Ocular adnexal diffuse large B-cell lymphoma: local disease correlates with better outcomes.

PURPOSE: To describe the clinical, immunohistochemical and prognostic features, as well as outcomes of a large series of patients with orbital and periorbital diffuse large B-cell lymphoma (DLBCL). DESIGN: This study is a multicentre, retrospective non-comparative consecutive case series. METHODS: The setting for this study was institutional. A total of 37 consecutive patients identified from the institutions' databases with periorbital and orbital DLBCL were enrolled in the study. A retrospective chart review was used for observation. The main outcome measures were patient demographics, clinical features, imaging, immunohistochemical and histopathological data, treatments administered, and survival. RESULTS: A total of 20 out of 37 cases (54.1%) represented localised periorbital disease (group L), 11 of 37 (29.7%) had systemic disease at presentation with periorbital disease (group S1), and 6 of 37 (16.2%) had previous history of systemic lymphoma (group S2). In all, 28 out of 30 (93.3%) patients were CD20+, 5 of 25 (20%) were CD3+, and 11 of 11 (100%) were CD79a+ (varying denominators reflect the different numbers of patients tested). A total of 25 out of 32 patients (78.1%) received chemotherapy, 14 (43.8%) received rituxmab plus chemotherapy, and 19 (59.3%) received radiotherapy. Nine deaths occurred, one in group L (not lymphoma related), six in group S1, and two in group S2. Five-year Kaplan-Meier survival estimates were 55.9% for all cases, 90.9% for group L, 36.0% for group S1, and 0% for group S2. One-year progression-free survival estimates in groups S1 and S2 combined were 58.3% for patients treated with rituximab and 28.6% for those who were not. CONCLUSIONS: To our knowledge, this report represents the largest series of patients with periorbital and orbital DLBCL in the literature. The difference in survival between groups L, S1 and S2 was striking, reflecting the grave prognosis of systemic DLBCL, but conversely the relatively optimistic outlook for patients with localised disease. Rituximab plus chemotherapy may be associated with increased survival.

Craniofacial fibrous dysplasia: clinical characteristics and long-term outcomes.

AIM: To present the clinical features and management outcomes in a large longitudinal series of patients with craniofacial fibrous dysplasia (CFD). METHODS: Retrospective interventional consecutive case series. Main outcome measures included signs and symptoms, radiographic findings, long-term outcomes, and postoperative complications. RESULTS: A total of 42 patients with CFD were identified. The mean age at presentation was 16.7 years; mean follow-up was 12.6 years. Out of these 42 patients, 37 (88.1%) had unilateral involvement and 5 (11.9%) had bilateral involvement, of which 3 (7.1%) had McCune-Albright syndrome. The commonest presenting symptom was facial asymmetry (36 cases, 86%). The frontal bone was the most commonly involved (27 cases, 64.3%), zfollowed by the sphenoid (24 cases, 57.1%). The most common pattern of bone involvement was monostotic (32 cases, 76.2%). Radiological optic canal involvement occurred in 18 eyes of 15 (37.5%) patients, with optic atrophy in 9 eyes (18.8%) of 7 patients (16.7%). Surgical intervention was performed in 30 (71.4%) cases for both functional and reconstructive reasons. Optic canal decompression was performed in three cases, in all of which stabilization of vision was achieved; no patient lost vision as a result of surgery. CONCLUSIONS: In this large longitudinal series of CFD, visual loss was not uncommon and occurred insidiously. The presenting clinical and radiological features, surgical interventions, and outcomes are discussed.

Are we training junior doctors to respond to major incidents? A survey of doctors in the Wessex region.

OBJECTIVE: To assess the current status of awareness and training of junior medical staff in the Wessex region in the event of a "conventional" major incident. METHODS: A telephone questionnaire of specialist registrars (SpRs) (or equivalent, for example, staff grade) in six core specialties was performed in all the 11 acute hospitals in the Wessex region on the same evening. This group was selected to represent a sample of the most senior medical staff "on site" at each hospital. RESULTS: 56 of 64 (87.5%) SpRs participated. Nine of the 56 (16%) SpRs questioned had previously been involved in a major incident, and 18 (32%) had experienced some form of major incident training exercise. Subgroup analysis of the specialties showed that although there were no significant differences in numbers of training experiences between specialties, only one of nine (11%) orthopaedic SpRs had ever been involved in a training exercise. Twenty five of the 56 (45%) SpRs felt that they were confident of their role in the event of an incident. CONCLUSION: Most middle grade staff in Wessex were not confident of their role in the event of a major incident. Most SpRs questioned had never attended a major incident training exercise.