Aaron Fay

American Joint Committee on Cancer Classification Predicts Outcome of Patients with Lacrimal Gland Adenoid Cystic Carcinoma

PURPOSE To investigate whether American Joint Committee on Cancer (AJCC) classification at initial diagnosis of lacrimal gland adenoid cystic carcinoma predicts outcome of treatment on local recurrence. DESIGN Retrospective chart review. PARTICIPANTS Consecutive patients with adenoid cystic carcinoma of the lacrimal gland treated at 8 institutions between January 1986 and December 2007. METHODS Clinical records, including pathology reports and imaging studies, were reviewed. MAIN OUTCOME MEASURES AJCC classification, histologic subtype, local recurrence rate, and survival. RESULTS AJCC classification at initial diagnosis was assessable for 53 patients and was as follows: T1N0M0, 7 patients; T2N0M0, 8 patients; T3aN0M0, 14 patients; T3aNxM0, 1 patient; T3aN0M1, 1 patient; T3bN0M0, 13 patients; T3bN0M1, 1 patient; T4aN0M0, 2 patients; T4bN0M0, 4 patients; T4bN0M1, 1 patient; and T4bNXM0, 1 patient. Thirty-eight (72%) of the 53 patients had >T3 tumors at presentation. Of the 38 patients with >T3 tumors, 20 were treated with orbital exenteration and postoperative adjuvant radiotherapy (RT), 6 were treated with orbital exenteration without RT, and 12 were treated with globe-preserving surgery (10 with RT and 2 without RT). Of the 15 patients with <T3 tumors, 6 were treated with globe-preserving surgery and RT, 2 were treated with globe-preserving surgery without RT, 6 were treated with orbital exenteration with bone removal and RT, and 1 was treated with orbital exenteration with bone removal without RT. Only 1 patient with a <T3 tumor, had local recurrence. Among patients with >T3 tumors, the risk of local recurrence (in the orbit or skull base) was higher in patients treated with conservative surgery as opposed to orbital exenteration and RT. Only 4 (20%) of the 20 patients treated with orbital exenteration and RT had local recurrence, compared with 3 (50%) of the 6 patients treated with orbital exenteration without RT and 8 (67%) of the 12 patients treated with globe-preserving surgery. Overall, 17 (45%) of the 38 patients with >T3 tumors and only 1 (7%) of the 15 patients with <T3 tumors died of disease during the study period. CONCLUSIONS In patients with lacrimal gland adenoid cystic carcinoma, AJCC >T3 disease at initial diagnosis correlates with worse outcomes than does AJCC <T3 disease. FINANCIAL DISCLOSURE(S) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Idiopathic fibroinflammatory disease of the face, eyelids, and periorbital membrane with immunoglobulin G4-positive plasma cells.

Progressive sclerosing orbital pseudotumors are a subset of usually primary and localized idiopathic fibroinflammatory disorders. We report on a 66-year-old man who developed this condition along the facial tissue planes with extension into the orbit and preferential involvement of the periorbital membrane. Fibrocollagenous tissue with scattered lymphoid aggregates without follicle formation dominated the process. There was a light dispersion of B and T lymphocytes and histiocytes in the stroma. Immunoglobulin G4 (IgG4)-positive plasma cells (>35 per high-power field) were identified mostly in the lymphoid clusters, as has been discovered in similar IgG4-related fibrosclerosing conditions of other nonorbital sites. No associated systemic disease emerged during a 20-year clinical course. Previously reported orbital cases of IgG4-positive disease have all involved the lacrimal gland, usually bilaterally, and more closely resembled hypercellular reactive lymphoid hyperplasias with moderate interlobular fibrosis, rather than representing an essentially sclerosing process from the beginning.

Pharmacologic Therapy for Periocular Infantile Hemangiomas: A Review of the Literature

Infantile hemangiomas are common vascular tumors of childhood with a propensity for the head and neck. In the periocular region, they can cause functional and cosmetic deformity. The proliferation and involution of hemangiomas are controlled by complex interactions of molecular, cellular, and hormonal regulators. There is no single uniformly safe and effective treatment option. Various modalities, including local and systemic pharmacologic agents, lasers, surgery and embolization, are employed to halt growth and to induce regression. This article will review the rapidly evolving field of pharmacologic treatment of infantile hemangiomas.

Propranolol for Isolated Orbital Infantile Hemangioma

1. Slaughter K, Sullivan T, Boulton J, O’Reagan P, Gole G. Early surgical intervention as definitive treatment for ocular adnexal capillary haemangioma. Clin Experiment Ophthalmol. 2003;31(5):418-423. 2. Schwartz SR, Blei F, Ceisler E, Steele M, Furlan L, Kodsi S. Risk factors for amblyopia in children with capillary hemangiomas of the eyelids and orbit. J AAPOS. 2006;10(3):262-268. 3. Peralta RJ, Glavas IP. Review of capillary hemangioma. EyeNet. February 2009: 35-37. 4. Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008; 358(24):2649-2651. 5. Weiss AH, Kelly JP. Reappraisal of astigmatism induced by periocular capillary hemangioma and treatment with intralesional corticosteroid injection. Ophthalmology. 2008;115(2):390-397, e1. 6. Siegfried EC, Keenan WJ, Al-Jureidini S. More on propranolol for hemangiomas of infancy. N Engl J Med. 2008;359(26):2846-2847.

Current theories on the pathogenesis of infantile hemangioma.

Infantile hemangiomas are the most common tumors of infancy and although the natural history and progression of these lesions are well described, their origin remains unclear. Considerable progress has been achieved in the past two decades towards understanding the etiology of these lesions. New investigations have produced sophisticated hypotheses on the origin of these tumors and their behavior. These include suggestions of placental origin, intrinsic defect or somatic endothelial mutation, and extrinsic factors creating a conducive milieu for growth. While no current hypothesis explains all the characteristics of infantile hemangiomas, continued research targeting pathophysiology will ultimately lead to new treatment options.

Changes in Gene Expression in Human Meibomian Gland Dysfunction

PURPOSE Meibomian gland dysfunction (MGD) may be the leading cause of dry eye syndrome throughout the world. However, the precise mechanism(s) underlying the pathogenesis of this disease is unclear. This study was conducted to identify meibomian gland genes that may promote the development and/or progression of human MGD. METHODS Lid tissues were obtained from male and female MGD patients and age-matched controls after eyelid surgeries (e.g., to correct entropion or ectropion). Meibomian glands were isolated and processed for RNA extraction and the analysis of gene expression. RESULTS The results show that MGD is associated with significant alterations in the expression of almost 400 genes in the human meibomian gland. The levels of 197 transcripts, including those encoding various small proline-rich proteins and S100 calcium-binding proteins, are significantly increased, whereas the expression of 194 genes, such as claudin 3 and cell adhesion molecule 1, is significantly decreased. These changes, which cannot be accounted for by sex differences, are accompanied by alterations in many gene ontologies (e.g., keratinization, cell cycle, and DNA repair). The findings also show that the human meibomian gland contains several highly expressed genes that are distinct from those in an adjacent tissue (i.e., conjunctival epithelium). CONCLUSIONS The results demonstrate that MGD is accompanied by multiple changes in gene expression in the meibomian gland. The nature of these alterations, including the upregulation of genes encoding small proline-rich proteins and S100 calcium-binding proteins, suggest that keratinization plays an important role in the pathogenesis of MGD.

Idiopathic sclerosing orbital inflammation: a review of demographics, clinical presentation, imaging, pathology, treatment, and outcome.

Purpose: To characterize clinical features, diagnostics studies, treatments, and outcomes of patients with histologically proven idiopathic sclerosing orbital inflammation (ISOI), to define optimal management for this recalcitrant disease, and to determine changes in characterization and management by comparing our results with the last significant literature review. Methods: A search of the U.S. National Library of Medicine: National Institutes of Healths electronic database for cases and case series in the English literature of biopsy-proven ISOI published between March 1994 and September 2010 was conducted. A cross-literature review was performed to tabulate demographics, clinical findings, studies, treatments, and outcomes, which were compared with the ISOI data published by Rootman et al. (1994). Results: Sixty-one cases, 71 eyes from 17 published reports, met inclusion criteria. No ethnic, sex, or comorbidity predilection was established. Patients typically presented in the fourth decade with proptosis (73%), pain (49%), and normal vision (44%). Orbital imaging and histopathology were sparsely reported. Most common treatments involved systemic corticosteroids either alone (34%) or combined with other modalities (51%). Conclusions: Characteristics of the disease remain unchanged, and best management was not determined due to inconsistent reporting methods across the literature. Collaboration with established groups (i.e., European Group On Graves Orbitopathy (EUGOGO), International Thyroid Eye Disease Society (ITEDS)) or the formation of a new group of physicians and scientists to help develop a systematic approach for future reporting and evaluation was proposed.

Orbital complications of hydrogel scleral buckles.

Purpose: Hydrogel is a biocompatible osmotic expansile material used in contact lenses, scleral buckles, and orbital expanders. Three unique complications from hydrogel scleral buckle use are presented: orbital cellulitis mimicry, fornical shortening with orbital prosthetic intolerance, and orbital pseudotumor. Methods: A retrospective chart review was conducted on three subjects with unknown hydrogel scleral buckle complications at a tertiary care oculoplastics practice. Demographics, imaging studies, and pathologic specimens were reviewed. Results: All three patients had a history of encircling hydrogel scleral buckle placed for retinal detachment. The mean time from implantation to presentation was 10.7 years (range, 7 to 15 years). One patient had an onset of presumed orbital cellulitis caused by an enlarged buckle and no abscess. The second patient had inability to wear an evisceration prosthesis as the result of a shortened fornix from an enlarged hydrogel scleral buckle implant. The third patient presented with an orbital mass caused by the enlarged implant. In all cases, surgical removal of the expanded buckle relieved the patient’s symptoms. Conclusions: Although the hydrogel scleral band is designed to expand in a controlled fashion, overexpansion can occur. Expansion can cause buckle extrusion, prosthetic intolerance, a pseudo-orbital cellulitis, or orbital pseudotumor and may be due to a chemical change in the hydrogel polymer. Patients with hydrogel scleral buckles should be followed long-term for possible complications. Patients with newer uses of hydrogel, such as orbital expanders, should also be observed for long-term complications.

Magnetic resonance imaging of optic perineuritis

Optic perineuritis, an uncommon variant of orbital pseudotumor, may be clinically indistinguishable from retrobulbar optic neuritis. Because treatment and prognosis for these two entities are different, early diagnosis is important. We report a case of a 47 year-old woman with clinical findings suggestive of retrobulbar optic neuritis, but whose magnetic resonance images suggested optic perineuritis. A dramatic clinical response to oral corticosteroids was observed. Optic perineuritis should be considered in cases of presumed retrobulbar optic neuritis. MRI may differentiate these two entities in the acute stage, and should be considered before treatment is decided.

Endoscopic resection of orbital hemangiomas

Cavernous hemangiomas are the most common orbital tumors in adults. Traditional orbitotomy approaches to resect these intraconal lesions are associated with patient morbidity due to difficulties with visualization and access in the region of the orbital apex.