Clinicopathological Features and Outcomes in Lymphoma of Extraocular Muscles.

PURPOSE: To describe the clinicopathological features and report the outcomes of treatment in lymphoma involving the extraocular muscles (EOM), the largest reported case-series. METHODS: A retrospective case series of patients with lymphoma involving the EOM from a single tertiary referral orbital center, between March 1992 and March 2018. Patients with other histopathologic diagnoses or who did not have an EOM biopsy were excluded. The main outcome measures were histologic evaluation and clinical follow-up including tumor response, recurrence, and survival. RESULTS: Twenty-five patients were included, 16 female; 9 male; median age 64.7 years (range 33.8-92.6 years). Unilateral involvement was present in 23 cases (92%). Lymphoma was found to be primary in 15 patients (60%), and any EOM could be affected by disease. Fourteen patients (56%) displayed impaired duction in the direction of the affected muscle. The commonest histologic type was extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in 16 cases (64%) followed by follicular lymphoma in 3 cases (12%), diffuse large B-cell lymphoma in 2 cases (8%), gamma delta T-cell lymphoma in 2 cases (8%), small lymphocytic lymphom and lymphoplasmacytic lymphoma in 1 case each (4%). All 4 patients with high-grade lymphomas (diffuse large B-cell or T cell lymphoma) were found to have systemic disease. Patients were treated with combinations of radiotherapy, chemotherapy, or immunotherapy, with low toxicity. Two patients developed treatment-related complications. With a median follow-up of 26 months (range 3-108 months), there was only 1 recurrence, which was successfully salvaged with Rituximab, and no deaths due to lymphoma. CONCLUSIONS: Lymphoma of the EOM is a rare tumor with good response to treatment, few treatment-related complications and low risk of recurrence or mortality.A retrospective review of 25 patients with lymphoma of the extraocular muscles demonstrates excellent prognosis with a high rate of local remission (96%), 2 treatment-related complications, one recurrence, and no disease-related deaths.

Review of extraocular muscle biopsies and utility of biopsy in extraocular muscle enlargement.

AIMS: To review the distribution of pathology in extraocular muscle (EOM) biopsies performed at a tertiary orbital centre, identify clinical and imaging features which are associated with benign or malignant diseases and indicate when biopsy is necessary for EOM enlargement. METHODS: Retrospective case series including 93 patients with EOM enlargement who underwent an EOM biopsy. Clinical, radiological and histopathological information was recorded from the medical records. Statistical analysis was used to compare variables between patients with malignant and benign biopsies. RESULTS: The median age of subjects was 61.1 years. Forty-eight cases (52%) were benign and 45 (48%) were malignant. Those with malignant pathology were significantly older (P<0.0001). Males were more likely affected by a benign disease and females by a malignancy (P=0.029). A history of malignancy (P<0.0001) and diplopia (P=0.029) were significant factors in predicting a malignancy. Pain (P=0.005) and eyelid erythema (P=0.001) were more likely in benign conditions. Idiopathic orbital inflammation was the most common benign diagnosis and lymphoma the most common malignancy. CONCLUSIONS: Biopsy is warranted in those with an atypical presentation of EOM enlargement or suspected of having a malignancy. Some features such as age, gender, pain, diplopia, history of malignancy and eyelid erythema may help indicate a particular diagnosis; however, clinical features and imaging findings are often not pathognomonic of each disease.

Visual outcomes and late complications in paediatric orbital rhabdomyosarcoma.

BACKGROUND: Orbital rhabdomyosarcoma is a rare but important malignancy for an ophthalmologist. We aimed to review the management and outcome, including late orbital complications and visual acuity over 25 years from a specialist paediatric ophthalmology department. DESIGN: This was a retrospective longitudinal case series. PARTICIPANTS: All patients presenting to our institution between December 1989 and December 2014 with a histopathological diagnosis of rhabdomyosarcoma originating from (primary) or invading into the orbit (paranasal) were included. METHODS: The oncology and ophthalmology databases were cross referenced to identify patients. MAIN OUTCOME MEASURES: Baseline demographics, chemotherapy, surgical and radiation dose, visual acuity, ocular and systemic complications, local and distant recurrence and mortality were recorded for each patient. Outcomes were reported with descriptive statistics. RESULTS: Eighteen patients were included. Median age was 4.3 years (range 4 months to 16 years) with average follow-up of 9 years. The 5-year disease-specific survival was 100% for the orbital group and 25% for the paranasal group; 29% of the orbital group maintained vision better than 6/12 in their treated eye, and the overall globe conservation rate was 71%. The most common ocular complications were cataract and keratopathy in both the orbital and paranasal groups. Other ocular complications included orbital hypoplasia or fat atrophy, eyelid malposition and lacrimal duct stenosis. CONCLUSIONS: Ophthalmic late effects are seen in a significant proportion of patients with orbital rhabdomyosarcoma. There is excellent survival in these patients, and continued efforts should be made to reduce the late effects of therapy.