Ocular adnexal lymphoma - a retrospective study and review of the literature.

BACKGROUND: To review the characteristics of all Slovenian patients with ocular adnexal lymphoma (OAL) in the period of 24 years with the aim of evaluating demographic data, lymphoma location and type, disease stage, treatment modality, local control rate and survival rate. PATIENTS AND METHODS: All patients with histologically diagnosed OAL in the main tertiary centre of Slovenia, Eye Hospital, University Medical Centre Ljubljana, who were treated at Institute of Oncology Ljubljana were included in the study. Patients' data were collected from October 1995 through April 2019. RESULTS: Seventy-four patients were included in the study having a median age of 68 years at diagnosis. The majority of lymphomas were of B-cell origin (98.6%). The most frequent type was the extranodal marginal zone B-cell lymphoma (MALT) (71.6%). Orbital lymphomas were diagnosed in 56 cases (75.7%) and conjunctival in 18 cases (24.3%). Ocular manifestation was the first sign of the disease in 78.4% of patients and in 67.6% of patients ocular adnexa were the only disease location. Fifty-one patients (68.9%) were treated with radiotherapy, 7 patients (9.4%) with systemic treatment, 5 patients (6.8%) with combined radiotherapy and systemic treatment and in 11 patients, biopsy and active surveillance strategy was applied (14.9%). Local control of the disease was achieved in 96.6% of treated patients. Median overall survival of the whole study group has not been reached yet. Five-year overall survival rate was 80.1% (95% CI 68.1% - 88.5%) and 5-year lymphoma specific survival rate was 87.2% (95% CI 83.2%-91.2%). CONCLUSIONS: OALs comprise a group of heterogeneous diseases with variable outcomes depending predominately on the patient's age and lymphoma type, with low grade lymphomas carrying good prognosis even in elderly patients.

Response-Adapted Ultralow-Dose Radiation Therapy for Orbital Indolent B-Cell Lymphoma: A Phase 2 Nonrandomized Controlled Trial.

Importance: Radiation therapy to doses of 24 to 36 Gy is currently used to treat indolent B-cell lymphoma of the ocular adnexa; however, ocular adverse effects are common. Objective: To determine if a response-adapted radiation therapy strategy will result in excellent disease outcomes while reducing orbital morbidity. Design, Setting, and Participants: This single-institution, phase 2 prospective nonrandomized controlled trial of a response-adapted strategy involved 50 evaluable patients with stage I to IV indolent B-cell lymphoma of the ocular adnexa enrolled between July 2015 and January 2021. This treatment approach was also retrospectively evaluated with a separate 55-patient cohort treated between March 2013 and October 2021. All data were analyzed between November 2021 and December 2023. Interventions: Patients were treated with ultralow-dose radiation therapy to 4 Gy in 2 fractions and assessed for response at 3-month intervals. Patients with persistent orbital lymphoma were offered an additional 20 Gy in 10 fractions to complete the response-adapted treatment. Main Outcome and Measures: The primary end point was 2-year local orbital control within the irradiated field after response-adapted therapy. Secondary end points included overall survival and complete response rate. Results: The 50 prospective patients were a median (range) of 63 (29-88) years old, and 31 (62%) were female. Among the 50 patients, 32 (64%) had mucosa-associated lymphoid tissue lymphoma, 12 (24%) had follicular lymphoma, and 6 (12%) had unclassifiable low-grade B-cell lymphoma. Thirty-one patients (62%) had stage I disease, and 36 (72%) were newly diagnosed. At a median follow-up of 37.4 (95% CI, 33.7-52.5) months, the 2-year local control rate was 89.4% (95% CI, 81.0%-98.7%), and the 2-year overall survival rate was 98.0% (95% CI, 94.1%-100%); 45 patients (90.0%; 95% CI, 78.2%-96.7%) experienced a complete response to response-adapted radiation, including 44 patients with a complete response to ultralow-dose radiation and 1 patient with a complete response after an additional 20 Gy. No local recurrences were observed among patients with a complete response to response-adapted therapy. No grade 3 or higher toxic effects were observed. In a planned subset analysis of 22 patients with newly diagnosed, untreated stage I mucosa-associated lymphoid tissue lymphoma, the 2-year local control rate was 90.7% (95% CI, 79.2%-100%), and the 2-year freedom from distant relapse rate was 95.2% (95% CI, 86.6%-100%). Conclusion and Relevance: In this nonrandomized controlled trial, response-adapted ultralow-dose therapy for indolent orbital B-cell lymphoma resulted in reduced radiation exposure, negligible toxic effects, and excellent disease outcomes. Trial Registration: ClinicalTrials.gov Identifier: NCT02494700.

Primary Orbital Melanoma: An Investigation of a Rare Malignancy Using the National Cancer Database.

OBJECTIVES: Primary orbital melanoma (POM) is a rare disease with limited data on survival and best treatment practices. Here we utilize the National Cancer Database (NCDB) to determine the overall survival (OS) and covariates that influence mortality. STUDY DESIGN: Retrospective cohort study. METHODS: All patients diagnosed with POM from 2004 to 2016 were identified in the NCDB. Patient and oncologic data were analyzed using the Kaplan-Meier method and multivariate models for the primary outcome of OS. RESULTS: A total of 129 patients were identified. Median OS was 36.9 months (95% confidence interval [CI] 24.1-78.7 months) with mean 5-year survival of 42.0% (CI 33.2%-53.2%). Treatments received included surgery alone (43.4%), radiation alone (23.3%), and surgery followed by radiation (20.2%). The multivariate model demonstrated an increased risk of death associated with age over 80 years (hazard ratio [HR] 3.41, CI 1.31-8.86, P = .012), a Charlson-Deyo comorbidity score of 2 or greater (HR 5.30, CI 1.87-15.03, P = .002), and no treatment (HR 2.28, CI 1.03-5.06, P = .042). For every 1 cm increase in tumor size, there was an increased risk of death (HR 1.06, CI 1.00-1.13, P = .039). When compared to surgery alone, no other treatment modality had an effect on OS. CONCLUSIONS: This study leveraged multiyear data from the NCDB to provide prognostic and demographic information on the largest known cohort of POM cases. Increased age, increased comorbidities, not receiving treatment, and larger tumor size were associated with increased mortality. There was no clear survival advantage for specific treatments. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:1790-1797, 2021.

Prognostic factors for relapse and survival among patients with ocular adnexal lymphoma: validation of the eighth edition of the American Joint Committee on Cancer (AJCC) TNM classification.

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.

Orbital metastases from breast cancer: A retrospective analysis of 28 cases.

Breast cancer account about half of the cases of orbital malignancies due to metastasis. The prognosis is adverse, with average survival from 2 to 34 months. OBJECTIVE: To analyze the clinical and pathological characteristics and outcomes of patients with orbital metastasis from breast cancer. MATERIAL AND METHODS: A retrospective, cross-sectional study of 28 patients seen in the Oncology Hospital of the National Medical Center XXI Century, from February 2003 to February 2019. RESULTS: Of the 28 patients, the median age was 50.8 years. The average time from the diagnosis of breast cancer to the development of orbital metastases was 64 months. 42.8% of the patients were stage IV of onset. The lobular vs ductal ratio was 5.2:1. 92.8% of the cases had luminal profile (one case with overexpression of HER-2), while only one case was triple negative. The most frequent symptoms were: decrease in visual acuity (64.2%), pain (57.1%), amaurosis (28.5%), alterations in mobility (28.5%) and edema (21.4%). Bilateral activity was presented in 25%. A third of the patients received local treatment (radiotherapy, surgery, anti-VEGFR). Overall survival was 67.5 months (1-184 months); however, the overall survival from the diagnosis of orbital metastasis was 26.4 months (1-98 months), with an average progression-free survival of 13.7 months (1-48 months). CONCLUSION: Ophthalmologic evaluation should be performed on all patients with breast cancer and ocular symptoms. Early local treatment should be offered in order to prevent severe complications and reduce the negative impact on quality of life.

45 GyRBE for group III orbital embryonal rhabdomyosarcoma.

Purpose: Despite widespread concerns of radiotherapy toxicity in children with head and neck tumors, recent Children's Oncology Group (COG) findings suggest that the use of 45 Gy results in an unacceptably high rate of local recurrences in patients with low-risk orbital rhabdomyosarcoma. We therefore evaluated outcomes in our pediatric patients who received 45 GyRBE using proton therapy. Material and methods: To assess disease control and toxicity, we reviewed the medical records of 30 children (≤21 years old) with COG stage 1, group III embryonal orbital rhabdomyosarcoma enrolled on a prospective outcome study and treated with proton therapy between 2007 and 2018. Results: Median age at the time of radiation was 4.8 years old. Twenty-one and nine patients received ifosfamide- and cyclophosphamide-based chemotherapy according to their respective cooperative group regimens. Median duration between the start of induction chemotherapy and radiation was 12 weeks. Two patients had a complete response to induction chemotherapy and two had stable disease. Twenty-six patients had a partial response to induction chemotherapy, with a median volume reduction of 66%. With a median follow-up of 4.0 years (range, 0.5-9.5 years), we observed 1 local failure 6 months following treatment in a patient who had a partial response to cyclosphophomide-based induction chemotherapy. The 5-year local control, progression-free survival, and overall survival rates were 97%, 97%, and 100%, respectively. Serious late toxicities included 18 patients with cataracts, 4 with exposure keratoconjunctivitis resulting in permanently reduced visual acuity, and 1 with chronic sinusitis. Conclusion: 45 GyRBE offers effective local control for most patients with group III orbital rhabdomyosarcoma. The delivery of proton therapy to the postinduction tumor volume plus a small margin can mitigate early- and intermediate-term toxicity, but side effects still occur and long-term data are needed to demonstrate the dosimetric advantage of proton therapy.

Outcomes of orbital malignancies treated with eye-sparing surgery and adjuvant particle radiotherapy: a retrospective study.

BACKGROUND: To report the clinical experience of eye sparing surgery (ESS) and adjuvant carbon-ion or proton radiotherapy (CIRT or PRT) for orbital malignancies. METHODS: An analysis of the retrospective data registry from the Shanghai Proton and Heavy Ion Center for patients with orbital tumors was conducted. The 2-year local progression-free, regional recurrence-free, distant metastasis-free, progression-free, and overall survival (LPFS, RRFS, DMFS, PFS, OS) rates as well as associated prognostic indicators were analyzed. Radiotherapy-induced acute and late toxicities were summarized. RESULTS: Between 7/2014 to 5/2018, 22 patients with orbital malignancies of various pathologies received ESS followed by CIRT (18), PRT (1), or PRT + CIRT boost (3). With a median follow-up of 20.25 (range 3.8-38.8) months, the 2-year OS, PFS, LPFS, RRFS, and DMFS rates were 100, 57.9, 92.9, 93.3, and 72.8%, respectively. No acute severe (i.e., ≥grade 3) toxicity was observed. Two patients experienced severe visual impairment as late toxicities. CONCLUSION: With few observed acute and late toxicities, particle radiotherapy following ESS provided effective local control with infrequent severe toxicities for patients with orbital malignancies.

Minimally disseminated disease and outcome in overt orbital retinoblastoma.

In this retrospective study of patients with overt orbital retinoblastoma, we evaluated minimally disseminated disease (MDD) in bone marrow and cerebrospinal fluid (CSF) using CRX and/or GD2 synthase as markers. Ten patients were evaluated-five (50%) at diagnosis and five upon relapse. MDD was detected in four cases (one in the bone marrow, two in the CSF, and in one case in both sites). All patients received chemotherapy and four received orbital radiotherapy. Seven patients relapsed or progressed and all of them died. Three patients remain in complete remission. There was no apparent correlation between MDD and the outcome.

Clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma involving the ocular adnexa.

AIMS: To evaluate the clinical characteristics and treatment outcomes of natural killer/T-cell lymphoma (NKTL) involving the ocular adnexa. METHODS: Retrospective, comparative, observational case series. A total of 350 patients with NKTL, including 27 patients with NKTL involving the ocular adnexa from 1999 to 2016. The patients were grouped into two groups: group 1 comprised patients presenting with ophthalmic symptoms, and group 2 comprised patients presenting with symptoms from other organs but subsequently developed ophthalmic involvement. RESULTS: Group 1 comprised 12 patients (44.4%) and group 2 comprised 15 (55.6%). Mean duration of symptoms in group 1 was 1.8±1.2 months, while the time from diagnosis of NKTL to development of ophthalmic symptoms in group 2 was 45.3±65.6 months. Periorbital swelling was the most common presenting symptom in both groups (83.3% in group 1 and 73.3% in group 2). Symptoms mimicking cellulitis and pseudotumor were present in 50.0% and 16.7% of cases, respectively. The 5-year overall survival rate was 18.5% in group 1 and 26.4% in group 2, while the 5-year progression-free survival rate was 0% and 13.3%, respectively. CONCLUSIONS: Our series is to our knowledge the largest cohort study on NKTL reported to date and demonstrates that ocular adnexal NKTL is a rare but seriously fatal disease. It is characterised by acute inflammatory signs as present in as many as two-thirds of our patients in this series. It should be considered as a differential diagnosis in patients presenting with rapidly progressing proptosis and diagnosed promptly for optimal management.

Factors related to survival outcomes following orbital exenteration: a retrospective, comparative, case series.

BACKGROUND: Orbital exenteration is a disfiguring procedure that aims to achieve local control. It is commonly a part of the management of malignant orbital tumor which is a life-threatening condition. It is necessary to determine predictive factors associated with overall survival (OS) following orbital exenteration. METHODS: This was a retrospective, comparative, case series of 39 patients with malignant tumors who underwent orbital exenteration. Patient records were reviewed for age, clinical presentation, preoperative visual acuity (VA), tumor size, surgical margin, tumor invasiveness, recurrent disease, and status of distant metastasis. Kaplan-Meier curves were used to assess OS and event-free survival (EFS). The predictive factors related to OS were identified using multivariate analysis. RESULTS: The mean age was 62.9 years (range, 5.5 to 89.7 years), 68.4% presented with VA < 20/400. The mean size of all tumors was 32 ± 18 mm. Distant metastasis at diagnosis was reported in 11 patients (28.2%). Twenty-two patients died during follow-up. The median OS and EFS were 3.89 years and 3.01 years, respectively. The predictive factors for worse OS on multivariate analysis were preoperative VA < 20/400 (adjusted hazard ratio [aHR] 4.67, P = 0.003), tumor size larger than 20 mm (aHR 3.14, P = 0.022,) and positive distant metastasis at diagnosis (aHR 15.31, P <  0.001). CONCLUSIONS: The prognostic factors for poor survival outcome following orbital exenteration were a preoperative VA < 20/400, tumor size > 20 mm, and distant metastasis at diagnosis mostly due to patient negligence.

Orbital invasion routes of non-melanoma skin cancers and survival outcomes.

PURPOSE/OBJECTIVES: Overall non-melanoma head and neck skin cancer has a good prognosis; however, rarely patients have an aggressive variant which results in orbital invasion via perineural spread or direct extension. Despite these consequences, there are limited published studies defining this clinical entity. The main objectives of the current study are to describe orbital invasion patterns of non-melanoma head and neck skin cancers and their impact on survival. METHODS: Retrospective case series from a tertiary-care, academic institution performed between 2004 and 2014. Demographic and tumour characteristics are reported as well as patterns of orbital invasion, types of treatments received, and survival outcomes. RESULTS: There were 17 consecutive patients with non-melanoma skin cancer and orbital invasion who met inclusion criteria. Average age at orbital invasion diagnosis was 70.8 years old. 76% were male. Mean follow-up time was 28.5 months. Of these patients, 71% had squamous cell carcinoma and 29% had basal cell carcinoma. Brow (41%) was the most common primary sub-site followed by cheek (23%) and temple (12%). 76% of patients had a history of prior treatment. The lateral orbital wall (41%) was the most common site of invasion, followed by the medial orbital wall (29%) and antero-superior invasion (23%). Age, histology, and location of orbital invasion were associated with disease-specific and overall survival. CONCLUSION: Orbital invasion for non-melanoma head and neck skin cancers creates a treatment dilemma and the patterns of invasion are described. In addition, the location of orbital invasion is associated with survival outcomes.

Orbital exenteration for sinonasal malignancies: indications, rehabilitation and oncologic outcomes.

PURPOSE OF REVIEW: Review the recent literature regarding the management of orbital invasion in sinonasal malignant tumors. RECENT FINDINGS: There is a recent trend in preserving the orbit in cases of minimal invasion of periosteum and limited periorbit involvement, as well as in presence of good response to neoadjuvant chemotherapy, mainly in squamous cell carcinoma and neuroendocrine histologies. SUMMARY: The decision about orbital exenteration in cases of sinonasal malignancies is facilitated if the patient already has clear clinical signs of intraconal invasion such as visual loss, restriction of ocular mobility or infiltration of the eyeglobe. However, in borderline situations, confirmation of orbital involvement should be performed intraoperatively. In selected cases with minimal orbital invasion without functional compromise, orbit sparing surgery can be done with acceptable oncological outcomes.

Spheno-Orbital Meningiomas Surgery: Multicenter Management Study for Complex Extensive Tumors.

BACKGROUND: Spheno-orbital meningiomas (SOMs) are complex tumors involving the sphenoid wing, the orbit, and sometimes the cavernous sinus with bone hyperostosis and sheet-like dural involvement. Optimal removal, proptosis cure, and visual preservation remain a challenge. OBJECTIVE: To study the management of surgically treated SOMs. METHODS: The clinical records of 130 consecutive patients undergoing surgery for SOMs were retrospectively collected in a database during a 20-year period to analyze symptoms, surgical technique, clinical outcome, and follow-up. RESULTS: Among the 130 patients (mean age 51.2 ± 9.5 years), 91.5% were female. The most typical symptoms recorded were proptosis in 94.6%, visual impairment in 37.7%, and oculomotor paresis in 10.0%. Simpson grade I-II removal was achieved in 97 patients (74.6%). After 1 year, proptosis was improved in 60% of cases. A total of 44.9% of the patients with preoperative visual acuity impairment were improved. Periorbital excision was statistically linked to proptosis decreasing (P = 0.0001) and optic canal decompression was linked to visual stabilization (P = 0.03). Bone reconstruction prevented temporal muscle atrophy (P = 0.01) and unaesthetic results (P = 0.0001). Mean follow-up was 76.5 months (range 3-288 months), and the mean time for recurrence was 54.2 months. CONCLUSIONS: A single-stage optimal surgery with bone reconstruction appears to be the best first-line treatment, on a case-by-case analysis. Optic canal decompression and periorbital excision have to be performed in case of visual disturbance and proptosis. SOMs require a long-term follow-up because of a delayed high rate of recurrence.

Orbital invasive squamous cell carcinoma with adnexal involvement clinically mimicking feline restrictive orbital myofibroblastic sarcoma: 19 cases (1990-2016).

OBJECTIVE: To describe the clinical presentations of patients diagnosed with ocular adnexal or orbital squamous cell carcinoma (SCC), which possess features similar to feline restrictive orbital myofibroblastic sarcoma (FROMS). PROCEDURES: A retrospective review of adnexal and/or orbital SCC was performed. Cases were collected from the University of Georgia College of Veterinary Medicine and the Comparative Ocular Pathology Laboratory of Wisconsin (COPLOW) (1990-2016). Data included signalment, ophthalmic clinical signs, nonophthalmic history and clinical signs, clinician suspicion of FROMS, advanced imaging results, and subsequent histopathologic diagnosis. FROMS cases from the COPLOW over the same time span were reviewed and compared statistically to the SCC cases with a significance threshold of 0.05. RESULTS: Nineteen cases (20 eyes) were identified with adnexal SCC with features similar to FROMS, including keratitis and eyelid/third eyelid restriction and/or thickening. There were no statistically significant differences between clinical findings in the SCC cases and the identified and compared FROMS cases (57 cases; 67 eyes), except for exophthalmos and/or resistance to retropulsion, which was less common in SCC cases (20%) than in FROMS cases (47.8%) (P = 0.027); and clinical or imaged presence of an overt eyelid or orbital mass, which was more common in the SCC cases (30%) than in the FROMS cases (4.5%) (P = 0.0010). CONCLUSIONS: SCC with adnexal involvement has many features similar to FROMS. In addition to FROMS, SCC should be considered a differential diagnosis in cats with restrictive adnexal or orbital signs and corneal changes.

Radiotherapy in the Management of Orbital Lymphoma: A Single Institution's Experience Over 4 Decades.

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.

Clinicopathological Features of Ocular Adnexal Mantle-Cell Lymphoma in an International Multicenter Cohort.

Importance: To our knowledge, the clinical features of ocular adnexal mantle-cell lymphoma (OA-MCL) have not previously been evaluated in a large multicenter cohort. Objective: To characterize the clinical features of OA-MCL. Design, Setting, and Participants: This retrospective multicenter study included patient data collected from January 1, 1980, through December 31, 2015, at 6 eye cancer centers in 4 countries. Medical records of 55 patients with OA-MCL were reviewed; the median length of follow-up was 33 months. Main Outcomes and Measures: Overall survival, disease-specific survival, and progression-free survival were the primary end points. Results: Fifty-five patients were included; ocular adnexal MCL was found to be most common in older individuals (mean age, 70 years) and men (n = 42 of 55; 76%). Patients with OA-MCL frequently presented with disseminated lymphoma (n = 34 of 55; 62%), and were likely to experience stage IVE disease (n = 35 of 55; 64%), with bilateral involvement (n = 27 of 55; 47%), tumor masses (n = 27 of 36; 75%), and involvement of the orbit (n = 32 of 55; 58%). Chemotherapy with or without external beam radiation therapy was the most frequently used treatment. Overall survival rates for the entire cohort were 65% at 3 years (95% CI, 52%-78%) and 34% at 5 years (95% CI, 21%-47%). Disease-specific survival after 5 years was 38% for the entire cohort (95% CI, 25%-51%); the disease-specific survival adjusted by eye cancer center was better in patients who had received rituximab in addition to the chemotherapy regimen (hazard ratio, 3.3; 95% CI, 1.0-14.7; P = .06). The median progression-free survival was 2.3 years (95% CI, 1.8-2.7 years) in patients who experienced recurrence after primary treatment, and 4.1 years (95% CI, 3.9-4.3 years) in patients who presented with a relapse of systemic lymphoma in the ocular adnexal region. Conclusions and Relevance: These results suggest that the distinctive features of OA-MCL are its appearance in older male individuals, advanced stage and bilateral manifestation at the time of diagnosis, and aggressive course. The prognosis of patients with OA-MCL might be improved by addition of rituximab to chemotherapy treatment.

A correlation study between IgH gene rearrangement and orbital lymphoma removal operation prognosis.

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.

45 Gy is not sufficient radiotherapy dose for Group III orbital embryonal rhabdomyosarcoma after less than complete response to 12 weeks of ARST0331 chemotherapy: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

BACKGROUND: Recent Children's Oncology Group (COG) trials tested the efficacy of reduced therapy in an effort to lessen late effects compared to the Intergroup Rhabdomyosarcoma Study (IRS) IV regimen with associated hematologic and hepatic toxicity, and infertility. Here, we analyze the efficacy of 45 Gray (Gy) local radiotherapy (RT) in patients with Group III orbital embryonal rhabdomyosarcoma (ERMS) enrolled on the COG low-risk study ARST0331. PROCEDURE: Sixty-two patients with Group III orbital ERMS were treated on ARST0331 with four cycles of vincristine (VCR), dactinomycin (DACT), and cyclophosphamide (CPM; VAC, total cumulative CPM dose 4.8 g/m2 ) followed by four cycles of VCR and DACT over 22 weeks. Forty-five Gray of radiation was administered in 25 fractions beginning at week 13 of therapy. RESULTS: Fifty-three patients were evaluable for this response analysis; seven had missing week 12 response evaluation data and two had progressive disease prior to starting RT. Median follow-up was 7.8 years. None of the 15 patients with radiographic complete response (CR) compared to 6 of the 38 patients with