Report of a Conjunctival Myxoma Case and Review of the Literature

CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES

SECTION EDITOR: W. RICHARD GREEN, MD

1 case (Ͻ0.001%).5 Because of its rar- mass had 2 parts, including a solid Report of a Conjunctival ity, conjunctival myxoma can simu- basal part measuring 12ϫ11ϫ6 Myxoma Case and Review late other conjunctival tumors, such mm and a cystic apical part measur- of the Literature as amelanotic nevus, amelanotic mela- ing 5ϫ5ϫ2 mm. The clinical dif- noma, squamous cell carcinoma, ferential diagnosis included amela- lipoma, and cyst.6 We describe a notic nevus, amelanotic melanoma, Myxoma is a benign soft tissue 31-year-old African American man myxoma, fibrous histiocytoma, and tumor that presumably derives from who had a yellow, translucent sub- lipoma. The lesion was excised primitive mesenchyme. It is char- conjunctival mass that proved to be using the previously described acterized histopathologically by a conjunctival myxoma on histo- no-touch technique.7 abundant mucoid material, a loose pathological examination. Histopathological examination meshwork of reticulin fibers, and revealed a lesion composed of loose, relatively small numbers of spindle- Report of a Case. A 31-year-old myxoid areas that alternated with 1 and stellate-shaped cells. The African American man had a 2-year areas of spindle and stellate cells, stromal matrix is rich in glu- history of conjunctival swelling in which was consistent with benign cosaminoglycans and hyaluronic his left eye. The lesion was diag- conjunctival myxoma (Figure 2). acid, and it is sparse in collagen and 1 nosed as a conjunctival cyst, and Both cellular groups had moder- vascular structures. Myxoma can drainage was attempted. Eighteen ately large hyperchromatic and appear as localized disease or as a months later, the swelling wors- slightly pleomorphic nuclei and were component of Carney complex, ened and a solid conjunctival mass embedded in a loose mucoid stroma. which includes cutaneous and car- was noted, prompting referral to the The stroma contained reticulin fi- diac myxomas, multiple pig- Oncology Service, Wills Eye Hospi- bers, sparse small blood vessels, and mented lesions, and endocrine over- tal, Philadelphia, Pa. The patient had collagen fibers (Figure 2). Some of activity, leading to significant morbidity and mortality.2 Myxoma no history of ocular trauma and was the tumor cells had clear, circular, is the most common primary car- otherwise healthy. intranuclear inclusions, and some diac tumor. This tumor can arise in The visual acuity was 20/20 OU. contained clear cytoplasmic vacu- the skeletal muscle, bone, skin, geni- The right eye was normal. External oles. Numerous mast cells were scat- tourinary system, gastrointestinal examination of the left eye dis- tered throughout the myxoid stroma tract, and nasal sinuses.3 It can rarely closed a well-circumscribed, yellow- (Figure 3). involve ocular structures and has pink, translucent mass on the nasal Systemic evaluations of the been recognized in the eyelid, bulbar conjunctiva (Figure 1). The patient for cardiac, endocrine, and orbit, and conjunctiva.4-6 In a review of 2455 conjunctival A B lesions submitted to an ophthalmic pathology laboratory, only 4 patients (0.002%) were found to have conjunctival myxoma.4 In a clinical review of 1643 patients with conjunctival lesions, myxoma was found in

Figure 1. Anterior segment of the left eye showing a well-circumscribed, yellow-pink, translucent mass with a solid basal part and a Figure 2. Conjunctival myxoma. The hypocellular stroma contains delicate collagen fibers cystic apical part on the nasal bulbar (hematoxylin-eosin, original magnification ϫ50) (A) and abundant mucoid material confirmed by intense conjunctiva. positive staining with Alcian blue (original magnification ϫ20) (B).

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©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 cular structures, and mature colla- excisional biopsy. Of the 22 pub- gen fibers.11,13,14 The mucinous ma- lished cases with conjunctival trix is composed predominantly of myxoma, no recurrence or malig- hyaluronic acid, with a lesser nant transformation after exci- amount of chondroitin sulfate. sional biopsy of the lesion was ob- Ultrastructurally, myxoma cells have served.8-21 In a review of 58 patients intracytoplasmic vacuoles and with myxomas of the soft tissue, only cytoplasmic filaments arranged in 2 (3%) had recurrence after 8 to 10 loose, wavy bundles.11,13,14 These months following excision. These 2 Figure 3. Hypocellular myxoma contains vacuoles are consisted with dilated patients underwent reexcision, and multinucleated spindle cells with intranuclear rough endoplasmic reticulum. It both were free of recurrent tumor af- vacuoles. Arrow indicates mast cell seems that the mucoid matrix and ter a mean of 5 years. Recurrence of (hematoxylin-eosin, original magnification ϫ250). extracellular fibrils are produced by myxoma can be related to inad- cutaneous abnormalities including myxoma cells. There are also equate excision, multicentricity of echocardiography and levels of thy- intranuclear vacuoles that are con- the tumor, the presence of genetic roid-stimulating hormone, growth sistent with invaginations of predisposition, or malignant trans- hormone, and adrenocorticotropic markedly folded nuclear mem- formation. In a review of 107 hormone were negative. After 11 brane. Immunohistochemical stud- cardiac myxomas submitted to a 13,14,17,21 years of follow-up, the patient re- ies showed myxoma cells pathology laboratory, atypical mained healthy without recur- were immunoreactive for vimentin cells simulating malignancy were ␣ 22 rence of the conjunctival mass or evi- and smooth muscle actin but nega- observed in only 3%. dence of a systemic abnormality. tive for S-100 protein, desmin, and Myxomas can be a component of myoglobulin. Carney complex, Mazabraud syn- Comment. Myxoma rarely occurs in Conjunctival myxoma differs clini- drome (bone fibrous dysplasia and the conjunctiva. To our knowl- cally and histopathologically from intramuscular myxoma), and edge, only 22 well-documented cases myxomas of extraocular soft tissues McCune-Albright syndrome (cafe´au of conjunctival myxoma have been in some aspects. Unlike extraocular lait spots and multinodular goiter). published in the English literature soft tissue myxomas, conjunctival Carney complex is an autosomal myxoma often contains cyst-like cavi- dominant syndrome that includes 2 (Table).8-21 The tumor usually occurs in adults and is uncommon in ties. The latter were noted in 9 of the or more of the following condi- 22 published cases.8-21 In our case, the tions: multiple foci of mucocutane- children. The mean age of the 22 patient was diagnosed as having a ous pigmentation (face, trunk, lips, published cases was 48 years (me- conjunctival cyst, and drainage was conjunctiva), cardiac and extracar- dian age, 50 years; age range, 18-80 attempted before referral to our ser- diac (skin, breast) myxomas, years), and 2 patients (9%) were vice. Additionally, on histopathologi- endocrine overactivity (including younger than 20 years.8-21 Both sexes 8-21 cal examination, mast cells can be ob- Cushing syndrome), pituitary ad- were affected equally. Similarly, served in conjunctival myxoma.11,13,14 enoma, unusual testicular tumors, a review of 58 patients who all had The differential diagnosis of con- and psammomatous melanotic soft tissue myxomas revealed a mean junctival myxoma includes nevus, schwannoma.23 Patients with age of 55 years, with only 1 patient 3 amelanotic melanoma, fibrous his- myxoma, mucocutaneous pigmen- (2%) younger than 20 years. tiocytoma, cyst, lymphangioma, myx- tation, and/or a family history of Conjunctival myxoma is typi- oid neurofibroma, spindle cell li- Carney complex should undergo a cally seen as a well-circumscribed, poma, rhabdomyosarcoma, and systemic evaluation including yellow-pink, translucent, cystic myxoid liposarcoma.6 Unlike con- echocardiography, computed to- 8-21 and/or solid mass. Of the 22 re- junctival amelanotic nevus and mography or magnetic resonance ported cases, the conjunctival mass melanoma, myxoma does not have imaging of the body, testicular ul- was described as well-circum- prominent intrinsic vascularity or pig- trasound, analysis of the thyroid- scribed in 20 cases (91%) and dif- mentation.6 Myxoid neurofibroma stimulating hormone level, adreno- 8-21 fuse in 2 cases (9%). The color of can have a similar clinical picture. It corticotropic hormone level, and the conjunctival mass, when men- contains cells with spindle-shaped nu- growth hormone level, and genetic tioned, was yellow in 9 cases (50%), clei with thick, wavy bundles of col- analysis (PRKAR1A gene at chromo- pink in 8 cases (44%), and white in lagen and can be associated with sys- some 17q2).23 Ophthalmic manifes- 1 case (6%).8-21 The conjunctival le- temic neurofibromatosis.11 Both tations of Carney complex include sion was translucent in 14 cases fibrous histiocytoma and myxoma facial and eyelid lentigines, pigmen- (64%) and solid in 8 cases (36%).8-21 stain positively with Alcian blue, tation on the caruncle or conjunc- Conjunctival myxoma has char- which is abolished by pretreatment tival semilunar fold, and eyelid acteristic histopathological fea- with hyaluronidase. Unlike fibrous myxoma.23,24 Kennedy et al15 de- tures.11,13,14 It consists of scattered, histiocytoma, myxoma does not show scribed a patient with Carney com- relatively small numbers of stel- vascularity or significant nuclear plex who had a myxoma of the late- and spindle-shaped cells dis- pleomorphism.1 palpebral conjunctiva and an addi- posed in a mucinous matrix with The preferred management of tional lesion on his eyelid margin. delicate reticulin fibers, sparse vas- conjunctival myxoma is complete None of the 22 published cases of

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Duration of Age, y/ Systemic Symptoms, Clinical Conjunctival Conjunctival Source Sex Disease Eye Symptoms mo Features Color Site Location Outcome Ffooks (1962)8 72/M† None OD Conjunctival 6 Rubbery, White Limbus Superior Stable mass circumscribed subconjunctival mass Doughman and 49/F† None OD Conjunctival 12 Rubbery, Yellow Limbus Temporal Stable Wenk (1970)9 mass circumscribed subconjunctival mass Stafford (1971)10 54/F† None OD Conjunctival 108 Translucent, Pink Limbus Temporal Stable cyst circumscribed subconjunctival mass Patrinely and 50/F None OD Conjunctival 24 Translucent, diffuse Yellow Bulbar Nasal Stable Green (1983)11 mass conjunctival mass 53/F None OS Conjunctival 12 Solid, circumscribed Yellow Bulbar Temporal Stable cyst conjunctival mass 18/F None OS Conjunctival 15 Semitranslucent, Pink Bulbar Superior Stable mass circumscribed conjunctival mass 42/F None OD Conjunctival 36 Translucent, Yellow Limbus Temporal Stable mass circumscribed subconjunctival mass Mottow-Lippa 54/M† None OS Conjunctival 288 Translucent, Pink Limbus Temporal Stable et al (1983)12 mass circumscribed subconjunctival mass Pe’er et al 18/F† None OD Conjunctival 4 Translucent, Pink Bulbar Nasal Stable (1984)13 mass circumscribed subconjunctival mass Pe’er and Hidayat 48/M None NA Conjunctival NA Conjunctival cyst Yellow Fornix Inferior Stable (1986)14 cyst 61/M None NA Conjunctival NA Solid, circumscribed NA Bulbar Temporal Stable growth subconjunctival mass 34/M None NA Conjunctival 36 Conjunctival cyst Yellow Bulbar Temporal Stable cyst 64/M None NA Conjunctival NA Conjunctival mass Pink Bulbar Temporal Stable mass 33/F None NA Conjunctival 8 Solid, circumscribed Pink Bulbar NA Stable mass conjunctival mass 20/M None NA Conjunctival 6 Translucent, Yellow Limbus Temporal Stable cyst circumscribed conjunctival mass 50/M None NA Conjunctival 24 Translucent, Yellow Bulbar Nasal Stable cyst circumscribed conjunctival mass 50/F None NA Conjunctival 12 Solid, circumscribed Pink Bulbar NA Stable mass conjunctival mass 76/M None NA Conjunctival NA Translucent, NA Limbus Superior Stable cyst circumscribed conjunctival mass 65/F None NA Conjunctival NA Translucent, diffuse NA Bulbar Temporal Stable mass conjunctival mass Kennedy et al 23/M Carney Conjunctival 4 Translucent, Yellow Palpebral Superior Stable (1987)15 complex mass circumscribed conjunctival mass Horie et al 80/F None OS Conjunctival 60 Solid conjunctival NA Bulbar Nasal Stable (1995)16 mass mass Ramaesh et al 36/M Zollinger-Ellison OD Conjunctival NA Translucent, Pink Bulbar Nasal Stable (2001)17 syndrome swelling circumscribed and thickness conjunctival mass of interatrial septum

Abbreviations: F, female; M, male; NA, not applicable; OD, right eye; OS, left eye. *Cases were published in the English literature and were well documented. For all of the patients, the therapy was excisional biopsy and the histopathological examination result was myxoma. †Patients were white.

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©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 conjunctival myxoma in the litera- 12. Mottow-Lippa L, Tso MOM, Sugar J. Conjunc- strate the possibility that absolute tival myxoma: a clinicopathologic study. ture were associated with Mazabraud Ophthalmology. 1983;90:1452-1458. alcohol caused the corneal toxicity or McCune-Albright syndromes.8-21 13. Pe’er J, Ilsar M, Hidayat A. Conjunctival myxoma: in this case. Insummary,conjunctivalmyxoma a case report. Br J Ophthalmol. 1984;68:618- 622. can appear as a well-circumscribed, 14. Pe’er J, Hidayat A. Myxomas of the conjunctiva. Report of a Case. A 79-year-old man translucent, yellow-pink conjunctival Am J Ophthalmol. 1986;102:80-86. hada3½-month history of a Seidel- 15. Kennedy RH, Flanagan JC, Eagle RC Jr, Car- mass in middle-aged patients. Man- ney JA. The Carney complex with ocular signs positive bleb leak and declining vi- agement is generally complete surgi- suggestive of cardiac myxoma. Am J Ophthalmol. sual acuity. Two and one-half years cal resection. 1991;111:699-702. earlier, he had undergone com- 16. Horie Y, Ikawa S, Nagata M, Tamai A. Myxoma bined phacoemulsification and tra- Hakan Demirci, MD of the conjunctiva: a case report and review of the literature. Jpn J Ophthalmol. 1995;39:77-82. beculectomy with 0.5 mg/mL mito- Carol L. Shields, MD 17. Ramaesh K, Wharton SB, Dhillon B. Conjunc- mycin C for 4 minutes under Ralph C. Eagle, Jr, MD tival myxoma, Zollinger-Ellison syndrome and abnormal thickening of the inter-atrial sep- retrobulbar anesthesia. On exami- Jerry A. Shields, MD tum: a case report and review of the literature. nation, his visual acuity had de- Eye. 2001;15:309-312. 18. Maucione L. Di una rara forma di tumore epib- creased from a baseline of 20/25 to Correspondence:DrC.L.Shields,On- ulbare (Mixoma puro) suttocongiuntivale. Arch 20/80. Applanation intraocular pres- cologyService,WillsEyeHospital,840 Ottamol. 1914;21:300-306. sure measured ranged between 5 and Walnut St, Philadelphia, PA 19107 19. Majewska-Cembrynska A, Majewski H. Sluzak nagalkowy. Klin Oczna. 1973;43:827-830. 10 mm Hg on serial examinations. (carol.shields@shieldsoncology 20. El-Rifai M, Ghaly AF. Myxoma of the conjunctiva. Slitlamp examination revealed a .com). Bull Ophthalmol Soc Egypt. 1975;68:31-35. large, avascular bleb with 2 epithe- 21. Konari K, Suzuki J, Oyachi H. Ocular pain as Financial Disclosure: None. the initial sign of conjunctival myxoma. Rin- lial defects that were briskly posi- Funding/Support: This work was sho Ganka. 1993;47:1361-1364. tive on Seidel test. The cornea supported by the Macula Founda- 22. Burke AP, Virmani R. Cardiac myxoma: a clinicopathologic study. Am J Clin Pathol. 1993; showed folds in the Descemet mem- tion, New York, NY (Dr C. L. Shields), 100:671-680. brane. No corneal guttata were seen. the Eye Tumor Research Founda- 23. Carney JA. Carney complex: the complex of Examinations of the cornea prior to tion, Philadelphia, Pa (Dr C. L. myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol. the bleb leak showed no signs of cor- Shields), the Noel T. and Sara L. 1995;14:90-98. neal guttata, early edema, or other Simmonds Endowment for Ophthal- 24. Kennedy RH, Waller RR, Carney JA. Ocular pig- disease. There was no sign of gut- mented spots and eyelid myxomas. Am J mic Pathology, Wills Eye Hospital, Ophthalmol. 1987;104:533-538. tata or other corneal disease in the Philadelphia (Dr Eagle), and the Paul fellow eye. The disc showed a 0.8 Kayser International Award of Merit cup. The macula was flat. in Retina Research, Houston, Tex The patient underwent revision of Corneal Decompensation (Dr J. A. Shields). trabeculectomy using a conjuncti- Following Bleb Revision val advancement flap under topical 1. Weiss SW, Goldblum JR, Franz M. Benign soft With Absolute Alcohol: tissue tumors of uncertain type. In: Enzinger and subconjunctival but not intra- FM, Weiss SW, eds. Soft Tissue Tumors. St Louis, Clinical Pathological cameral anesthesia. An incision was Mo: Mosby; 2001:1045-1053. Correlation 2. Carney JA, Gordon H, Carpenter PC, Shenoy made around the bleb and the sur- BV, Go VL. The complex of myxomas, spotty rounding tissues were freed from the pigmentation and endocrine overactivity. Medi- Topical alcohol has been com- underlying sclera. The bleb was de- cine (Baltimore). 1985;64:270-283. 3. Ireland DCR, Soule EH, Ivins JC. Myxoma of monly used to facilitate removal of epithelialized using 100% ethanol somatic soft tissues: a report of 58 patients, 3 corneal epithelium.1 More re- (absolute alcohol) soaked on a cot- with multiple tumors and fibrous dysplasia of bone. Mayo Clin Proc. 1973;48:401-410. cently, a technique for bleb revi- ton swab. We used a cotton swab 4. Grossniklaus HE, Green WR, Luckenbach M, sion has been described in which ab- rather than the cellulose spear origi- Chan CC. Conjunctival lesions in adults: a clini- solute alcohol is used topically to nally described2 because it had been cal and histopathologic review. Cornea. 1987; 6:78-116. remove the epithelium of a previ- observed that the anhydrous etha- 5. Shields CL, Demirci H, Karatza EC, Shields JA. ously existing bleb.2 Unlike other nol did not expand the spear, and Clinical survey of 1643 melanocytic and non- techniques in which the prior bleb thus little alcohol was applied with melanocytic conjunctival tumors. Ophthalmology. 2004;111:1747-1754. is excised, this technique has the it. More alcohol could be applied with 6. Shields JA, Shields CL. Fibrous, neural, xan- advantage of leaving the prior bleb the cotton that did absorb the alco- thomatous and myxomatous tumors. In: Shields JA, Shields CL, eds. Atlas of Eyelid and Con- in place while resurfacing it, thus hol. Residual epithelium was re- junctival Tumors. Philadelphia, Pa: Lippincott minimizing the potential for con- moved using a sharp blade. The con- Williams & Wilkins; 1999:277-288. traction that may otherwise occur junctival advancement flap was then 7. Shields JA, Shields CL, De Potter P. Surgical ap- proach to conjunctival tumors: the 1994 Lynn during formation of an entirely sutured into place at the limbus with B. McMahan Lecture. Arch Ophthalmol. 1997; new bleb. a combination of polyglactin su- 115:808-815. We report a case of a patient who tures at the ends of the flap and a mat- 8. Ffooks OO. Myxoma of the conjunctiva. Br J Ophthalmol. 1962;46:374-377. suffered acute corneal decompen- tress-style nylon suture at the mid- 9. Doughman DJ, Wenk RE. Epibulbar myxoma. sation following surgical bleb point of the flap. A circumferential Am J Ophthalmol. 1970;69:483-485. 10. Stafford WR. Conjunctival myxoma. Arch revision in which a large amount of relaxing incision through conjunc- Ophthalmol. 1971;85:443-444. absolute alcohol had been used to tiva was cut as posteriorly as pos- 11. Patrinely JR, Green WR. Conjunctival myxoma: de-epithelialize a leaking bleb. sible to relieve tension on the flap. a clinicopathologic study of four cases and a review of the literature. Arch Ophthalmol. 1983; Results of an experimental rabbit One week postoperatively, the 101:1416-1420. model are presented to demon- patient’s visual acuity measured

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