequency of lesions of the caruncle. ferentiated carcinoma in the lacrimal sac. Histo- management is largely empirical. Am J Ophthalmol. 1986;102:771-778. pathology. 1996;28:71-75. The mainstay of treatment tends to 3. Percorella L, Garner A. Ostensible oncocytoma of 16. Weiss LM, Gaffey MJ, Shibata D. Lymphoepithe- be surgical excision8-12 with orbital accessory lacrimal glands. Histopathology. 1997; lioma-like carcinoma and its relationship to Ep- 30:264-270. stein-Barr virus. Am J Clin Pathol. 1991;96:156- exenteration in reserve for late pre- 4. Hirsch C, Holz FG, Tetz M, Volcker HE. Clinical as- 158. 8 9 sentation and local recurrence. The pects and histopathology of caruncular tumors. 17. Kielar RA. Sebaceous carcinoma of the caruncle. efficacy of adjuvant chemotherapy Klin Monatsbl Augenheilkd. 1997;210:153-157. South Med J. 1975;68:347-50.

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