|LETTER TO THE EDITOR
|Year : 2013 | Volume
| Issue : 2 | Page : 132-134
Papillary adenocarcinoma of ciliary body
Anup P Bhandari, Sunil Y Swami, Rasika U Gadkari
Department of Pathology, S. R. T. R. Medical College, Ambajogai, Maharashtra, India
|Date of Web Publication||19-Aug-2013|
Sunil Y Swami
Bhagwanbaba Chowk, Gitta - Road, Shepwadi, Taluka, Ambajogai, Beed - 431 517, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bhandari AP, Swami SY, Gadkari RU. Papillary adenocarcinoma of ciliary body. Oman J Ophthalmol 2013;6:132-4
Tumors arising from the pigmented and nonpigmented epithelia of the iris and ciliary body are extremely rare. 
In a review of 20 histopathologically proved cases of adenocarcinoma of the ciliary body or iris pigment epithelium, Dryja and his co-authors reported the mean age at diagnosis to be 45 years. 
We describe herein an adenocarcinoma of the ciliary body occurring in a 70-year-old female as an accidental finding. This tumor is clinically indistinguishable from a melanoma or medulloepithelioma.
A 70-year-old female presented with pain, tearing, diminution of vision, and photophobia of the right eye for a period of 2 months.
On right ocular examination, eyelids were edematous, conjunctiva was congested, and pupils were semidilated, fixed, showing yellowish reflex. Intraocular pressure was increased. Visual acuity showed no perception of light. On fundoscopy, media was hazy. Left eye examination was normal. General and systemic examination was unremarkable. Diagnosis of right eye endophthalmitis was made. Treatment advised was enucleation of right eye. During operation, tiny mass was found attached behind the iris in posterior chamber.
Grossly, tumor mass of size 0.5 × 0.5 cm attached posterior to iris with some cystic areas and an area of brownish black discoloration was received.
Microscopy showed tumor tissue near the iris [Figure 1], composed of cells arranged in papillary and tubular fashion [Figure 2]. Individual cells were round to oval cells with hyperchromatic nuclei, high nuclear cytoplasmic ratio, and scanty cytoplasm. Infiltration of sclera with tumor cells was present. Surrounding stroma showed desmoplastic reaction along with melanin pigment and sparse chronic inflammatory infiltrate deposition [Figure 3].
|Figure 2: Tumor cells showing tubular and papillary pattern (H and E, ×10)|
Click here to view
|Figure 3: Higher magnification of round to oval tumor cells with vesicular nuclei, prominent nucleoli, scanty cytoplasm, and high N:C ratio (H and E, ×40)|
Click here to view
Histopathological differential diagnosis included papillary adenocarcinoma of ciliary body, medulloepithelioma (adult variant), and malignant melanoma. Immunohistochemistry was done to confirm the diagnosis.
Immunohistochemistry showed immunopositivity for cytokeratin [Figure 4] and epithelial membrane antigen (EMA) [Figure 5].
HMB-45 antibody (human melanoma black) [Figure 6] and neuron-specific enolase (NSE) [Figure 7] were negative, which confirms nonpigmented ciliary epithelium adenocarcinoma. Retrospective search for any primary tumor elsewhere in the body was negative.
Adenocarcinoma is a term that describes a tumor derived from the pigmented or nonpigmented epithelium of the ciliary body and/or iris that showed local invasion, cellular dedifferentiation, and the potential for distant metastasis.  Most patients with these tumors are between the ages of 30 and 70 years.  Ciliary body melanoma and medulloepithelioma (adult variant) were considered in the differential diagnosis of this pigmented mass.
A possible diagnosis of tumor metastatic to the eye like breast, lung, and gastrointestinal tract carcinomas were ruled out after careful history, physical examination, and appropriate laboratory studies. 
Adenocarcinoma of ciliary body is rare tumor with incidence of less than 3% of all ciliary body tumors. Clinically, it presents as incidental finding or causes subluxation of the lens, segmental cataract, or secondary glaucoma. Mostly they are localized in globe, but can exhibit extraocular extension. Generally it does not have tendency to metastasize.
This tumor needs to be reported as it has better prognosis than more common medulloepithelioma.
| Acknowledgment|| |
We are thankful to Professor and Head, Department of Pathology, AFMC Pune for IHC and expert opinion.
| References|| |
|1.||John JP, Kenichi A, Tatsuo H, Kasuo T, Kasuaki H, Daniel MA. Adenocarcinoma of the Ciliary Body Pigment Epithelium in a Child. Arch Ophthalmol 1984;102:100-3. |
|2.||Myron Y, Joseph WS. Ocular melanocytic tumors. In: Ocular pathology. 6 th ed. St Louis, MO: Mosby/Elsevier; 2009. p. 693. |
|3.||Streeten BW, McGraw JL. Tumor of the ciliary pigment epithelium. Am J Ophthalmol 1972;74:420-9. |
|4.||Wolter JR, James BR. Adult type of medulloepithelioma of the ciliary body. Am J Ophthalmol 1958;46:16-26. |
|5.||Wolfgang EL, Jerry AS, Carol LS, George LS. Mucinous adenocarcinoma metastatic to the iris, ciliary body and choroid. Br J Ophthalmol 1990;74:373-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]