Differential Diagnoses of Diseases Involving the Extrinsic Ocular Musculature ­ A Pictorial Essay

Introduction There are some inflammatory, infectious, and neoplastic diseases affecting the extrinsic orbital musculature (EOM) that present with pain, decreased visual acuity, and proptosis. Imaging is fundamental to the differential diagnoses of these diseases with similar clinical presentations. The present case series report has as main objective to illustrate and discuss the main pathologies that affect the orbit. Material and Methods The present series of cases discusses the main pathologies that can affect the extraocular musculature that can be characterized by computed tomography (CT) or magnetic resonance imaging (MRI) using cases from our institution. Results and Discussion The present study compiled several cases of ophthalmopathy from our institution to illustrate and address some of these pathologies, such as orbital lymphoma, Grave disease, metastases, periorbital cellulitis, and idiopathic orbital inflammatory syndrome. The diseases are discussed according to the presentation of clinical cases with emphasis on the main imaging findings of each pathology. Conclusion Computed tomography and MRI can help in the diagnosis and follow-up of the diseases that affect the EOM. We must be conversant with the main characteristics of the pathologies presented in the present case series report, since such findings together with clinical data can confirm the diagnosis of these diseases or at least help to narrow the differential diagnoses.

The value of DWI combined with DCE in the differential diagnosis of orbital lymphoma and inflammatory pseudotumor / 实用放射学杂志

Objective To evaluate the value of MR DWI combined with dynamic contras-t enhanced (DCE)scanning in the differential diagnosis of orbital lymphoma and inflammatory pseudotumor.Methods The clinical and imaging data of 1 5 cases with orbital lymphoma and 10 cases with inflammatory pseudotumor confirmed by surgery and pathology were analyzed retrospectively.25 patients were performed with conventional MR scanning,DWI scanning and DCE scanning.ADW4.7 software was used for DWI images and DCE images pos-t processing for analyzing the two groups of lesions in the TIC subtyping.Independent sample t test statistical analysis was used for comparing two groups of ADC values and Tmax values.The ROC curve was used for determining the statistically significant differences in measurement data of the threshold.With pathologic and immunohistochemical results as the gold standard,the accuracy,sensitivity and specificity of DWI combined with DCE in the diagnosis of orbital lymphoma and inflammatory pseudotumor were further detected.Results Among the 1 5 patients with orbital lymphoma,8 cases (8/15)occurred in the lacrimal gland area,5 cases (5/1 5)occurred in the outer cone space and 2 cases (2/15)occurred in the eyelid.Among the 10 patients with inflammatory pseudotumor,6 cases (6/10)occurred in the lacrimal gland and 4 cases (4/10)in the bulb.Among the 25 patients,9 of them had the TIC curve of outflow,6 of them had the TIC curve of platform and 10 of them had the TIC curve of inflow.The ADC value of orbital lymphoma was (0.573 ±0.1 1 6)×10－3 mm2/s,and the Tmax value was (248.2±1 59.4)ms.The ADC value of inflammatory pseudotumor was (1.186±0.244)×10－3 mm2/s,and the Tmax value was (5 5 7.6 ±98.7)ms.With the ADC＝0.885×10－3 mm2/s for the threshold,the AUC was 0.9 3 6 ,with diagnostic accuracy of 9 1.3%, the sensitivity of 90.1% and specificity of 80%.With the Tmax＝481.2 ms as the threshold,the AUC was 0.927,with the diagnosis accuracy rate of 88%,the sensitivity of 80%,and the specificity of 86.7%. Finally,DWI combined with DCE could reach the AUC 0.945, diagnostic accuracy of 89.5%,sensitivity of 93.3%,and specificity of 82.6%.Conclusion Conventional MRI scan combining DWI with DCE could show the histological components of the lesion of orbital tumor,which is of certain significance for the qualitative diagnosis of orbital lymphoma and inflammatory pseudotumor.

Effect of radiotherapy for primary orbital lymphoma: analyses of 28 cases / 白血病·淋巴瘤

Objective@#To analyze the effect and adverse reactions of radiotherapy in patients with primary orbital lymphoma.@*Methods@#A total of 28 patients with primary orbital lymphoma from Tianjin Union Medical Center, the First Central Hospital of Tianjin and Tianjin Dagang Oil Company Hospital between March 2006 and August 2012 were retrospectively analyzed. All the patients received orbital tumor dissection or biopsy, then received radiation therapy. Three patients received chemotherapy with CHOP protocol before radiotherapy. Radiotherapy was delivered routinely with 3D-conformal radiation therapy (CRT) technique with daily 2-2.5 Gy for 5 times per week and 3-5 fields. Total dosage was 20-50 Gy. There were 18 cases of 30 Gy, 1 case of 22 Gy, 1 case of 25 Gy, 1 case of 20 Gy, 1 case of 28.8 Gy, 3 cases of 40 Gy, 1 case of 42 Gy, 1 case of 46 Gy and 1 case of 50 Gy.@*Results@#All the patients had complete remission (CR) after radiotherapy during follow-up. One patient recurred after radiotherapy of 4 months and received CR with radiotherapy again. One patient died of lung infiltration. Acute complications during radiotherapy were conjunctivitis (28 cases, 100.0%) and keratitis (1 case, 3.6%), long-term complications with ophthalmoxerosis (10 cases, 35.7%) and decreased visual acuity (1 case, 3.6%).@*Conclusion@#Radiotherapy is an effective method for primary orbital lymphoma, and the adverse reactions can be tolerated for most patients.

A Case of Surgical Diagnosis and Treatment of Idiopathic Orbital Myositis with Sudden Vision Loss

PURPOSE: We report a case of idiopathic orbital myositis with sudden onset of unilateral visual loss and hypertrophy of the lateral rectus muscle at the apex of the orbit that was diagnosed using orbital biopsy and treated with intravenous corticosteroids. CASE SUMMARY: We examined a 20-year-old woman who complained of decreased visual acuity and binocular diplopia for 2 weeks. She showed 40-prism diopter left esotropia at the primary position. Her visual acuity with eyeglasses was 0.7 and 0.2 in the right and left eyes, respectively. Brain magnetic resonance imaging (MRI) revealed a round mass on the left lateral rectus muscle at the apex of the orbit with optic nerve compression. We planned orbital biopsy to exclude orbital lymphoma and other biopsy-requiring diseases. After 3 weeks, the visual acuity of her left eye decreased to 0.02. Repeated MRI revealed enlargement of all extraocular muscles on the left orbit. With biopsy showing several lymphocytes infiltrating muscle fibers, we made a diagnosis of idiopathic orbital myositis. Intravenous injection of corticosteroids was administered during hospitalization. After 2 weeks of corticosteroid therapy, the visual acuity of her left eye was improved to 0.63, the esotropia disappeared, and the enlargement of the left lateral rectus muscle was improved on orbital MRI. CONCLUSIONS: For non-specific idiopathic orbital myositis located at the apex of the orbit and enlargement of the extraocular muscle in a short period with decreased visual acuity, administration of intravenous injection of corticosteroids before orbital biopsy could help restore vision.

A Case of Ocular Adnexal Benign Reactive Lymphoid Hyperplasia Recurred as Systemic Malignant Lymphoma

PURPOSE: We report a case of recurred systemic malignant lymphoma developed after the treatment for ocular adnexal benign reactive lymphoid hyperplasia. CASE SUMMARY: A 71-year-old female visited our hospital for right upper eyelid swelling and proptosis that had been progressing slowly for 4 years. Orbital computed tomography (CT) showed enlargement of the bellies of lateral and superior rectus muscles in the right orbit, but other abnormal findings were not detected in the systemic evaluation. Through incisional biopsy, benign reactive lymphoid hyperplasia was diagnosed on histopathologic examination. Upper eyelid swelling and proptosis resolved after radiation therapy. Fifty-one months after the treatment of benign reactive lymphoid hyperplasia, the patient visited our hospital again for a painful mass that had developed in the left neck. On neck CT, it showed enlargement of the left cervical lymph node and asymmetrical thickening of the left oropharyngeal wall. Systemic MALT lymphoma was confirmed, and radiation therapy was performed. According to neck CT, four months after radiation therapy, the enlargement and thickening resolved. CONCLUSIONS: Because there is a possibility of systemic malignant lymphoma after benign reactive lymphoid hyperplasia, continuous follow-up and repeated systemic evaluation should be required after treatment of ocular adnexal benign reactive lymphoid hyperplasia.

T-Cell Lymphoma Presenting as Painful Ophthalmoplegia

PURPOSE: To present a case of peripheral T-cell lymphoma presenting as painful ophthalmoplegia. METHODS: A 61-year-old woman presented with a 2-week history of headache and eyeball pain. Examination showed mild exophthalmos, complete ophthalmoplegia, and ptosis of the left eye. Under the impression of nonspecific orbital inflammation, she was treated with oral prednisone with initial response. Two months later, she revisited the clinic with exacerbated symptoms. Anterior orbitotomy and incisional biopsy was performed for the inferior rectus muscle lesion. RESULTS: Histopathologic examination revealed an infiltrate of atypical lymphoid cells between degenerative muscle bundles. It was consistent with peripheral T-cell lymphoma. A metastatic workup was performed without any evidence of extraorbital tumor. The patient was recommended to be treated with chemotherapy, however, refused to take the treatment. The patient died of progression of the disease in a month. CONCLUSIONS: T-cell lymphoma in the orbit can present as painful ophthalmoplegia and take a rapid clinical course. The disease should be regarded as one of the differential diagnosis for painful ophthalmoplegia refractory to corticosteroid therapy.

Treatment of Orbital and Ocular Adnexal Malignant Lymphoma

PURPOSE: The aim of this study was to evaluate the clinical features, histologic classification and treatment outcomes of patients with malignant lymphoma of the eye. METHODS: The participants included 22 patients with malignant lymphoma of the orbit and ocular adnexa treated at our hospital between May 1994 and September 2001. The authors retrospectively analyzed the sex, age, location of mass, histopathologic type, stage and results of the treatment. RESULTS: Of the 22 patients, 14 cases were male and 8 were female. The age ranged from 26 to 68 years (mean age 43 years). The most common presenting complaint was slowly growing mass of the eye. Histopathologic studies revealed extranodal marginal zone B-cell lymphoma, MALT type in 20 cases (91%). The stages of lymphomas were included I AE in 14 cases (63%), II AE in 3 cases (14%), III AE in 3 cases (14%) and IV A in 2 cases (9%). Patients received radiotherapy and/or chemotherapy. Twenty out of 22 cases (91%) achieved a complete remission. During the mean follow-up of 26 months, two patients died of progression of the disease; one patient had extranodal marginal zone B-cell lymphoma, MALT type of stage III AE and the other patient had diffuse large cell lymphoma of stage II AE. CONCLUSIONS: Radiotherapy can produce durable remission in the majority of the patients with localized primary orbital lymphoma. Chemotherapy should be performed for the patients with highly malignant lymphomas or lymphomas with systemic manifestations.

Primary Orbital Lymphoma: A Retrospective Analysis of Results of Radiation Therapy / 대한방사선종양학회지

PURPOSE: This study evaluated the treatment outcomes, patterns of failure, and treatment related complications of primary lymphoma patients who received definitive radiation therapy. MATERIALS AND METHODS: A retrospective analysis was undertaken for 31 patients with primary orbital lymphoma at the Asan Medical Center between February 1991 and April 2001. There were 18 males and 13 females with ages ranging from 3 to 73 years (median, 44 years). The involved sites were 9 conjunctivae, 12 eyelids and 10 other orbits. The histological types were 28 MALT lymphomas (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type), 1 diffuse large B-cell lymphoma, 1 anaplastic large cell lymphoma and 1 lymphoblastic lymphoma. The Ann Arbor stages were all IE (100%). Ann Arbor stage III or IV patients were excluded from this study. Bilateral orbital involvement occurred in 6 cases. Radiation therapy was given with one anterior port of high energy electrons (6~16 MeV) for the lesions located at the anterior structures like the conjunctivae or eyelids. Lesions with a posterior extension or other orbital lesions were treated with 4 or 6 MeV photons with appropriately arranged portals. In particular, lens blocks composed of lead alloy were used in conjunctival or eyelid lesions. Twelve patients received chemotherapy. The median follow-up period was 53 months. RESULTS: The 5-year overall, cause-specific, and disease-free survival was 91%, 96%, and 80%, respectively. The complete response rate 6 months after radiation therapy was 100%. Local recurrences were observed in 2 patients at 16 and 18 months after completion of radiation treatment. They were salvaged with additional radiation therapy. Two patients developed distant metastases. A MALT lymphoma patient with a lung relapse was successfully salvaged with radiotherapy, but the other lymphoblastic lymphoma patient with bone marrow relapse expired. There were no severe complications but 5 patients developed radiation-induced cataracts and 2 patients developed dry eye. CONCLUSION: Most primary orbital lymphomas consisted of MALT lymphomas. Radiation therapy was a successful treatment modality for orbital lymphoma without any severe complications. In cases of local relapses, radiation therapy is also a very successful salvage treatment modality.

Non-Hodgkin's Lymphoma of the Orbit and Ocular Adnexa: Clinical Analysis of 22 Cases / 대한혈액학회지

BACKGROUND: Non-Hodgkin's lymphoma presenting in the eye is a rare disorder. The aim of this study was to evaluate the clinical features, treatment outcome and complications of patients with non-Hodgkin's lymphoma of the eye. METHODS: We designed retrospective, observational case study. The participants included 22 consecutive patients with non-Hodgkin's lymphoma of the orbit and ocular adnexa treated at the Yeungnam University Hospital between 1992 and 2001. RESULTS: There were 16 males (73%) and 6 females (27%). The median age at ocular presentation was 47. The most common presenting complaint was slowly growing mass at the eye. Histopathologic studies revealed extranodal marginal zone B-cell lymphoma, MALT type in 19 cases (86%), B-cell small lymphocytic lymphoma in one case, diffuse large B-cell lymphoma in one case, and lymphoplasmacytoid lymphoma/immunocytoma in one case. The stages of lymphomas were IA(E) in 17 cases (82%), IIA(E) in 2 cases (9%), IIIA(E) in 2 cases (9%). Radiotherapy alone was employed in 17 cases, chemoradiotherapy was done in 3 cases, chemotherapy alone was administered in one case. Nineteen out of 21 cases (90%) achieved a complete remission, and 5 year survival rate was approximately 90%. Subgroup analysis of the patients with lymphomas in stage IA(E) revealed that complete response rate was 100% and 5 year survival rate was 100% CONCLUSION: Radiotherapy alone can produce durable remission in the majority of the patients with localized extranodal marginal zone B-cell lymphoma, MALT type. However, combination chemotherapy or chemoradiotherapy should be considered in the patients with aggressive lymphomas or indolent lymphomas with systemic manifestation.

Primary lymphoma of the eye

Lymphomas in the eye tend to be localized and to have a better prognosis. We reviewed the clinical presentation, treatment outcome and complications of primary lymphomas of the eye at a single institute focusing on the clinical significance of adequate staging and the prognostic value of pathological subtypes. Twenty-four patients with lymphoproliferative lesion of the eye were treated and followed up. The orbit was the most commonly involved site. Twenty-one patients had unilateral diseases and three patients had bilateral. Histological findings revealed 22 malignant lymphomas, one inflammatory pseudotumor and one atypical lymphoid hyperplasia. Using the NCI Working Formulation, most of the malignant lymphomas were primarily low grade: diffuse small lymphocytic, 15; diffuse small cleaved three; diffuse large cell, three; and diffuse mixed, one. The stages of lymphoma were IAE in 21 patients, IBE in one patient, and IIAE in two patients. Twenty-five of the 27 treated eyes remained disease-free during the median follow-up period of 16 months. Nineteen patients received radiotherapy, five patients received chemo-radiotherapy and one patient received only surgical excision. Two patients relapsed outside the treated field. None of the patients complained of severe dry eye syndrome or cataract formation. In conclusion, after complete staging work-up, locally-confined low grade orbital lymphomas can be up to 90% controlled with radiotherapy alone. By contrast, combined treatment is advised in patients with intermediate and high grade lesions.