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Year : 2020  |  Volume : 63  |  Issue : 1  |  Page : 134-135
Primary adenocarcinoma of ciliary body: An exceptional rare tumor

1 Department of Ophthalmic Pathology Laboratory, L V Prasad Eye Institute, Hyderabad, Telangana, India
2 Operation Eyesight Universal Institute for Eye Cancer, L V Prasad Eye Institute, Hyderabad, Telangana, India

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Date of Web Publication31-Jan-2020

How to cite this article:
Mishra DK, Kaliki S. Primary adenocarcinoma of ciliary body: An exceptional rare tumor. Indian J Pathol Microbiol 2020;63:134-5

How to cite this URL:
Mishra DK, Kaliki S. Primary adenocarcinoma of ciliary body: An exceptional rare tumor. Indian J Pathol Microbiol [serial online] 2020 [cited 2022 Jan 21];63:134-5. Available from: https://www.ijpmonline.org/text.asp?2020/63/1/134/277378

Tumors arising from the pigment and non-pigment epithelium of ciliary body and iris are very rare.[1]

Here we have described the primary adenocarcinoma of non-pigmented ciliary body in an 85-year-old-female who was diagnosed as malignant melanoma clinically.

An 85-year-old female presented with progressive painful vision, watering of right eye associated with vomiting for a period of one week consulted with local ophthalmologist who diagnosed her right-angle glaucoma and ciliary body melanoma. Patient was referred for better management to ophthalmic oncology department of LV Prasad eye institute, Hyderabad.

On right eye slit lamp examination, eye lids were edematous, conjunctiva was congested with feeder vessels, sclera was normal, cornea was clear and showed arcus senilis, peripheral anterior chamber depth was shallow and pupils were hazy. Iris/ciliary body showed a raised amelanotic lesion extending from 2 to 9 o'clock position and occluding the angle. Ultrasound bio-microscopy showed an acoustic dense ciliary mass inferiorly [Figure 1]a. Her intraocular pressure was raised and it was 39 mm of mercury (Hg) at the time of examination. Left eye examination was relatively normal in comparison to right eye. Systemic workup and general examination were within normal limits. Clinical diagnosis of right eye angle closure glaucoma with iris/ciliary body melanoma was made and enucleation was performed.
Figure 1: (a) A blood-stained amelanotic lesion involving the iris inferiorly. Ultrasound bio-microscopy of right eye showed an acoustically dense ciliary body mass inferiorly. (b) Histopathology of the lesion showed the tumor cells in glandular arrangement with hyper-chromatic nuclei and scanty cytoplasm, Hematoxylin and Eosin stain (×100). (c) Immunostaining positive for pan-cytokeratin (×100) and (d) Immunostaining negative for Melan-A (×100)

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Enucleated eye was sent to ophthalmic pathology laboratory for the histopathological examination. A gross examination of the sectioned eye showed a pale grey white mass of size (10 × 5 × 3) mm in iris and ciliary body region with foci of haemorrhage, which was obliterating the anterior chamber.

Microscopy showed tumor in iris and ciliary body and composed of cells arranged in tubules, sheets, and glandular pattern [Figure 1]b. These tumor cells have round to oval hyperchromatic pleomorphic nuclei, vesicular chromatin, and high nucleo-cytoplasmic ratio. Cytoplasm of these tumor cells were scanty, and they were devoid of melanin pigments. Surrounding stroma showed desmoplastic reaction and few chronic inflammatory cells.

On this histomorphology, differential diagnosis of amelanotic melanoma, metastatic deposits, and primary adenocarcinoma was offered and further immune stain was done for the confirmation of diagnosis.

Immunohistochemistry for cytokeratin showed immunopositivity [Figure 1]c; HMB-45, Melan-A [Figure 1]d, neuron specific enolase (NSE), CK-7, and CK20 were immunonegative, which confirm the non-pigmented primary adenocarcinoma of ciliary body epithelium. Systemic and general examination was also non- contributory of any primary tumor elsewhere.

Primary tumors of the non-pigmented ciliary epithelium are rare.

Zimmerman[2] divided these tumors into two classes, congenital and acquired. Congenital tumors arise in the embryonic or early postnatal period and includes medulloepithelioma and glioneuromas.

The acquired tumors are seen in adulthood and include hyperplasia of the non-pigmented ciliary body epithelium, adenoma and adenocarcinoma of the non-pigmented ciliary epithelium.[3],[4]

Our patient developed primary adenocarcinoma of ciliary body at the age of 85 years; Bhandari et al. reported adenocarcinoma of ciliary body in a 70-year-old-female as an accidental finding.[5]

Zhang et al. from china reported Adenocarcinoma of the non-pigmented ciliary epithelium manifested as an anterior chamber mass in a 17-year-old male.[6]

Amelanotic melanoma is also very close differential diagnosis, but in our case, HMB-45 and Melan-A were negative that also excludes the diagnosis of melanoma. Medulloepithelioma is the childhood tumor with median age of diagnosis is five years[7], and this tumor is positive for the NSE; in our case, the age of presentation was in old age and also tumor was negative for the NSE that excludes the diagnosis of medulloepithelioma. The prognosis of this tumor is relatively good in comparison to melanoma of ciliary body. Tumor relapse or metastasis after tumor resection or enucleation of the eye has not been reported.[8] In our case, after six months of follow-up, patient was not presented with recurrence or metastasis elsewhere.

To conclude, non-pigmented primary adenocarcinoma of ciliary body epithelium is rare tumor and its diagnosis clinically and histopathologically is challenging because it mimics with melanoma, medulloepithelioma, and metastatic tumors from solid organs, hence immune stain and systemic workup is essential to reach the final diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

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.  Back to cited text no. 1
Zimmerman LE. The remarkable polymorphism of tumors of the ciliary epithelium. Trans Aust Coll Ophthalmol 1970;2:114-25.  Back to cited text no. 2
Shields JA, Shields CL. Tumors of the non-pigmented ciliary epithelium. In: Shields JA, Shields CL, editors. Intraocular Tumors. A Text and Atlas. Philadelphia: WB Saunders; 1992. p. 461-87.  Back to cited text no. 3
Green Retina WR. Acquired neuroepithelial tumors of the ciliary body. In: Spencer WH, Font RL, Homes EL, editors. Ophthalmic Pathology. An Atlas and Textbook, Vol 2. Philadelphia: WB Saunders; 1985. p. 1254-62.  Back to cited text no. 4
Bhandari AP, Swami SY. Papillary adenocarcinoma of ciliary body. Oman J Ophthalmol 2013;6:132-4.  Back to cited text no. 5
[PUBMED]  [Full text]  
Bai HX, Wei WB, Li B, Xu XL, Yang WL, Gao F, et al. Adenocarcinoma of the nonpigmented ciliary epithelium manifested as an anterior chamber mass. Int J Ophthalmol 2017;10:485-7.  Back to cited text no. 6
Wolter JR, James BR. Adult type of medulloepithelioma of the ciliary body. Am J Ophthalmol 1958;46:16-26.  Back to cited text no. 7
Rodrigues M, Hidayat A, Karesh J. Pleomorphic adenocarcinoma of ciliary epithelium simulating an epibulbartumor. Am J Ophthalmol 1988;106:595-600.  Back to cited text no. 8

Correspondence Address:
Dilip K Mishra
Department of Ophthalmic Pathology Laboratory, L V Prasad Eye Institute, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_37_19

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