Ocular Adnexal Lymphoma Research Articles

Systemic Rituximab Immunotherapy in the Management of Primary Ocular Adnexal Lymphoma: Single Institution Experience

Purpose: To evaluate the efficacy of systemic rituximab immunotherapy in the management of primary ocular adnexal lymphomas (OAL).Materials and methods: Clinical records of 10 consecutive patients (11 eyes) with biopsy-proven OAL managed with systemic anti-CD20 monoclonal antibody (rituximab; 375 mg/m2 intravenously once every three weeks for 6–8 cycles) between June 2008–March 2013 were evaluated retrospectively. Orbital magnetic resonance imaging and positron emission tomography were performed to evaluate any orbital and systemic involvement, respectively. Clinical response was classified as complete or partial.Results: The age of patients ranged between 27–85 (median, 55) years. Nine patients (90%) presented with unilateral and one (10%) with bilateral conjunctival involvement. Orbit was affected in 4 patients (40%), one of which had also choroidal involvement (10%). None of the patients had systemic involvement at initial presentation. All patients received an average of 7 cycles (range, 6–8) of systemic immunotherapy. After a median follow-up of 31 months (range, 10–61 months), complete response without recurrence could be achieved in 4 eyes (36%) with rituximab monotherapy. No systemic or ocular side effects were observed in any patient. Additional radiotherapy was required in 6 patients (7 eyes; 64%) with partial response or recurrence.Conclusions: Complete regression of primary OALs without recurrence was observed in about one-third of eyes after systemic rituximab monotherapy. Adjunctive radiotherapy was required in remaining two-thirds of the cases to achieve complete response. Thus, considering the balance between high rate of local control and potential ocular complications of radiotherapy, systemic rituximab can be considered as a first-line therapeutic option in the management of primary OAL.

Treatment Outcome of Primary Ocular Adnexal MALT Lymphoma by Radiation Therapy: Prognostic Effect of the AJCC Tumor-Node-Metastasis Clinical Staging System for Ocular Adnexal MALT Lymphoma

Treatment Outcome of Primary Ocular Adnexal MALT Lymphoma by Radiation Therapy: Prognostic Effect of the AJCC Tumor-Node-Metastasis Clinical Staging System for Ocular Adnexal MALT Lymphoma

Predictive Factors for Ophthalmic Toxicity in Patients Receiving Radiation Therapy for Ocular Adnexal or Periorbital Lymphoma

Predictive Factors for Ophthalmic Toxicity in Patients Receiving Radiation Therapy for Ocular Adnexal or Periorbital Lymphoma

The Outcome of Limited-Stage Plasmablastic Lymphoma Treated With and Without Radiation Therapy: A Single-Institutional Experience

Purpose/Objective(s): To quantify the risks of acute and late ophthalmic toxicity in lymphoma patients receiving radiation therapy (RT) to ocular or periorbital structures, and to identify dosimetric factors predictive of these risks. Materials/Methods: The study population included 30 consecutive lymphoma patients who received radiation therapy to ocular or periorbital structures at our institution from 2004 to 2010. Dose-volume histograms and dosimetric data were generated for the lacrimal glands, lenses, globes and orbits bilaterally. Radiation-related toxicities including conjunctivitis, xerophthalmia and cataracts were collected from the medical record and adverse effects were graded using the Common Terminology Criteria for Adverse Events (CTCAE) v4.0. Descriptive statistics were used to characterize dosimetric factors and the distribution of toxicities. Results: The median age at diagnosis was 65 (range, 31 to 89). Marginal zone lymphoma was the most common histology (10 cases), followed by diffuse large B-cell lymphoma (6 cases). 11 patients (37%) received treatment to the bilateral orbits. 3D conformal therapy was used in all cases, including intensity-modulated radiation therapy in 3 cases. The median prescribed dose was 2750 cGy (range, 1980 cGy to 5000 cGy). At a median follow up of 51 months, there were no local relapses. Within the first 6 months after initiating radiation therapy, 3 patients (10%) experienced grade 2 ophthalmic toxicity comprising acute conjunctivitis or xerophthalmia. Beyond 6 months, 17 patients (57%) subsequently manifested late grade 2 toxicity comprising xerophthalmia requiring intervention or cataracts requiring surgery. There was no correlation between maximum lens dose (range 7 cGy e 3967 cGy) and the development of cataracts in the study cohort (p Z 0.42). Beyond 6 months, the lacrimal gland V5 and globe V10 were significantly correlated with the development of late xerophthalmia and grade 2 toxicity. V5 covering the entire (100%) lacrimal gland and V10 covering the entire globe were associated with late xerophthalmia (p Z 0.03 and p Z 0.006, respectively) and with the development of grade 2 toxicity (p Z 0.003 and p Z 0.005, respectively). The median globe V10 was 39.8% for patients without late xerophthalmia, in contrast to 100% for those who did develop late xerophthalmia (p Z 0.006). Conclusions: In patients receiving radiation therapy for ocular adnexal or periorbital lymphoma, attempts to spare part of the lacrimal gland and limit the globe V10 to less than 40% may reduce the risk of late xeropthalmia. Author Disclosure: L.Z. Braunstein: None. Y. Chen: None. S.W. Lee: None. P.M. Mauch: None. A.K. Ng: None.

Clinical Outcome of Radiation Therapy for Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphomas

role of radiation therapy in localized (Ann Arbor stage I/II) NGNO MALT lymphoma. Materials/Methods: We retrospectively reviewed 47 patients who were histologically diagnosed as NGNO MALT lymphoma with Ann Arbor stage I/II from Feb 1993 to Oct 2012. Primary sites were head and neck (n Z 19), gastrointestinal tract (n Z 8), lung (n Z 7), and others. Their median age was 53 years (range 15-83 years). Forty-one patients had relatively good performance status (Eastern Cooperative Oncology Group performance score 1). Twenty-nine patients received radiation therapy (RT) containing treatment (14 RT alone, 11 surgery + RT, 2 chemotherapy (CTx) + RT, 2 surgery + RT + CTx) with a median RT dose of 30.6 Gy (22.5 to 45 Gy). Other 18 patients did not receive RT (9 surgery alone, 5 surgery + CTx, 4 CTx alone). Results: Median follow-up time was 45.6 months (range 4.8-224.5 months). The estimated 5-year progression-free survival (PFS) and overall survival rates were 61% and 92%, respectively. Among the stage I/II patients, 29 patients who received RT all achieved complete remission (CR) and showed significantly higher 5-year PFS than who did not receive RT (82% vs 34%; p Z 0.003). Four patients among these 29 patients experienced out-field relapse, which were well salvaged by RT (3 patients) or CTx (1 patient), and there was no in-field relapse. Ten out of 18 patients (61%) who did not receive RT with stage I/II achieved CR, and 11 out of these 18 patients have relapsed while 4 patients progressed into diffuse large B-cell lymphoma. Three of them who showed high grade transformation died within 3 months since relapse. Conclusions: In localized NGNO MALT lymphoma, low dose RT resulted in excellent outcome and relapses out of RT field were also successfully salvaged by RT. Early use of RT should be considered in localized NGNO MALT lymphoma and should be applied as a preferred treatment modality in case of localized relapse. Author Disclosure: J. Lee: None. K. Kim: None. J. Cho: None. C. Suh: None.

Follicular lymphoma of the ocular adnexal region: a nation-based study.

To characterize the clinicopathological features of follicular lymphoma of the ocular adnexal region. Retrospective nation-based study of Danish patients with ocular adnexal follicular lymphoma from January 1st 1980 through December 31st 2009. Twenty-four patients with ocular adnexal follicular lymphoma were identified. Fourteen (58%) of the patients were females. The median age was 63 years (range: 42-96 years). Eleven (46%) of the patients had primary ocular adnexal lymphoma, seven (29%) had an ocular adnexal lesion in conjunction with a concurrent systemic lymphoma and six patients (25%) presented with an ocular adnexal relapse. The most frequently affected sites were the lacrimal gland (38%) and the orbit (33%). Thirteen patients (54%) presented with Ann Arbor stage IE lymphoma, four (17%) had stage IIE, two patients (8%) stage IIIE, and five patients (21%) had stage IV lymphoma. Radiotherapy was primarily used in patients with primary lymphoma and those with a stage IE/IIE relapse (82%), while stage IIIE/IV lymphomas most frequently received alkylating chemotherapy (67%). Complete remission was observed in 19 of the patients (79%), but of these 11(58%) had a relapse. The 10-year overall survival for the entire cohort was 59%. The translocation t(14;18) was detected in 16 patients (16/24, 76%). Recurrence was only observed in patients with the t(14;18) (p=0.05, log-rank). Ocular adnexal follicular lymphoma is more commonly found in elderly female patients. The lacrimal gland is relatively frequently involved. Radiotherapy is the treatment of choice for localized ocular adnexal follicular lymphoma providing a favourable prognosis for majority of patients.

Ocular adnexal lymphoma: Five case reports and a literature review

This article reports the clinical course and treatment of ocular adnexal lymphoma based on a retrospective review of five cases with a histologically approved ocular adnexal lymphoma at Kaohsiung Veterans General Hospital over 10 years. Extranodal B-cell lymphoma in the orbit, lacrimal gland, eyelid, or conjunctiva was found in these patients. Four of them were female, and they were aged 45–64 years. All patients were also consulted with hematologists for possible systemic involvement and therapeutic plan. The patient with retrobulbar and orbital apex involvement received systemic chemotherapy. The patient with lacrimal gland involvement experienced tumor recurrence after local excision, and therefore received adjuvant radiotherapy. The remaining three patients had localized lymphoma on the eyelid or bulbar conjunctiva, and they all showed no recurrence after surgical excision. The incidence of ocular adnexal lymphoma has risen worldwide over the last few decades. Although most cases are confined to ocular adnexal, some may also be associated with disseminated lymphoma. Accurate diagnosis and staging is mandatory for appropriate treatment. Generally speaking, localized and low-grade ocular adnexal lymphoma involved eyelid or conjunctiva seem to have good outcome after surgical excision only. Systemic chemotherapy should be considered in patients with advanced disease or systemic manifestations, and radiotherapy also offers a good choice for lacrimal gland lymphoma.

Ocular adnexal follicular lymphoma: a multicenter international study.

The clinical features of the follicular subtype of ocular adnexal lymphoma (OAL) have not been previously evaluated in a large cohort. To characterize the clinical features of follicular OAL. We performed a retrospective multicenter study that involved 6 eye cancer centers from January 1, 1980, through December 31, 2010. A total of 105 patients with follicular OAL were identified, of which 7 patients were excluded because of missing clinical data. The median follow-up time was 52 months (range, 13-118 months). Overall survival, disease-specific, and progression-free survivals were the primary end points. Ninety-eight eligible patients with follicular OAL were included; 60 (61%) were women. The median patient age was 63 years (range, 32-96 years). Sixty-nine patients (70%) had primary OAL, 19 (19%) had OAL in conjunction with a concurrent systemic lymphoma, and 10 (10%) presented with an ocular adnexal relapse. The lacrimal gland (28%), conjunctiva (28%), and orbit (28%) were the most frequently involved sites. Of the 69 patients with primary follicular lymphoma, 38 (55%) presented with Ann Arbor stage IE lymphoma, and 31 (45%) had stage IIE lymphoma. Patients with disseminated lymphoma had stage IIIE (9 of 19 [47%]) and stage IV (10 of 19 [53%]) disease, whereas patients with a relapse of systemic lymphoma presented with stage IE (8 of 10 [80%]), stage IIE (1 of 10 [10%]), and stage IIIE (1 of 10 [10%]) disease. Patients with primary follicular lymphoma (n = 69) and those with isolated ocular relapse (n = 9) were treated with external beam radiation therapy (EBRT) (35 of 78 [45%]) or EBRT plus chemotherapy (22 of 78 [28%]). Patients presenting with stage IIIE-IV follicular lymphoma (n = 20) most frequently received chemotherapy (9 of 20 [45%]) or EBRT plus chemotherapy (4 of 20 [20%]). The 10-year overall survival for the entire study cohort was 60%. Primary patients treated with EBRT had a better disease-specific survival compared with patients receiving ERBT plus chemotherapy (10-year disease-specific survival, 94% vs 40%; P = .02 by log-rank test). Follicular OAL was more commonly found in elderly female patients. These tumors were equally noted to involve the conjunctiva, lacrimal gland, and orbit. Patients with ocular adnexal follicular lymphoma primarily treated with EBRT had a more favorable long-term disease-specific survival.

Systemic rituximab immunotherapy in the management of primary ocular adnexal lymphoma: Single institution experience.

e19503 Background: To evaluate the efficacy of systemic rituximab immunotherapy in the management of primary ocular adnexal lymphomas (OAL). Methods: Clinical records of 10 consecutive patients (11...

Expression of vascular endothelial growth factor in human ocular adnexal lymphoma.

To examine the expression of VEGF in extranodal marginal zone B-cell lymphoma (EMZL) and reactive lymphoid hyperplasia (RLH) of human ocular adnexa, and analyze the correlation with the intratumoral microvessel density (MVD). Twenty-two EMZL and 16 RLH tissues were examined in this study. Paraformaldehyde-fixed, paraffin-embedded tissue sections were processed for immunohistochemistry with antibodies against VEGF and CD20. Vascular endothelial growth factor expression was analyzed using the ELISA and RT-PCR in the EMZL tissues. Microvessel density was determined based on the immunoreactivity for anti-CD34 antibody. Vascular endothelial growth factor immunoreactivity was detected in the cytoplasm of lymphoid cells in EMZL and RLH. ELISA and RT-PCR confirmed VEGF protein and mRNA expressions in the EMZL tissue, respectively. Vascular endothelial growth factor-immunopositive rate in B-cells was significantly higher in 12 conjunctival EMZLs than four RLHs (P < 0.01) and 10 orbital EMZLs than 12 RLHs (P < 0.05). The MVD showed a significant positive correlation with the VEGF-immunopositive rate in conjunctival and orbital EMZLs. This study demonstrated increased VEGF expression in human conjunctival and orbital EMZL compared with that in RLH, suggesting that VEGF plays a significant role in the pathogenesis and tumor angiogenesis of ocular adnexal lymphoma.

Crystal-Storing Histiocytosis Masquerading Ocular Adnexal Lymphoma

A case of crystal-storing histiocytosis (CSH) associated with mucosa-associated lymphoid tissue (MALT) lymphoma of orbit is reported. The patient was a 53-year-old man who presented with an 8-year history of a slowly enlarging tumor in his right orbit. Histopathologic examination revealed that the tumor was composed predominantly of sheets of spindle-shaped cells resembling striated muscle cells and scattered aggregates of atypical lymphoid cells, showing prominent plasmacytoid differentiation. Immunohistochemical analysis demonstrated that the spindle-shaped cells were CD68-positive histiocytes containing abundant crystals in their cytoplasm, consistent with the diagnosis of CSH. The aggregates of atypical lymphoid cells were diagnosed as MALT lymphoma based on their immunophenotype. Although CSH is a well-recognized manifestation in lymphoproliferative disorders, CSH complicated by MALT type of ocular adnexal lymphoma has rarely been reported. Given the rarity of this, every case presenting with such crystal-storing histiocytes warrants a thorough search for a hidden lymphoid dyscrasia.

Lymphoma of the ocular adnexa.

Over the last decades, the incidence of ocular adnexal lymphomas has been increasing. Over the last decade advances in the imaging of ocular adnexal lymphomas have enabled precise biopsies of the tumors. Biopsies are necessary for immunophenotyping, correct molecular classification and the immediate start of the appropriate treatment. In a retrospective study, we collected data from 16 patients (19 eyes) treated for lymphoma of the ocular adnexa between 2006 and 2011 with various tumor sites. We evaluated ocular symptoms of the patients in correlation to the tumor localization on imaging and determined the access of biopsy. Follow-up period (13.7 ± 15.4 months) and therapy were analyzed. The mean age of study patients was 67.4 ± 13.6 years. Seven patients presented with exophthalmos, 6 with upper lid swelling, 5 with double vision, 5 with reduced visual acuity and one patient with retinal detachment. None of the patients complained of pain. In 7 cases the location of the OAL was superior, in 6 retrobulbar, in 4 patients in the lacrimal gland (1 bilaterally affected), one case with lacrimal sac infiltration and another with isolated subconjunctival lymphoma. Patients with retrobulbar tumors complained of visual loss, whereas patients with anterior orbital tumors showed localized nodular swelling. In 11 cases (58%) the diagnosis was marginal zone B-cell lymphoma (MALT), in 5 (26%) follicular lymphoma. 11 patients (69%) received curative and one HIV-positive patient palliative radiation. Three patients were treated with systemic rituximab due to generalized lymphoma and one of them was complemented with CHOP therapy. One patient received chemotherapy alone (CHOP). During the follow-up the HIV-positive patient died. All our lymphoma patients showed full tumor regression, without side effects from radiotherapy. In this study we emphasize the need to perform a diagnostic biopsy. The risk of biopsy by a trained surgeon is small. Early and accurate diagnosis is crucial for proper treatment. In unclear situations repeated biopsies might be necessary.

Ocular adnexal lymphoma: validation of American Joint Committee on Cancer seventh edition staging guidelines

Background/aimsTo validate the prognostic significance of the American Joint Committee on Cancer (AJCC) seventh edition staging criteria for ocular adnexal lymphoma (OAL) of all histologic subtypes.MethodsRetrospective review of clinical records...

Ocular adnexal lymphomas: report of 2 cases of mantle cell lymphomas and short review of literature.

Mantle cell lymphoma in ocular region is a rare phenomenon which can be either primary or secondary. Most of these cases are usually diagnosed after excisional biopsy of the involved area with first visit being in the Ophthalmology OPD. We share our experience of two such cases being referred from Ophthalmology OPD. 1st case is about a 52-year-old man who came for complaints of redness of left eye with excessive lacrimation. Examination revealed congestion of left temporal bulbar conjunctiva and a small pinkish outgrowth 2×2cm adherent to temporal bulbar conjunctiva. 2nd case is a 55-year-old gentlemen who presented with complaints for 8months duration of swelling left eyelid. Excisional biopsy and histopathological examination in both the cases were done to confirm the diagnosis. CECT head and neck were done at baseline and during follow up. These cases are being presented due to the rarity and dramatic response to chemotherapy.

Current therapeutic concepts in mucosa-associated lymphoid tissue (MALT) lymphoma

While eradication of Helicobacter pylori leads to durable remissions in up to 75 % of patients with gastric mucosa-associated lymphoid tissue (MALT) lymphoma and therefore, has been well established as first-line therapy of choice, there is no standard of care for relapsed, refractory or extragastric MALT lymphoma. Both in gastric as well as extragastric MALT lymphoma, local therapy results in high rates of local control, but MALT lymphoma may be disseminated in a significant number of patients, making systemic treatment approaches reasonable. Different classical cytostatic agents have successfully been tested in the past. However, MALT lymphoma usually runs an indolent clinical course and thus, recent trials have focussed on chemotherapeutics with a favourable toxicity profile, e.g. chlorambucil or bendamustine with or without rituximab (R), and new immunomodulatory drugs, e.g. lenalidomide. Results are promising and further, and especially more mature data with a longer follow-up are awaited. In addition, efforts have been made to characterise the activity of antibiotic therapy in extragastric MALT lymphoma, particularly in the setting of ocular adnexal manifestations. Doxycyclin-treatment achieved response rates of 45–65 % in several series, which was independent of the presence of a Chlamydophila psittaci infection. Furthermore, the macrolide antibiotic clarithromycin has become a matter of interest for the treatment of ocular adnexal MALT lymphoma as this substance exerts not only antibiotic, but also direct anti-tumor and immunomodulatory effects. In view of recent data, this short review focuses on highlights and recent results of systemic and antimicrobial therapy in MALT lymphoma.

Localized Ocular Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma Treated With Radiation Therapy: A Long-Term Outcome in 86 Patients With 104 Treated Eyes

To evaluate the natural history, behavior of progression, prognostic factors, and treatment-related adverse effects of primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML). Eighty-six patients with histologically proven stage I POAML treated with radiation therapy at National Cancer Center Hospital, Tokyo between 1990 and 2010 were retrospectively reviewed. The median age was 56 years (range, 18-85 years). The median dose administered was 30 Gy (range, 30-46 Gy). Seventy-seven patients (90%) were treated by radiation therapy alone. The median follow-up duration was 9 years (range, 0.9-22 years). The 5- and 10-year overall survival (OS) rates were 97.6% and 93.5%, respectively, and no patients died of lymphoma. Patients with tumor sizes ≥4 cm showed a greater risk of contralateral relapse (P=.012). Six patients with contralateral relapse were seen and treated by radiation therapy alone, and all the lesions were controlled well, with follow-up times of 3 to 12 years. There was 1 case of local relapse after radiation therapy alone, and 3 cases of relapse occurred in a distant site. Cataracts developed in 36 of the 65 eyes treated without lens shielding and in 12 of the 39 patients with lens shielding (P=.037). The majority of patients with POAML showed behavior consistent with that of localized, indolent diseases. Thirty gray of local irradiation seems to be quite effective. The initial bilateral involvement and contralateral orbital relapses can be also controlled with radiation therapy alone. Lens shielding reduces the risk of cataract.

Orbital Immunoglobulin G4-Related Disease: A Systematic Review.

Immunoglobulin G4-related disease (IgG4-RD) is a clinically distinct systemic condition that can involve the orbital tissue. Characterized by a triad of a mass-forming lesion, infiltration by IgG4-positive plasma cells, and elevated serum IgG4 titers in many cases, IgG4-RD has clinicopathologic features that overlap with ocular adnexal lymphomas and orbital inflammatory conditions. Although most cases of orbital IgG4-RD respond well to steroids, it may become necessary to include supplemental immunosuppressant therapy in the management.

This issue ofActa, printed and electronic

The cover image is a schematic drawing of a retinal implant system for the blind. This is a described in an article by Velikay-Parel from Austria and Germany in the electronic part of this Issue of ACTA. Andrew and associates in Australia review inflammatory diseases in the orbit and their pathophysiology. Bjerrum et al. in Copenhagen found significantly higher risk of postoperative endophthalmitis after cataract surgery at a few private hospitals and clinics in Denmark compared with public hospitals. Hietala et al. in Helsinki and Australia found that individuals with higher age of onset of type 1 diabetes are more likely to develop macular oedema. Bertelsen et al. in Norway and UK found that microalbuminuria may be a useful risk predictor for diabetic retinopathy. van Dijk et al. in the Netherlands and USA found considerable variability in photocoagulation treatment plans influenced among other things by the assessment with slit lamp biomicroscopy versus OCT. Kofoed et al. in Copenhagen found abnormal cone function to correlate with carotid artery stenosis and reduced perfusion pressure. Vandewalle et al. Belgium and Iceland found that topical phenylephrine and tropicamide did not influence retinal oxygen saturation measurements or retinal vessel diameter. Ernest et al. in the Netherlands found a considerable prevalence of visual impairment in patients with glaucoma at the end of their life but the visual impairment was in many cases caused by other eye diseases. Ilmarinen et al. in Finland cultivated oral mucosal epithelium and treated severe ocular surface injuries with limbal stem cell deficiency. Konradsen and Zetterström in Stockholm found that flat cornea in Marfan syndrome reduces the myopic consequence of long axial length. Hirayama et al. in Japan describe symptomatic relief of ocular dryness and improved tear stability with moist cool air devices in offices. Pathak et al. in Oslo contribute a case series of patients who receive transplantation of ex vivo-expanded limbal epithelial cells with good results. González-López et al. in Spain found that only one temporal artery biopsy in four is positive in patients suspected of giant cell arteritis and suggest ways of improving this ratio. Næser et al. in Denmark present a case series of patients with Wegener's granulomatosis who underwent dacryocystorhinostomy. Peter Kristian Rasmussen presents his thesis on diffuse large B-cell and mantle cell lymphoma of the ocular adnexal region and lymphoma of the lachrymal gland. The following articles are published electronically only and are accessible on www.actaophthalmologica.com Lamoureux et al. in Australia and Singapore found good reliability and reproducibility with retinal oxygen saturation measurements. Velikay-Parel et al. in Austria and Germany tested an investigational epiretinal implant in a patient with retinitis pigmentosa. The device was found to be safe and stable. Koinzer et al. in Germany describe differences between OCT images and histology of photocoagulation lesions in retina. Hoffmann et al. in Germany, Italy and Portugal use Heidelberg retina tomography to study optic disc changes in people with ocular hypertension. They found strong associations between various structural optic nerve parameters. Saenz-Frances et al. in Spain find that peripheral corneal thickness may be important in intraocular pressure measurements and glaucoma in addition to central corneal thickness. Garcia-Martin et al. in Spain documented retinal nerve fibre layer loss in patients with multiple sclerosis, using OCT technology. Januschowski et al. in Germany used a microsensor to measure the wearing time of glasses and ocular patches in patients with amblyopia. Lara et al. in Italy examined gene mutations and immunoreactivity in conjunctiva melanomas. Montero et al. in Spain examined intravitreal drug dispersion with different size needles. Maschi-Cayla et al. in France used proton beam to treat conjunctival melanomas. Lesiewska-Junk et al. in Poland looked for oxidative stress markers on erythrocytes in patients with pseudoexfoliation. Tassignon et al. in Belgium used Scheimpflug camera to examine location of phakic intraocular lenses. Jakob and Van Calster in Belgium associate skin tattoos with uveitis. Brunner and Binder in Austria surgically excised retinal embolae. Purtskhvanidze et al. in Germany report open globe injuries from rotating wire brushes. Zhou et al. in China and Germany describe a series of bilateral retinal detachments. I wish you Merry Christmas and a Happy New Year and hope that you enjoy reading ACTA during the holidays.

Clinical Characteristics of 95 Patients With Ocular Adnexal and Uveal Lymphoma: Treatment Outcomes in Extranodal Marginal Zone Subtype

Lymphoma rarely presents in the ocular adnexa but is usually extranodal marginal zone (ENMZ) lymphoma when it does. Involved-field radiotherapy (IFRT) is the standard of care for unilateral disease, but the optimal management of more extensive disease is unclear. We retrospectively evaluated the clinical characteristics and outcomes of 95 patients with ocular adnexal lymphoma (OAL) or uveal lymphoma treated or diagnosed at our institution. All patients identified were included in the risk factor analysis for progression-free survival (PFS). The initial treatment-related outcomes were assessed for ENMZ OAL only (n= 62). With a median follow-up of 32 months, significant risk factors for PFS after initial treatment were age (hazard ratio, 1.33; 95% confidence interval, 1.02-1.74), female gender (hazard ratio, 2.04; 95% confidence interval, 1.04-4.00), and a history of lymphoma (hazard ratio, 2.31; 95% confidence interval, 1.12-4.78). In ENMZ, IFRT was associated with improved PFS (median, 5.4 years; P< .001). Progression occurred in 7 of 39 (23%), with 6 of the 7 (86%) at systemic sites. Single-agent rituximab was typically used for bilateral ocular or systemic presentations of ENMZ OAL. Progression occurred in 7of 11 (64%), with no progression at systemic sites. All progression events in those initially treated with rituximab occurred in the ocular adnexa. The results of the present study have confirmed IFRT as the standard for unilateral ENMZ OAL. Single-agent rituximab was an effective agent for bilateral ocular or systemic ENMZ OAL, particularly for systemic control, but ocular progression should be closely monitored. Combined modality therapy should be studied further in bilateral and systemic ENMZ OAL.

Comparison of American Joint Committee on Cancer TNM-based Staging System (7th edition) and Ann Arbor Classification for Predicting Outcome in Ocular Adnexal Lymphoma

Objective: To compare the TNM and Ann Arbor staging systems in predicting outcome in ocular adnexal lymphoma (OAL).Methods: Retrospective review of the clinical, imaging and histopathologic records of OALs between 1986 and 2009. Outcome measures included local recurrence and progression.Results: One hundred and sixty patients of OAL were included. Mean age was 65 ± 15 years (range 20–97) and 68 (43%) were male. The median follow-up of all OAL patients was 65 months (range 2.5–238). Histopathology identified low-grade, indolent B-cell lymphomas in 140 patients (87.5%) and rest had aggressive grades. Of 134 indolent OAL patients, those with unilateral disease had a 10-year progression free survival of 72% versus 48% in their bilateral counterparts (p = 0.001). Amongst unilateral OAL patients staged within the T1-2 group, a significantly better outcome was noted for patients without nodal or metastatic involvement compared to those with such involvement (p = 0.0001). The above observations helped to formulate a simple scoring system to prognosticate OALs based on their laterality and node/metastatic status. Amongst the 3 groups identified, group 1 with a score of 0 (unilateral OALs with no nodes or metastasis) had a 10-year progression free survival of 75%; group 2 with score 1 (either bilateral or positive nodes/metastasis) 50% and group 3 with score 2 (both bilateral OAL with positive nodes/metastasis) zero at 10 years (p < 0.00001).Conclusions: The TNM-based staging system better predicts outcome in OAL than the Ann Arbor system primarily by delineation of bilateral disease and nodal/metastatic involvement at presentation.