Accessory lacrimal gland tumours of the eye region.

PURPOSE: Tumours of the accessory lacrimal glands (ALGs) are rare and need to be differentiated from other tumours involving the eyelids and caruncle. We report on a consecutive series of patients with ALG tumours and describe the clinical characteristics. METHODS: A retrospective case series including all patients who were operated for ALG tumours at two different centres. RESULTS: The study included 27 patients diagnosed with ALG tumours. Mean age at presentation was 55.9 years (range, 24-81 years; median, 56 years). Overall, a slight male preponderance was observed (M:F = 1.4:1). Histologically, pleomorphic adenoma was the commonest diagnosis (24 of 27; 88.9%), followed by adenoid cystic carcinoma (2 of 27; 7.4%) and oncocytoma (one of 27; 3.7%). A complete surgical excision was curative in all patients. CONCLUSION: Accessory lacrimal gland (ALG) tumours are rare and may therefore pose diagnostic difficulties for clinicians. Histopathological examination of these tumours is the cornerstone of the diagnostic evaluation. Complete surgical excision is the treatment of choice in these tumours.

Isolated Orbital Aspergillosis in Immunocompetent Patients: A Multicenter Study.

OBJECTIVE: To report clinicopathologic features, radiologic findings, and treatment outcomes of isolated, orbital aspergillosis. DESIGN: Multicenter, retrospective case series. METHODS: setting: Multicenter. PARTICIPANTS: There were 8 lesions in 8 eyes of 8 patients with isolated, orbital aspergillosis. PROCEDURE: Review of medical records and histopathology slides. MAIN OUTCOME MEASURES: Disease control. RESULTS: Of 34 patients with orbital aspergillosis, 8 (23.5%) had isolated orbital involvement at presentation. The mean age at presentation was 34.5 years (median, 43 years: range, 0.5-72 years). Gradually progressive proptosis and eyelid swelling were the most common presenting features (each 4/8). Proptosis ranged from 4 mm to 9 mm (median, 5.5 mm; mean, 5.75 mm). Restriction of ocular motility was seen in all 8 patients. Other examination findings included palpable mass (2/8), conjunctival chemosis (2/8), hyperglobus (1/8), hypoglobus (1/8), and resistance to retropulsion (1/8). Microbial culture results were available in 1 patient and showed Aspergillus fumigatus. Two patients were treated with complete surgical excision alone while 6 were treated with antifungal medications. Complete resolution of proptosis and restoration of ocular motility were seen in all patients following treatment. Visual disturbances present in 1 were corrected following treatment. Recurrence was observed in 1 patient. CONCLUSION: Isolated orbital aspergillosis, though rare, should be considered in the differential diagnosis of a patient presenting with a gradually progressive orbital mass, especially in Asian individuals. Early recognition will help reduce the morbidity and mortality associated with this disease.

Clinicopathologic features of orbital immunoglobulin G4-related disease (IgG4-RD): a case series and literature review.

BACKGROUND: Involvement of orbital structures by immunoglobulin G4-related disease (IgG4-RD) is not uncommon. We conducted this study to evaluate the clinicopathologic features of orbital IgG4-RD. MATERIAL/METHODS: This was a retrospective, clinicopathologic study. Clinical records, light microscopic features, results of immunostaining with IgG & IgG4 and laboratory findings were reviewed in 16 patients diagnosed with orbital IgG4-RD. RESULTS: Eleven patients had a bilateral disease, and the lacrimal gland was involved in 14. Dense sclerosis, plasma cell aggregates and dense lymphoplasmacytic infiltrate were seen in all patients. Serum IgG4 titre was elevated in 12 patients. Nine patients responded completely to glucocorticoid treatment. Five patients had a relapse on discontinuation of treatment. CONCLUSION: Orbital IgG4-RD is a distinct clinicopathologic entity requiring increased awareness and needs to be differentiated from other orbital lymphoproliferative lesions.

Periocular sebaceous gland carcinoma: do androgen receptor (NR3C4) and nuclear survivin (BIRC5) have a prognostic significance?

PURPOSE: The study aimed at evaluating the expression of androgen receptor (AR) and nuclear survivin (NS) in periocular sebaceous gland carcinoma (SGC) and to determine whether this expression is associated with histopathological features, markers of apoptosis and proliferation and with clinical outcomes. METHODS: This was a retrospective, comparative case series which included 56 patients with a biopsy-proven periocular SGC. Immunohistochemical staining for AR, survivin, p53 and Ki-67 was analysed in all cases. RESULTS: All patients expressed AR, p53 and Ki-67 in the nucleus of tumour cells. Twenty-four patients (42.8%) had a high AR score, and 32 patients (57.2%) had a low AR score. Twenty-four (42.8%) patients expressed survivin in the nucleus of tumour cells. Nine (37.5%) had a high NS score, and 15 (62.5%) had a low NS score. Patients with a high AR score had a greater recurrence (p < 0.005), higher expression of Ki-67 (p < 0.0001) and a lower p53 expression (p < 0.005). Nuclear expression of survivin correlated with a high Ki-67 labelling index (0.0001) and low p53 expression (<0.005). Neither nuclear expression of survivin nor the NS score correlated with any clinicopathological features. CONCLUSION: Expression of AR significantly impacts prognosis and is thus promising prognostic marker in periocular SGC.

Orbital extra-medullary granulocytic sarcoma: clinicopathologic correlation with immunohistochemical features.

Orbital granulocytic sarcoma (chloroma), a rare tumor of immature myeloid cells, has nonspecific clinical and radiological features that make it a diagnostic challenge. Light microscopy and confirmation by immunostaining aid in the diagnosis. Knowledge of this entity is important because early diagnosis and prompt treatment are associated with better prognosis. We report a series of 31 biopsy-proven orbital granulocytic sarcomas with their immunohistochemical features and review the literature.

Orbital immunoglobulin-G4-related disease: case series and literature review.

Immunoglobulin-G4-related disease (IgG4-RD) is a distinct group of diseases characterized by elevated serum IgG4 titres and infiltration of affected organs by IgG4-positive plasma cells. IgG4-RD can involve any ocular adnexal tissue. They have a distinct prognosis and pattern of tissue involvement and hence need to be differentiated from orbital lesions with similar clinicopathological features. We report three cases of IgG4-RD and review the literature extensively discussing various aspects of this novel entity.

Periocular sebaceous gland carcinoma: A comprehensive review.

Sebaceous gland carcinoma (SGC) is a rare tumour occurring at periocular and extra-ocular sites. SGC can be a challenging diagnosis for both clinicians and pathologists. High recurrence rates and a tendency for intra-epithelial spread, locoregional and distant metastases make it important for SGC to be suspected and be included in the differential diagnosis of an eyelid lesion. Early diagnosis, that may sometimes need ancillary testing, and prompt management using a multimodal approach can help reduce morbidity and mortality in patients with SGC.

Compressive effects of intravascular papillary endothelial hyperplasia.

A 45-year-old woman presented with an 8-month history of a firm mass over the right superonasal orbital rim, followed by an episode of hemorrhage and anesthesia 5 months later. Clinical history and CT were suggestive of a venous malformation with phleboliths. Excision biopsy was performed. Histopathology revealed intravascular papillary endothelial hyperplasia with fibrotic-calcific changes. Hypoesthesia in the supra-orbital region persisted postoperatively. Although a benign lesion, intravascular papillary endothelial hyperplasia should be considered a differential diagnosis of a vascular lesion with compressive effects.

Choroidal vitiligo masquerading as large choroidal nevus: a report of four cases.

PURPOSE: To describe 4 patients with choroidal vitiligo masquerading as large choroidal nevus. DESIGN: Retrospective chart review. PARTICIPANTS: Observational case series of 4 patients. METHODS: Retrospective chart review. MAIN OUTCOME MEASURES: Clinical features. RESULTS: Four patients referred with the diagnosis of large choroidal nevus were found to have unilateral (n = 1) or bilateral (n = 3) extensive patchy choroidal depigmentation classified as choroidal vitiligo. There was no evidence of choroidal nevus, and the pigmented "lesion" proved to be normal choroidal pigment surrounded by a region of pigment absence (vitiligo). There was no evidence of ocular inflammation or related retinal or retinal pigment epithelial changes. The choroidal vitiligo was clinically flat and measured 12 to 24 mm diameter, involving the post-equatorial fundus in all cases. There were no related anterior segment abnormalities. Cutaneous vitiligo was present in all cases. There was no documented progression of the choroidal or cutaneous vitiligo over a maximum 2-year follow-up. CONCLUSIONS: Choroidal vitiligo is an idiopathic benign process that can involve large segments of the posterior choroid, leaving only patches of residual choroidal pigment, simulating, in reverse, a large choroidal nevus. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Effectiveness of the gold weight trial procedure in predicting the ideal weight for lid loading in facial palsy: a prospective study.

PURPOSE: To evaluate the effectiveness of the gold weight trial procedure in predicting postoperative eyelid closure after gold weight implantation surgery for facial palsy. DESIGN: Prospective, interventional case series. METHODS: Patients with facial palsy undergoing gold weight implantation were enrolled prospectively to undergo the standard gold weight trial procedure. The criteria for ideal gold weight were defined before surgery as a minimum 50% reduction in lagophthalmos without induction of more than 2 mm ptosis during the trial procedure. The corrected weight (ideal gold weight + 0.2 g) was implanted in the pretarsal space. The main outcome measure was reduction in lagophthalmos according to predefined criteria at six weeks after surgery. RESULTS: Thirty eyes of 29 patients underwent gold weight implantation. The mean age at surgery was 41.6 years, and the median predicted ideal gold weight was 1.2 g. The mean preoperative lagophthalmos of 7 mm reduced to 2.3 mm at six weeks after surgery. As defined by the success criteria, undercorrection was noted in nine eyes (30%) at six weeks after surgery. A preoperative lagophthalmos of 8 mm or more was noted in seven (77.8%) of nine eyes in the undercorrection group, compared with six (28.6%) of 21 eyes in the success group. Six of the nine failures required implant exchange. CONCLUSIONS: The ideal gold weight as estimated by the trial procedure (with 0.2 g correction factor) led to undercorrection in 30% cases. Undercorrection was common (78%) in patients with preoperative lagophthalmos of more than 8 mm.