Composite lymphoma with mantle and follicular cell components is a challenging diagnosis. Flow cytometry, immunohistochemistry and molecular genetics are required to distinguish the two components, as often the more aggressive one is predominant and masks the other. A 58-year-old man with history of nodal composite lymphoma presented with right exophthalmos and diplopia. A head CT scan showed an orbital tumor. A biopsy of the tumor revealed a mantle cell lymphoma predominating over a follicular lymphoma. Immunoglobulin heavy chain and light chain rearrangements analysis by PCR proved that both components of the orbital tumor were recurrences of the same nodal composite lymphoma diagnosed two years earlier. The nodal lymphoma was composed of a follicular lymphoma and an in situ mantle cell neoplasia. Consensus view is that dominant lymphoma should be treated when needed but taking into account if the mantle cell lymphoma is an in situ neoplasia and if it expresses CD5 and SOX11.

OBJECTIVE: Retinoblastoma is the most common primary ocular malignancy in children. Although currently retinoblastoma has an excellent survival rate in developed countries, hereditary retinoblastoma survivors as well as those with a history of radiation therapy as children are at an increased risk for second primary tumors (SPTs), and specifically, for orbital sarcomas. Despite the known increased risk for SPTs in retinoblastoma survivors and the associated morbidity and mortality, no screening or treatment guidelines exist.
RESULTS: Understanding of retinoblastoma tumorigenesis and genomic expression has expanded significantly, and treatment has evolved with a shift away from radiotherapy. Until the last two decades, however, radiation was the therapy of choice for patients with bilateral disease. Because both hereditary retinoblastoma and radiation are independent risk factors for the development of SPTs such as sarcomas and these SPTs are often fatal, appropriate surveillance for retinoblastoma survivors is crucial.
CONCLUSIONS: As a result of the excellent survival rates for retinoblastoma patients, it is important to: recognize the risk of sarcoma, particularly in patients with hereditary retinoblastoma and/or prior radiation therapy; establish a screening protocol, such as the one proposed, to maximize early detection; and discuss and develop treatment guidelines for high-risk patients. Future directions of research for these patients will involve the development of molecularly targeted agents and the use of proton radiotherapy.

Extranodal lymphomas (ENLs) comprise about a third of all non-Hodgkin lymphomas (NHL). Radiation therapy (RT) is frequently used as either primary therapy (particularly for indolent ENL), consolidation after systemic therapy, salvage treatment, or palliation. The wide range of presentations of ENL, involving any organ in the body and the spectrum of histological sub-types, poses a challenge both for routine clinical care and for the conduct of prospective and retrospective studies. This has led to uncertainty and lack of consistency in RT approaches between centers and clinicians. Thus far there is a lack of guidelines for the use of RT in the management of ENL. This report presents an effort by the International Lymphoma Radiation Oncology Group (ILROG) to harmonize and standardize the principles of treatment of ENL, and to address the technical challenges of simulation, volume definition and treatment planning for the most frequently involved organs. Specifically, detailed recommendations for RT volumes are provided. We have applied the same modern principles of involved site radiation therapy as previously developed and published as guidelines for Hodgkin lymphoma and nodal NHL. We have adopted RT volume definitions based on the International Commission on Radiation Units and Measurements (ICRU), as has been widely adopted by the field of radiation oncology for solid tumors. Organ-specific recommendations take into account histological subtype, anatomy, the treatment intent, and other treatment modalities that may be have been used before RT.

OBJECTIVE: To establish guidelines for interpretation of orbital imaging by magnetic resonance imaging (MRI) and/or computed tomography (CT), and to apply these guidelines and examine their predictive value in 131 patients with biopsy-proven orbital tumors.
METHODS: Prospective evaluation of imaging studies.
METHODS: Imaging studies (CT and/or MRI) from 131 cases with biopsy-proven orbital tumors.
METHODS: Guidelines for reviewing orbital imaging studies (MRI and/or CT) were established based on 5 major characteristics: (1) anatomic location, (2) bone and paranasal sinuses involvement, (3) content, (4) shape, and (5) associated features. In total, 84 features were established by an experienced orbital surgeon and a neuroradiologist. Applying these 84 features, imaging studies of 131 biopsy-proven orbital tumors were evaluated by 3 physicians.
METHODS: Imaging features: characteristics, sensitivity, specificity, and positive and negative predictive values in various groups of orbital tumors and kappa values.
RESULTS: One hundred thirty-one cases of biopsy-proven orbital tumors were evaluated. Benign lesions were more likely to be smaller in size, round or oval in shape (29% of all benign tumors, 0% in malignant and inflammatory, P<0.001), and associated with hyperostosis (22% of all benign lesions, P<0.001). They were also more likely to be hyperdense or hypodense on CT imaging (15% and 11%, respectively; P<0.05 in comparison with inflammatory and malignant tumors). Inflammatory processes showed panorbital involvement (23% vs. 3%, and 0% in benign and malignant tumors, respectively; P<0.001). Orbital fat involvement and fat stranding were noticed only in inflammatory lesions (19% and 16%, respectively; P<0.001). None of the features occurred only in malignant tumors, but they tend to involve the anterior orbit more commonly (54% vs. 20%, and 29% in benign and malignant; P = 0.002), and were more likely to show bone erosion (31% vs. 6%, and 16% in benign and inflammatory tumors, respectively; P = 0.004) and molding around orbital structures (29% vs. 3% in benign, and 0% in inflammatory tumors, respectively; P<0.001). Features such as panorbital involvement, orbital fat, frontal sinus opacity, molding around orbital structures, perineural involvement, and fat stranding had specificity of 97% to 100%, but low sensitivity.
CONCLUSIONS: Guidelines for analysis of orbital imaging studies (CT or MRI) are suggested. Based on these guidelines several imaging features showed significantly different occurrences in benign, malignant, and inflammatory processes; although this can help in differential diagnosis, tissue diagnosis may still be required.

The ocular adnexal lymphomas represent the malignant end of the spectrum of lymphoproliferative lesions which occur in the conjunctiva, eyelids, lacrimal gland and orbit. The new \"W.H.O. Classification of Tumours of Haemopoietic and Lymphoid Tissues\" is the most suitable for subdividing the ocular adnexal lymphomas, whereby the extranodal marginal zone B-cell lymphoma (EMZL) represents the most common lymphoma subtype. Management of patients with ocular adnexal lymphomas includes a systemic medical examination to establish the clinical stage of the disease. Most patients have stage IE disease and current recommended therapy for this is radiotherapy, while disseminated disease is treated with chemotherapy. Despite usually demonstrating an indolent course, EMZLs are renowned for recurrence in extranodal sites, including other ocular adnexal sites. Furthermore, Blastic transformation of EMZL with a corresponding aggressive clinical course has been described. Long-term follow-up with half-yearly examinations are therefore recommended. Major prognostic criteria for the ocular adnexal lymphomas include the age of the patient, anatomical location of the tumour, stage of the disease at first presentation, serum lactate dehydrogenase level at the time of diagnosis, lymphoma subtype as determined using W.H.O. lymphoma classification and the tumour cell growth rate. The clinical symptoms and histopathological findings of the differential diagnosis of lymphoproliferative lesions of the ocular adnexa are discussed.

OBJECTIVE: Orbital venous anomalies and lymphangiomas have been generally classified on morphologic grounds. However, conflicting concepts of these lesions have hampered scientific dialogue and confused clinicians, with occasionally serious consequences for patients. As a step toward uniform nomenclature and better communication, a consensus on the terminology of orbital vascular malformations was sought among the members of the Orbital Society.
METHODS: A classification of orbital vascular malformations based on their hemodynamic relationships, as revealed by clinical and imaging features, was presented, discussed, and adopted at the 1998 Annual Meeting of the Orbital Society. A consensus statement was drafted and distributed to all members. The statement was then modified in accordance with members\' responses, which included minor stylistic recommendations but no substantive dissent.
RESULTS: Orbital vascular malformations may be classified according to their hemodynamic relationships as no flow, venous flow, and arterial flow lesions. Assignment to each group is based on pertinent clinical and imaging criteria. Mixed forms with both venous and no flow components are grouped within the venous flow category to emphasize the clinical importance of that relationship.
CONCLUSIONS: A hemodynamic classification of orbital vascular malformations emphasizes features most germane to their management. It should reduce the clinical confusion resulting from purely morphologic differentiation. Widely accepted definitions may allow a better comparison of therapeutic protocols for these troublesome lesions.