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.

Pleomorphic adenoma of the lacrimal gland: is there a role for biopsy?

AIM: To review the literature on biopsy of lacrimal gland pleomorphic adenoma (LGPA) and to examine the validity of the prohibition against biopsy in LGPA. METHOD: Literature review. RESULTS: LGPA is usually diagnosed preoperatively based on clinical and radiological characteristics, as current teaching advises complete excision without prior incisional biopsy. The caveat against biopsy is based on older studies that reported increased recurrence rates with increased risk of malignant transformation after incomplete excision or biopsy. On the basis of a detailed examination of the literature on biopsy of both LGPA and pleomorphic adenoma of the salivary glands, it appears that there is no clear evidence to support the claim that biopsy increases the risk of recurrence or of malignant transformation of LGPA. CONCLUSION: Lacrimal gland tumours are uncommon lesions and optimal management depends to a great extent on a definite preoperative diagnosis. Preoperative biopsy should therefore be considered in all lacrimal gland mass lesions and management should be tailored to the biopsy findings. If surgical resection is then required, it may be prudent to excise the biopsy tract to ensure complete removal of the tumour.

Metastatic renal cell carcinoma presenting as a circumscribed orbital mass.

PURPOSE: To report a case of renal cell carcinoma presenting as a well-circumscribed orbital tumor. METHODS: Retrospective interventional case report. RESULTS: A 60-year-old woman presented with proptosis of the left eye. Imaging showed a well circumscribed tumor in the region of the medial rectus muscle. Excision biopsy revealed a diagnosis of metastatic renal cell carcinoma that was confirmed on abdominal imaging. CONCLUSIONS: Renal cell carcinoma can rarely present as a well-circumscribed orbital mass and should be included in the differential diagnosis of such lesions.