1. Intraocular adenocarcinoma
Abdullah A. Al Qahtani, MD , Hind Alkatan MD
King Khalid eye specialist hospital & King Saud University, Riyadh, KSA
Purpose
Table1: Differentiating features of intraocular adenocarcinomas based on their origin
The initial histopathologic diagnosis was a high grade carcinoma. The cells were positive with CytoK 7, 8-18 and AE1/AE3 as well as CAM 5.2 while negative with Vimentin, HMB45 and Melan A. The diagnosis of CBE Adenocarcinoma possibly arising from a preexisting adenoma was made.
Histopathologic Report of two cases with intraocular adenocarcinoma and unique different presumed origin.
Background
Discussion:
Acquired malignant tumors that involve ciliary body epithelium (CBE) and retinal pigment epithelium (RPE) such as adenocarcinoma are extremely rare. Most of the histopathological changes involving these are benign like adenoma, congenital hypertrophy of retinal pigment epithelium (CHRPE) and reactive hyperplasia. Although it is known that CHRBE is known to enlarge slowly over long periods, malignant changes within CBE and RBE are extremely rare and few reports have been published where intraocular adenocarcinoma arises secondarily to ocular phthisis, CHRBE, chorioretinal scars or metastasis. Here in we are reporting two cases of intraocular adenocarcinoma one arising from the CBE and the other from RPE .Their histopathological features are described in details with comparison to previously reported cases.
Acquired malignant tumors involving CBE and RPE are extremely rare. The pathogenesis of intraocular adenocarcinoma is unclear. They may develop in association with inflammation, chorioretinal scars and in blind eyes following trauma, surgery or other conditions as in our first case.
Malignant changes are extremely rare with few reports of intraocular adenocarcinoma arising secondarily to phthisis, CHRBE, or metastasis. Our second case was clearly originating from the CBE possibly from a pe-existing adenoma which has not been reported.
Zimmerman sub-classified adenocarcinoma into solid, papillary and pleomorphic but was difficult to apply since most of the tumors were composed of all of these histological types.
RBE tumors may express both epithelial and melanocytic markers, therefore IHC staining results should be interpreted with caution since the differential diagnosis of these tumors will include melanoma, medulloepitheloma, and metastasis.
The origin of these may be difficult to determine, however based on our literature review, the differentiating features including IHC staining characteristics are summarized in Table 1.
Intraocular Adenocarcinoma may constitute diagnostic challenge to the ophthalmic pathologist. Good knowledge of the characteristic features and careful examination of routine evisceration tissue are a must to avoid overlooking such tumors or misdiagnosis specially when an underlying neoplasm is unsuspected.
features and careful examination of routine evisceration tissue are a must to avoid overlooking such tumors or misdiagnosis specially when an underlying neoplasm is unsuspected.  Try to find an article on malignant transformation of adenoma
 Try to find an article on malignant transformation of adenoma
Methods
Retrospective chart review of 2 patients with intraocular carcinoma documented by tissue diagnosis and fully evaluated by immunohistochemical (IHC) staining to further confirm the diagnosis. The differentiation of the tumor origin is based on careful observations and literature review of previously reported cases.
Figure 1: A: The clinical appearance of the left blind eye in case 1. B: The tubular pattern of the adenocarcinoma tumor cells (X100 PAS). C: The heterotopic bone formation in the phthisical globe (X200 H&E). D: Tumor cells staining with Cytok AE1/AE3 (X400)
Results
Conclusion:
Case 1:
A thirty-one year old male presented with history of blind painful left eye following history of trauma by a stick at the age of 5 years.
Histopathologic examination showed pleomorphic tumor cells arranged in nests, separated by fine septae with focal tubular and acinar growth patterns and invading the heterotopic bone. Some cells showed vacuolated cytoplasm. The intracytoplasmic vacuoles showed focal intense staining with PAS, Alcian blue and Mucicarmine.  IHC staining showed positivity with Cytokeratin AE1/AE2, NSE, Vimentin, CK8-18, CK19 and CK20. The diagnosis of intraocular adenocarcinoma was made presumably arising from the RPE. His systemic work up to rule out metastatic adenocarcinoma was negative.
Case 2:
A sixty-nine year old male referred as a case of orbital invasion from a squamous cell carcinoma presumably arising from the limbus. Exenteration specimen histopathologic examination showed a focus of benign lace and cribriform-like proliferation of an incidental CBE adenoma and an adjacent proliferation of high grade pleomorphic cells with giant tumor cells and frequent mitotic figures. The cells showed adenoid differentiation, vaculated appearance and intervening globules of PAS-positive material. Areas of massive necrosis, intra-ocular hemorrhage and calcification were also noted. The tumor showed extra-ocular extension as well as orbital vascular invasion.
Financial disclosures: None
Figure 2: Case 2
A: The tumor presumably arising from a pre-existing CBE adenoma (X100 H&E).
B: Tumor cells with secretory globules(X400 PAS). C: Perivascular invasion (X200 H&E).
D: IHC positive staining of tumor cells (X 200 CytoK 7)
References
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