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Background/aims: To validate the prognostic performance of the American Joint Committee on Cancer (AJCC) eighth edition classification for ocular adnexal lymphoma (OAL)., Methods: We performed a retrospective review of 140 consecutive patients treated for primary OAL between March 2010 and September 2017. Associations between T/N/M categories at presentation and disease-related outcomes, including relapse, progression-free survival (PFS) and overall survival (OS) were evaluated., Results: Seventy-nine women and 61 men (median age, 52 (range 20-84) years; median follow-up, 57 (range 7-131) months) were included. Histological subtypes included mucosa-associated lymphoid tissue lymphoma (92.1%, n=129), diffuse large B-cell lymphoma (5.0%, n=7), follicular lymphoma (1.4%, n=2) and mantle cell lymphoma (1.4%, n=2). Patients with ≥T2 disease had significantly higher risks of overall relapse (unadjusted HR)=4.32, p=0.016), decreased PFS (uHR=5.19, p=0.004) and decreased OS (uHR=9.21, p=0.047). Patients with ≥N1 disease had significantly higher risks of overall relapse (uHR=9.17, p<0.001) and decreased PFS (uHR=9.24, p<0.001). M1 disease was significantly associated with higher risks of overall relapse (uHR=3.62, p=0.036), decreased PFS (uHR=5.13, p=0.001) and decreased OS (uHR=9.24, p=0.013). On considering TNM categories as continuous data, the uHRs for per level increase in T, N and M categories were 1.77, 1.83 and 2.30 for overall relapse and 1.72, 1.87 and 2.78 for decreased PFS, respectively (p<0.05 for each comparison)., Conclusion: The T, N and M categories of the AJCC eighth edition classification have prognostic value for relapse and survival among patients with primary OAL. Particularly, nodal/metastatic involvement at presentation indicated less favourable outcome., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To report our institution's treatment techniques, disease outcomes, and complication rates after radiotherapy for the management of lymphoma involving the orbits., Patients and Methods: We retrospectively reviewed the medical records of 44 patients curatively treated with radiotherapy for stage IAE (75%) or stage IIAE (25%) orbital lymphoma between 1969 and 2013. Median follow-up was 4.9 years. Thirty-eight patients (86%) had low-grade lymphoma and 6 (14%) had high-grade lymphoma. Radiation was delivered with either a wedge-pair (61%), single-anterior (34%), or anterior with bilateral wedges (5%) technique. The median radiation dose was 25.5 Gy (range, 15 to 47.5 Gy). Lens shielding was performed when possible. Cause-specific survival and freedom from distant relapse were calculated using the Kaplan-Meier method., Results: The 5-year local control rate was 98%. Control of disease in the orbit was achieved in all but 1 patient who developed an out-of-field recurrence after irradiation of a lacrimal tumor. The 5-year regional control rate was 91% (3 patients failed in the contralateral orbit and 1 patient failed in the ipsilateral parotid). Freedom from disease, cause-specific survival, and overall survival rates at 5 and 10 years were 70% and 55%, 89% and 89%, and 76% and 61%, respectively. Acute toxicity was minimal. Ten patients (23%) reported worsened vision following radiotherapy, and cataracts developed in 17 patients. Cataracts developed in 13 of 28 patients treated without lens shielding (46%) and 4 of 16 patients (25%) treated with lens shielding., Conclusion: Radiotherapy is a safe and effective local treatment in the management of orbital lymphoma.

Objective: To explore the relationship between IgH gene rearrangement and orbital MALT (mucose-associated lymphoid tissue) lymphoma removal operation prognosis, and to quantify the effect of IgH gene rearrangement on primary orbital MALT lymphoma prognosis., Patients and Methods: Fifty-eight patient cases with primary orbital MALT lymphoma were included in this study. Orbital lymphoma specimens were embedded in paraffin for sectioning. IgH gene rearrangement was detected using PCR. The correlation between IgH gene rearrangement and the patient recurrence and survival rates were determined using statistical analysis. The aforementioned rates were calculated and a survival curve was determined. p-values lower than 0.05 was considered statistically significant., Results: We found that the 5-year disease-free survival rate was 90.8% in patients with orbital MALT lymphoma (mean value 56.7 months, range 52-60 months). The use of IgH gene rearrangement detection methods found that the non-recurrence rate of primary orbital MALT lymphoma cases was 79.3%. Survival analysis revealed that IgH gene rearrangement was significantly correlated with recurrence of orbital MALT lymphoma (p<0.001)., Conclusions: IgH gene rearrangement detection can be improved by the combined usage of multiple primer pairs, especially family specific primers. In the future, detection of IgH gene rearrangement may be used as a novel marker to predict the prognosis of patients with primary orbital MALT lymphoma.

Purpose: Primary radiation therapy (RT) achieves excellent local control and overall survival when treating localized orbital lymphoma. However, evidence supporting irradiation of partial orbit volumes to spare nearby critical structures is lacking. We sought to investigate outcomes for patients with localized orbital lymphoma treated with partial orbit irradiation., Methods and Materials: We retrospectively reviewed patients with orbital lymphoma treated with RT at our institution who met our inclusion criteria: biopsy-confirmed, low-grade lymphoma, localized disease, partial orbit treatment volumes, and follow-up >3months. The Kaplan-Meier method was used to measure overall survival (OS), and the cumulative incidence function adjusted for the competing risk of death was used to measure local failure (LF), contralateral orbit recurrence (COR), and progression. Patient characteristics were compared with outcomes using Fisher exact test for dichotomous variables and Wilcoxon rank-sum test for continuous variables., Results: Thirty-two patients meeting inclusion criteria were identified with median follow-up of 45.8months (range, 3.6-171.9). The majority had stage IEA disease; their sites included conjunctiva (n=20) and retrobulbar or lacrimal gland (n=12). Median partial orbit RT dose was 30.6Gy (range, 22.5-36). Five-year OS was 100%. Five-year cumulative incidence of LF, COR, and overall disease progression was 5.3%, 5.9%, and 21.4%, respectively. Five-year cumulative incidence of LF was 8.3% for conjunctival disease versus 0.0% for retrobulbar or lacrimal gland involvement (P=.15). No significant association was observed between the outcomes of LF, COR, or progression and pretreatment characteristics. Acute and late toxicity included grade 2 periorbital edema (n=3, 9.4%), dry eye (n=3, 9.4%), retinal vascular disorder (n=1, 3.1%), conjunctivitis (n=2, 6.3%), and grade 3 cataract (n=1, 3.1%)., Conclusions: Use of partial orbit irradiation in treating low-grade, localized orbital lymphoma achieves excellent survival with low rates of LF, COR, or progression., (Copyright © 2016 American Society for Radiation Oncology. Published by Elsevier Inc. All rights reserved.)

Purpose: The aim of this work was to retrospectively analyze efficacy, toxicity, and relapse rates of conventional (CRT) and low-dose radiotherapy (LDRT) in patients with indolent orbital lymphomas., Patients and Methods: From 1987-2014, 45 patients (median age 64 years) with 52 lesions of indolent orbital lymphomas were treated with CRT (median dose 36 Gy, range 26-46 Gy) and 7 patients (median age 75 years) with 8 lesions were treated with LDRT (2 fractions of  2.0 Gy)., Results: Median follow-up was 133 months (range 2-329 months) in the CRT group and 25 months (range 10-41 months) in the LDRT group. Overall response rates were 97.7 % (CRT) and 100 % (LDRT). The 2‑ and 5‑year local progression-free survival (PFS) rates were 93.5 and 88.6 %, distant PFS 95.0 and 89.9 %, and overall survival 100 and 85.6 % after CRT. In the LDRT group, 2‑year local PFS and overall survival remained 100 %, respectively, and distant PFS 68.6 %. Acute radiotherapy-related complications (grades 1-2) were detected in virtually all eyes treated with CRT. Cataracts developed in only patients who were irradiated with more than 34 Gy. LDRT was well tolerated with only mild acute and late complications., Conclusion: Primary radiotherapy of indolent orbital lymphomas is an effective treatment with high response rates and excellent local control in CRT and LDRT. In combination with close follow-up, LDRT may be an attractive alternative since re-irradiation even with conventional doses is still feasible.

The purpose of this study was to evaluate the long-term outcome and patterns of failure in patients treated with primary radiotherapy (RT) for orbital lymphoma (OL). Seventy-nine patients diagnosed with stage IE OL between 1995 and 2012 were included. Fifty-nine patients (75%) had mucosa-associated lymphoid tissue lymphoma and 20 patients (25%) had follicular lymphoma subtype. The median follow-up was 49.7 months. Major tumor sites were conjunctiva (29%), orbit (47%) and lacrimal gland (24%). After treatment to a median dose of 30.6 Gy, there were a total of no local, one contralateral orbital, two regional and two distant recurrences, all outside of the treatment fields. The 10-year local relapse-free, distant metastasis-free and overall survival rates were 100%, 94.2% and 98.2%, respectively. Definitive RT to 30 Gy was shown to be highly effective for indolent OL, and this study represents one of the largest single-institution studies using primary RT for stage IE OL.

Purpose: To evaluate the prognostic utility of the American Joint Committee on Cancer (AJCC) staging system for ocular adnexal lymphoma (OAL).
, Methods: A multicenter, consecutive case series of patients with biopsy-proven conjunctival, orbit, eyelid, or lacrimal gland/sac lymphoma was performed. The electronic pathology and clinical records were reviewed for new or recurrent cases of ocular adnexal lymphoma. The main outcome measures included pathology and clinical staging (AJCC and Ann Arbor systems), treatment, and recurrence (local and systemic). Statistical analysis included demographic evaluations and the Kaplan-Meier survival probability method.
, Results: Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue were the most common (n=60/83, 72%). The most common Ann Arbor clinical stages were IE (76%) followed by IIE (17%) and IIIE (7%). Pathology identified 13 cases (15%) that were upstaged to group IV (p=0.017). Similarly, AJCC clinical stages were cT1NOMO (21.7%), cT2NOMO (44.6%), cT3N0M0 (5%), and cT4NOMO (2.4%). Local control was achieved in 75% of treated patients. There were 19 local recurrences from which 14 (74%) belonged to the non-radiation treatment groups. Lower-risk groups (T1 and T2 without lymph node involvement or metastatic disease of AJCC and IE of Ann Arbor) had longer disease-free survival than the higher-risk groups (AJCC T1, T2 with nodal involvement or metastatic disease, T3, and T4 as well as Ann Arbor II, III, and IV). The overall mean follow-up was 43.3 months (range 6-274).
, Conclusions: Regardless of stage, recurrence and disease-free survival were more closely related to treatment and histopathology rather than tumor size or site-specific location.

Background: Lymphomas of the orbit and orbital adnexae are rare tumors, comprising only 1% of all non-Hodgkin's lymphoma. The majority of non-Hodgkin's lymphomas of the orbit are extranodal marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue type. Because of nonspecific clinical signs and symptoms, some diagnostic delay may occur. The purpose of the study was to evaluate the diagnostic approach in orbital lymphomas and to analyze their treatment outcome., Methods: In the period from 2005 to 2012, from a group of 135 patients with tumors of the orbit, we identified 11 patients diagnosed with orbital lymphoma. This patient cohort was reviewed retrospectively., Results: The patient group consisted of 11 patients (seven females, male males) with a median age of 57.7 years (range 42 to 88 years). Orbital swelling, pain and motility impairment were the leading clinical symptoms. Diagnosis was confirmed by surgical biopsy. Depending on the anatomic location of the tumor, a surgical biopsy was taken using a blepharoplasty incision, a lateral orbitotomy or a navigation-guided biopsy. The predominant histology was extranodal non-Hodgkin's lymphoma of mucosa-associated lymphoid tissue type (82%). All patients underwent complete clinical staging. These were clinical stage IEA in seven patients, and stages IIEA (n = 2) and IIIEA (n = 2) in four patients . Patients in stage IEA were treated with radiation therapy alone, with radiation doses between 25 and 40 Gy, and patients with stage IIEA received systemic chemotherapy with bendamustin/rituximab. Those two patients diagnosed with diffuse large B-cell lymphoma and mantle cell lymphoma received systemic chemotherapy according to the R-CHOP protocol., Conclusions: Owing to unspecific clinical symptoms, some diagnostic delay may occur in orbital lymphoma. If unspecific orbital symptoms are present, adequate imaging studies followed by early surgical biopsy will contribute to early diagnosis. Once diagnosis is established and staging is complete, radiation therapy is the recommended treatment for stage IEA patients. Systemic chemotherapy is indicated in selected stage IIEA patients and in patients with stage IIIEA disease.

Objective: To evaluate the risk for systemic lymphoma (SL) in the patients with orbital lymphoproliferative tumor (OLT)., Design: Observational, retrospective case series., Participants: One hundred sixty consecutive cases with OLT., Methods: Clinical features and treatment method were collected retrospectively. Data from 106 patients without systemic disease at presentation were analyzed for their impact on the main outcome measure using univariate and multivariate regression models., Main Outcome Measure: Occurrence of SL diagnosed based on the 6 monthly systemic evaluation., Results: Of 106 patients with OLT alone, SL subsequently developed in 16% of patients and 84% patients remained free of SL. Of 17 patients in whom SL developed subsequently, 29% had marginal zone, B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT), 24% had small lymphocytic lymphoma (SLL), 24% had atypical lymphoid hyperplasia (ALH), 6% each had mantle cell, follicular, and diffuse large B-cell lymphoma (DLCL). In these 17 patients, systemic disease appeared after a mean interval of 152 months, and the involved systemic sites were abdominal lymph nodes (LN) in 44% patients, pelvic LN in 40%, and head and neck LN in 31%. Of 17 patients, 53% had the same SL classification with orbital tumor and 47% had a different SL classification. Among 8 patients with different systemic and orbital lymphoma classifications, systemic SLL developed in 4 patients with orbital ALH and in 2 patients with MALT. Two patients with orbital SLL manifested systemic DLCL. Using Kaplan-Meier estimates of 106 OLT patients without systemic involvement, SL developed in 14% at 3 years, in 17% at 5 years, and in 33% at 10 years. Using Kaplan-Meier estimates of 24 patients with bilateral OLT alone, SL developed in 18% at 3 years, in 29% at 5 years, and in 72% at 10 years. In 82 patients with unilateral OLT alone, SL developed in 12% at 3, 5, and 10 years. Multivariate analysis showed that bilateral involvement at presentation was the only significant factor predictive of SL., Conclusions: In patients with OLT alone at presentation, SL eventually developed in 33% by 10 years in this retrospective case series. Classification of SL can be the same or different from OLT. Development of SL is significantly associated with bilateral involvement.

Purpose: To evaluate the clinical features, treatment, and outcomes of a cohort of patients with ocular adnexal lymphoproliferative disease classified according to the World Health Organization modification of the Revised European-American Classification of Lymphoid neoplasms and to perform a robust statistical analysis of these data., Methods: Sixty-nine cases of ocular adnexal lymphoproliferative disease, seen in a tertiary referral center from 1992 to 2003, were included in the study. Lesions were classified by using the World Health Organization modification of the Revised European-American Classification of Lymphoid neoplasms classification. Outcome variables included disease-specific survival, relapse-free survival, local control, and distant control., Results: Stage IV disease at presentation, aggressive lymphoma histology, the presence of prior or concurrent systemic lymphoma at presentation, and bilateral adnexal disease were significant predictors for reduced disease-specific survival, local control, and distant control. Multivariate analysis found that aggressive histology and bilateral adnexal disease had significantly reduced disease-specific survival., Conclusions: The typical presentation of adnexal lymphoproliferative disease is with a painless mass, swelling, or proptosis; however, pain and inflammation occurred in 20% and 30% of patients, respectively. Stage at presentation, tumor histology, primary or secondary status, and whether the process was unilateral or bilateral were significant variables for disease outcome. In this study, distant spread of lymphoma was lower in patients who received greater than 20 Gy of orbital radiotherapy.

Purpose: Although systemic or eyelid involvement by ocular adnexal lymphoma carries a worse prognosis, there have been few reports of the outcome in relation to clinical presentation. The outcome of malignant ocular adnexal lymphoma was, therefore, related to presenting clinical symptoms and signs., Design and Methods: A retrospective, noncomparative case-note review of 326 patients treated in the Orbital Clinic at Moorfields Eye Hospital. The associations between presenting symptoms or signs and three outcome measures (v.i.) were assessed by univariate and multiple variable regression together with Kaplan-Meier analysis., Main Outcome Measures: (i) Presence of extra-orbital disease at the time of presentation; (ii) development of systemic lymphoma after new presentation with solely ocular adnexal disease; and (iii) death attributable to widespread lymphoma., Results: Presentation with disseminated disease was rarer with over 1 year's ophthalmic symptoms (odds ratio (OR) 0.7; 95% CI 0.5-0.9) and much more frequent with bilateral adnexal disease (OR 5.8; 95% CI 3.0-11.2). With solely adnexal disease at presentation, subsequent extra-orbital lymphoma was more frequent and earlier with lacrimal gland disease (as compared to those without; hazard ratio (HR) 1.9; 95% CI 1.2-4.5) or with eyelid disease (compared to those without; HR 2.4; 95% CI 1.2-4.5), or with bilateral disease (compared to unilateral disease; HR 2.6; 95% CI 1.4-5.2). Prior or concurrent systemic disease was the most significant predictive factor for lymphoma-related death (HR 6.8; 95% CI 4.3-10.9), but tumour-related death was also commoner and earlier with bilateral disease (HR 2.4; 95% CI 1.4-4.0) or where a relative afferent papillary defect was present (HR 2.8; 95% CI 1.6-4.9). Similarly, the rate of tumour-related death was slightly less where symptoms had been present for more than a year (HR 0.8; 95% CI 0.7-1.0) and slightly greater in the elderly (HR 1.03; 95% CI 1.01-1.05). Conjunctival lymphoma had the lowest rate of extra-orbital spread and lymphoma-related death, the rate of these two events being sequentially greater for patients with predominantly deep orbital lymphoma, lacrimal gland lymphoma, or eyelid lymphoma., Conclusion: These data suggest that presenting symptoms and signs of patients with ocular adnexal lymphoma are significantly associated with the risk of systemic disease at orbital presentation, the rate of subsequent spread, and the rate of lymphoma-related death.

Purpose: To analyze the results of radiotherapy (RT) for malignant lymphoma of the orbit and to evaluate them compared with the World Health Organization (WHO) classification published in 2001., Methods and Materials: The data from 29 patients with malignant lymphoma of the orbit treated with RT at Gunma University Hospital between 1978 and 2001 were retrospectively analyzed. Pathologic slides from 23 cases were available and were reviewed by a hematopathologist according to the WHO classification. The original and reviewed diagnoses, patient characteristics, treatment results, and complications were analyzed. In principle, patients with low-grade or indolent lymphoma were treated with RT alone, using 30 Gy as the tumor dose. Survival data were calculated using the Kaplan-Meier method., Results: One case that proved to be a pseudotumor was excluded from evaluation. Of the 28 cases, 25 were Stage IAE, 1 was Stage IIAE, and 2 were Stage IVAE. The median follow-up was 71 months. According to the original classification and the Working Formulation, the 5- and 10-year overall survival rate of patients with low-grade lymphoma was 94% and 73%, respectively. The corresponding rates for those with intermediate-grade lymphoma were 67% and 67% (p = 0.15). In contrast, the WHO classification showed a significant difference in the survival curves. The 5- and 10-year overall survival rate of patients with mucosa-associated lymphoid tissue (MALT) lymphoma was 100% and 88%, respectively; for diffuse large B-cell patients, the rates were both 0% (p < 0.001). In patients with MALT lymphoma, one local and four distant relapses developed; two of them >10 years after initial treatment. All of the relapsed MALT lymphomas were controlled by salvage therapy., Conclusion: Excellent local control and survival can be achieved for patients with orbital MALT lymphoma using RT alone. A precise histopathologic diagnosis using the WHO classification and long-term follow-up for >10 years is recommended.

Background: This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. The lesions were categorized using the Revised European American Lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patient's age and sex., Methods: The clinical, histopathological, immunohistochemical and phenotypic analysis by flow cytometry and follow-up data were studied., Results: The ocular adnexal lymphoproliferative lesions included 70 lymphomas and six reactive lymphoid hyperplasia. Seventy-nine per cent had stage IE disease, 4% stage II, 1.5% stage III and 15.5% stage IV. Five patients (7%) had a past history of systemic lymphoma. Major histological types were extranodal marginal zone lymphoma (MZL) in 44 (63%), follicular (FL) in 12 (17%), diffuse large B-cell (DLBCL) in eight (11%), mantle cell (MCL) in two (3%), B-cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma in two (3%), peripheral T-cell lymphoma (PTCL) one (1.5%) and natural killer cell lymphoma (NKCL) in one (1.5%). Longest survival was seen in those with low-grade lymphomas (MZL and FL) and worst in PTCL and NKCL. Lymphoma-related mortality was 2% for MZL, 33% for FL, 38% for DLBCL, and 100% for MCL, PTCL and NKCL. Systemic lymphoma was present prior to, at presentation or at subsequent follow up in 26/68 (39%) of all lymphoma patients, 17% for MZL, 38% for DLBCL, 83% for FL, and 100% for MCL, CLL, PTCL and NKCL., Conclusion: The majority of ocular adnexal lymphomas were low-grade B-cell lymphomas (MZL). Multivariate analysis showed that the only significant independent predictors of all causes of mortality were the histological type of lymphoma and the stage of disease at presentation.

Purpose: With the recent increase in the incidence of orbital malignant lymphoma, it is important to make the appropriate decision on effective treatment. The purpose of the current study was to develop a protocol to treat orbital malignant lymphoma based on evidence from histopathological examination., Methods: In the present study we analyzed the records of 18 cases of orbital lymphoma treated in the Yamagata University Hospital over the past 14 years, including 6 cases of isolated orbital disease and 12 cases of systemic disease. The tentative strategy for selecting the treatment was as follows: the cases at clinical stage I (Ann Arbor criteria) were treated with curative intent, and the cases at stages II-IV were treated with palliative intent. This strategy was evaluated from the viewpoints of efficacy and side effects., Results: Histopathologically, all 6 cases of isolated orbital disease were classified as low-grade malignancy, 3 of the systemic disease cases were classified as low-grade, and 9 were classified as intermediate-grade malignancy. The isolated orbital disease cases at stage I were treated by radiation or chemotherapy. The systemic disease cases at stages II-IV were treated by chemotherapy and/or radiation. The lymphoma disappeared completely in all 6 isolated orbital disease cases. Although the survival rate was 42% in systemic disease cases after a mean period of 15-month observation, the ocular complications decreased with an improved quality of life., Conclusions: It is important to determine the histological features and clinical stages in order to choose the appropriate treatment methods. All the isolated orbital disease cases were treated effectively by radiation or chemotherapy. Our strategy for the treatment of the systemic disease cases contributed to the decrease in ocular complications and the improvement in the quality of life for the patients.

A study was undertaken to assess the natural history of periocular lymphoproliferative diseases, identify key prognostic factors, and clarify the role of orbital irradiation. Thirty-four patients with periocular lymphoproliferative disease were treated with orbital irradiation between 1975 and 1990. Eight patients had atypical lymphoid infiltrate, and 26 had malignant lymphoma. Forty-three eyes were irradiated with en-face electrons or 6-MV photons. Five-year disease-free survival for all stages was 65%; it was not significantly affected by bilaterality or site. Stage, distinction between atypical lymphoid infiltrate and malignant lymphoma, and working formulation grade were important prognostic indicators. A complete response during irradiation was achieved in 24 of 43 (56%) eyes at a median dose of 2,400 cGy, and a partial response was achieved in 19 (44%), with resolution at a median of 2.8 months. Patients with periocular reactive lymphoid hyperplasia or atypical lymphoid infiltrate may have or are at significant risk of developing lymphoma and dissemination. Local treatment remains important; orbital irradiation achieves prompt local control with acceptable morbidity.

The clinical characteristics and microscopic features of the biopsy specimens of 66 patients with orbital lymphoid tumors were analyzed with respect to clinical outcome (disease remaining localized to the orbit or developing associated extraorbital lesions). The patients' average age was 63 years, and their tumors had a marked tendency to arise in the anterosuperior orbit. No clinical or radiographic findings helped to distinguish the cases of localized disease from disseminated disease. The lesions were divided microscopically into three categories: reactive lymphoid hyperplasia (16), atypical lymphoid hyperplasia (38), and malignant lymphoma (12). Extraorbital lesions developed in 25%, 50%, and 75% of cases, respectively. The five-year mortality rates were also significantly different for each type of lesion: 6%, 19%, and 58%, respectively.

115 patients with lymphoid tumours presenting in the orbit were seen between 1970 and 1984. The histological types were high-grade malignant lymphoma--18, low-grade malignant lymphoma--43, and indeterminate lymphocytic lesions--54. Eighteen patients were found to have disseminated lymphoma at presentation. The majority of the patients received radiotherapy to the orbit; local control was achieved in all cases and the ocular morbidity from radiotherapy was low with 11 patients developing lens opacities and 5 a dry eye. Survival of patients with stage I low-grade lymphoma and indeterminate lymphocytic lesions was similar to that of a normal population of the same age distribution. The clinic features and dissemination pattern of the low-grade malignant lymphomata and the indeterminate lymphocytic lesions were identical, suggesting that most, if not all, lymphoid masses presenting in the orbit are neoplastic rather than reactive in nature.

Lymphoreticular tumors in the orbit are uncommon. Only 42 patients were identified from over 2000 patients with tumors of lymphoid tissue seen between 1958 and 1979. The patients were divided into 3 groups: primary malignant, secondary malignant, and benign lymphoma. In the primary malignant lymphoma groups, there were 24 patients with a median age of 64 (40 to 87) years and a 2.4:1 female to male ratio: 2 cases were bilateral. In 19 (79%) patients with the lymphoma limited to one orbit the tumor was controlled in every case with doses from 25 to 45 Gy. There were no serious complications. Subsequently lymphoma developed at other sites in 7 (37%) patients. The 5 and 10 year cause specific actuarial survival rates were 70 and 62%. In the 8 patients with secondary malignant lymphoma, the orbital disease was controlled by irradiation, although all patients required further treatment and died of their disease. Radiotherapy controlled all 7 patients with benign lymphoma without any complications.

The incidence, survival rate and causes of death of patients with orbital lymphomas and other lymphoid lesions of the orbit in Denmark, during the period 1943-62 inclusive, were studied by means of a clinico-pathological analysis. In the period examined, the incidence of histologically verified primary orbital tumours was 0.06%. The incidence of primary orbital lymphomas and lymphoid lesions was at least 0.012%. These can be histologically divided into 3 categories: benign, indeterminate (intermediary) and malignant. The present study confirms the relevance of this classification, as there is a definite relationship between the histological diagnosis and the clinical survival rate. For the patients in question, the chances of dying of a universal malignant disease were approximately 10(3) times greater than in the normal Danish population.

Eighty-eight patients with ocular adnexal lymphoid lesions examined at the Mayo Clinic between 1970 and 1982 were evaluated. The histopathologic type was reclassified using the working formulation. Paraffin sections were evaluated with immunoperoxidase staining. There were 11 "pseudotumors" and 77 lymphomas. Sixty lymphomas were small lymphocytic type A; 27 tissues had a monoclonal staining pattern, and 4 had a polyclonal staining pattern. Five of the pseudotumors had a polyclonal staining pattern; none had a monoclonal pattern. The remainder did not stain or tissue was not available. Thirty-six of the 44 lymphomas that presented in the ocular adnexa remained localized. There were no distinguishing features between conjunctival and orbital lymphomas. Significant damage to the ocular tissue occurred in 15 of the 63 patients treated with radiation therapy. Older, asymptomatic patients with a favorable histologic type may not require treatment; therapy is reserved for specific indications. The probability of survival to five or seven years differs little from that expected in the general population.