Adenoma of Nonpigmented Ciliary Epithelium with Smooth Muscle Differentiation

CLINICOPATHOLOGIC REPORT Adenoma of Nonpigmented Ciliary Epithelium With Smooth Muscle Differentiation

Jerry A. Shields, MD; Ralph C. Eagle, Jr, MD; Carol L. Shields, MD

35-year-old man underwent successful iridocyclectomy for a ciliary body mass that was subluxating the lens and causing a secondary cataract. Histopathologically the mass proved to be a spindle cell tumor, but leiomyoma and melanoma were ini- tially considered to be diagnostic possibilities. However, further studies and immu- Anohistochemical studies revealed that the tumor probably arose from the nonpigmented ciliary epithelium. The spindle cells represented smooth muscle differentiation within the mass. The final diagnosis was adenoma of the nonpigmented ciliary epithelium with smooth muscle differentiation. Arch Ophthalmol. 1999;117:117-119 Acquired adenoma of the nonpigmented ber and anterior vitreous cavity. There was ciliary epithelium (NPCE) is relatively rare a ciliary body mass superiorly that was sub- but recent authors have reviewed the lit- luxating the lens and producing a dense fo- erature on this tumor and have defined cal cortical cataract that partially obscured its clinical and histopathologic fea- the tumor (Figure 1). The posterior fun- tures.1-3 We describe a patient who had a dus was normal. Transillumination showed ciliary body tumor that was difficult to di- a shadow measuring 7 mm in diameter ex- agnose histopathologically because it was tending from the pars plicata to the midpor- composed mostly of amelanotic spindle tion of the pars plana. Fluorescein angiog- cells. Results of more detailed histopatho- raphy revealed early filling and late diffuse logic and immunohistochemical studies staining of the mass. B-scan ultrasonogra- showed that the tumor was an adenoma phy disclosed a pedunculated mass with me- of the NPCE with smooth muscle differ- dium internal reflectivity. entiation. See also page 113 REPORT OF A CASE Our clinical differential diagnoses in- A 35-year-old African American man was cluded ciliary body granuloma, adenoma referred for a ciliary body mass in his right of the NPCE, malignant melanoma, leio- eye. Prior to the detection of the tumor, myoma, and neurilemoma. Systemic evalu- he had been diagnosed as having intra- ation failed to reveal underlying granulo- ocular inflammation and was treated for matous disease such as tuberculosis or 1 month with systemic and topical anti- sarcoidosis. Because the patient failed to biotics and corticosteroids with no relief improve with antibiotic and corticoste- of his ocular pain or visual impairment. roid therapy, it was elected to locally resect the mass. Using a previously reported tech- His ocular and systemic histories were oth- 4 erwise unremarkable. nique, partial lamellar iridocyclectomy Best-corrected visual acuities were 20/ was performed without complications. The 60 OD and 20/20 OS. Intraocular pressures cataract progressed and 1 year later he had were 18 mm Hg in each eye. The affected cataract surgery with a resultant visual acu- right eye had fine keratic precipitates and ity of 20/20 OD. moderateflareandcellsintheanteriorcham- PATHOLOGIC FINDINGS From the Oncology Service (Drs J. A. Shields and C. L. Shields) and the Pathology Department (Dr Eagle), Wills Eye Hospital, Thomas Jefferson University, Grossly the tumor measured 7 ϫ 5 ϫ 4 Philadelphia, Pa. mm and rested on a base of normal cili-

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Figure 1. Clinical appearance of ciliary body mass producing a secondary Figure 2. Gross photograph of resected tumor showing amelanotic mass cataract. resting on the inner surface of the ciliary body.

Figure 3. Low-magnification photomicrograph showing the peripheral area Figure 4. Photomicrograph of spindle cells that composed most of the of viable cells and the central area of necrosis (hematoxylin-eosin, original tumor (hematoxylin-eosin, original magnification ϫ250). magnification ϫ10).

ary body and sclera 12 mm in diam- face of the ciliary body and did not middle molecular weight; 904, high eter (Figure 2). Low-magnifica- involve the stroma. Furthermore, molecular weight; and CAM 5.2, low tion microscopy showed that the continuity between the tumor and molecular weight) (Enzo Manufac- peripheral portion of the tumor was the NPCE was evident in a few sec- turers, New York, NY). The periph- composed of viable tumor cells that tions. A few bands of polarized epi- eral spindle cells showed intensely surrounded a large focus of central thelium that rested on periodic acid– positive immunoreactivity for both necrosis (Figure 3). The viable pe- Schiff–positive basement membrane muscle-specific actin and smooth ripheral region contained plump were observed near the base of the muscle actin (Figure 6), with the spindle cells with abundant eosino- tumor (Figure 5). ciliary musculature serving as an in- philic cytoplasm, fairly uniform nu- The tumor was studied with a ternal positive control. The latter ob- clei, and moderately prominent battery of immunohistochemical servation raised the diagnostic pos- nucleoli (Figure 4). Many spindle stains. The melanoma-specific an- sibility of leiomyoma, which occurs cells were encompassed by peri- tigen (HMB-45) and glial fibrillary as a spindle cell tumor in the ciliary odic acid–Schiff–positive matrix tis- acidic protein were negative. The tu- body and is immunoreactive for sue. Mitoses were not seen. Periph- mor cells displayed impressive im- smooth muscle actin. However, the erally, the necrotic material was munoreactivity for vimentin. The aforementioned epithelial features rimmed by foamy histiocytes. ciliary epithelium and a few foci of and the absence of ciliary body stro- Although the tumor appeared tumor cells also stained positively for mal involvement established that the on initial inspection to be a pri- S-100 protein. Neither the ciliary epi- tumor was an adenoma of the NPCE mary spindle cell neoplasm (Fig- thelium nor the tumor stained posi- with smooth muscle differentia- ure 4), it rested on the inner sur- tively for 3 keratin markers (903, tion and necrosis.

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Figure 5. Photomicrograph of area near base of tumor showing cords of Figure 6. Immunohistochemical preparation showing positive polarized epithelial cells resting on a basement membrane immunoreactivity for smooth muscle actin in peripheral viable cells. The (hematoxylin-eosin, original magnification ϫ250). ciliary muscle (upper left) serves as a positive control (original magnification ϫ25).

COMMENT of the NPCE generally is nonpig- cently, we have had remarkable suc- mented, yellow to light tan in cess in managing these tumors with Acquired neoplasms of the NPCE color, and often has an irregular, local resection.4 Because acquired tu- are relatively rare. Zimmerman1 sometimes multilobulated, free mors of the NPCE typically are cy- emphasized the remarkable poly- surface that directly impinges the tologically benign and the affected morphism of tumors of the NPCE vitreous cavity. Histopathologi- eye generally has good vision, it and categorized them into solid, cally, adenoma of the NPCE usu- seems reasonable to locally remove papillary, and pleomorphic types. ally is composed of nonpigmented the tumor rather than enucleate the In contrast to other reported cases, cuboidal or columnar cells that can affected eye. This is generally ac- the tumor reported here was com- assume solid, papillary, or pleo- complished by partial lamellar scler- posed largely of bland spindle morphic patterns.1 Most tumors of ouvectomy.3,4 cells, raising the possibilities of the NPCE show strands of polar- leiomyoma and melanoma. How- ized epithelial cells that rest on a Accepted for publication September 4, ever, the tumor rested on the inner prominent periodic acid–Schiff– 1998. surface of the ciliary body and positive basement membrane. This study was supported in part spared the uveal stroma, where the Some tumors contain prominent by the Paul Kayser Award of Merit in aforementioned spindle cell tu- pools of hyaluronidase-sensitive Retina Research, Houston, Tex (Dr J. mors would ordinarily reside. The acid mucopolysaccharide. Tumors A. Shields) and by the Eye Tumor Re- low-grade spindle cell component that show cytologic atypia exhibit search Foundation, Philadelphia, Pa. of the tumor displayed impressive growth and aggressive behavior Reprints: Jerry A. Shields, MD, positive immunoreactivity for the and local invasion. Despite the Oncology Service, Wills Eye Hospi- muscle markers, muscle-specific malignant classification, these tal, 900 Walnut St, Philadelphia, PA actin, and smooth muscle actin. tumors are generally not recog- 19107. Although decidedly unusual in an nized to undergo distant metasta- adenoma of the NPCE, smooth sis. In the case reported here, muscle differentiation should not necrosis was a prominent histo- REFERENCES be a totally unexpected finding pathologic feature. Our other cases because the iris dilator muscle is a of adenoma of the NPCE have not 3 1. Zimmerman LE. The remarkable polymorphism derivative of the neighboring neu- shown appreciable necrosis. The of tumors of the ciliary epithelium: the Norman 1 roepithelium. spindle cells in our case exhibited McAlister Gregg Lecture. Trans Aust Coll Oph- We have recently reviewed immunohistochemical characteris- thalmol. 1970;2:114-125. our clinicopathologic experience tics of smooth muscle differentia- 2. Grossniklaus HE, Lim JI. Adenoma of the non- with 9 cases of acquired neoplasms tion. Similar spindle cells were not pigmented ciliary epithelium. Retina. 1994;14: 452-456. of the NPCE, reviewed the litera- observed in other cases of adeno- 3. Shields JA, Eagle RC Jr, Shields CL, De Potter P. 3 ture, summarized the clinical and mas of the NPCE. Acquired neoplasms of the nonpigmented ciliary histopathologic features of these Historically, most cases of ac- epithelium (adenoma and adenocarcinoma). Oph- lesions, and pointed out the fea- quired neoplasms of the NPCE were thalmology. 1996;103:2007-2016. 4. Shields JA, Shields CL, Shah P, Sivalingam V. Par- tures that help differentiate them managed by enucleation because tial lamellar sclerouvectomy for ciliary body and from melanoma and other ciliary they were suspected clinically to be choroidal tumors. Ophthalmology. 1991;98: body lesions.3 Clinically, adenoma malignant melanoma. More re- 971-983.

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