Search

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., (© 2024 Lucka Boltezar et al., published by Sciendo.)

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., Competing Interests: Declaration of Competing Interests Rocio Grajales-Alvarez: I have no conflict of interest to declare. Alicia Gutierrez-Mata: I have no conflict of interest to declare., (Copyright © 2020 The Authors. Published by Elsevier Ltd.. All rights reserved.)

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.

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.

Retrospective analyses were done on 19 lacrimal adenoid cystic carcinoma (ACC) patients who underwent curative treatment between 1997 and 2013. Nine patients (47.4%) had T1-2 disease and ten (52.6%) had T4 disease. Surgical procedures were globe-preserving tumor resection in 11 patients (57.9%), incisional biopsy in five (26.3%), and orbital exenteration was undertaken in three (15.8%). Residual tumor burdens were R0/1 in 12 patients (63.2%) and R2 in seven (36.8%). Radiation therapy (RT) was recommended to all patients, and 16 (84.2%) completed RT (median 60 Gy). After median follow-up of 57.5 months, seven (36.8%) developed progression and three (15.8%) died. Local recurrence occurred in four patients (21.1%), distant metastasis in one (5.3%), and combined local recurrence and distant metastasis in two (10.5%). Progression-free survival and overall survival rates at 5-years were 64.5% and 82.6%, respectively. Among 12 patients following R0/1 resection, two (16.7%) developed local recurrence and none died, while among seven following R2 resection, five (71.4%) developed progression and three (42.9%) died. RT following R0/R1 resection could reduce progression. Globe-preserving surgery and RT seemed optimal strategy for T1-2 disease. Careful attention should to be paid to minimize residual tumor burden at surgery and effort for safe radiation dose escalation would be desired.

Objective: to analyze the epidemiology, clinical features and survival rate of patients undergoing orbital exenteration (OE) in a tertiary referral hospital., Methods: we conducted a retrospective study of all patients undergoing OE at the Hospital das Clínicas, FMUSP between January 2007 and December 2012. We collected data records related to gender, age, origin, length of stay, duration of the disease, other treatments related to the disease, number of procedures outside of the face related to the disease, follow-up and histological diagnosis., Results: we treated 37 patients in the study period. The average survival in one year was 70%, in two years, 66.1%, and 58.3% in three years. There was no significant difference in the one-year survival related to histological diagnosis (p=0.15), days of hospitalization (p=0.17), gender (p=0.43), origin (p=0.78), disease duration (p=0.27) or the number of operations for the tumor (p=0.31). Mortality was higher in elderly patients (p=0.02). The average years of life lost was 33.9 in patients under 60 years, 14.7 in patients in the 61-80 years range and 11.3 in patients over 80 years., Conclusion: the present series of cases is significant in terms of prevalence of orbital exenteration; on the other hand, it shows one of the lowest survival rates in the literature. This suggests an urgent need for improved health care conditions to prevent deforming, radical resections.

Ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) has been recognized as most common primary orbital malignancy. However, little was known about the response criteria for OAML. Our aim was to suggest response criteria for nonconjunctival OAML; the response evaluation of which using the conventional response criteria is inappropriate. A retrospective chart review of 34 eyes from 30 patients diagnosed with nonconjunctival OAML was conducted, focusing on the change in tumor size based on linear bi-dimensional and three-dimensional methods in magnetic resonance imaging (MRI) of the orbit. The maximum tumor response period of each case was investigated, and the expected optimal response period was calculated using regression analysis. In 30 evaluable patients, the median time taken for the maximum tumor response was 6 months (range, 3-18). More than 75 % of patients attained maximal tumor response in 6 months after initial therapy for follow-up period, the median value of which was 30 months (range, 15-77). Based on the regression analysis, it took 4.7 months for the maximum diameter (2r) of tumor to decrease by 50 % of initial lesion size. We cautiously suggest that optimal response could be defined as 50 % reduction of the maximum diameter in 6 months since the treatment was initiated, and that only observation without additional therapy is enough for nonconjunctival OAML, if optimal response is achieved.

Background: Orbital marginal zone B-cell lymphoma (OAML) constitutes for the most frequent diagnosis in orbital lymphoma. Relatively little data, however, have been reported in larger cohorts of patients staged in a uniform way and no therapy standard exists to date., Material and Methods: We have retrospectively analyzed 60 patients diagnosed and treated at our institution 1999-2012. Median age at diagnosis was 64 years (IQR 51-75) and follow-up time 43 months (IQR 16-92). All patients had undergone uniform extensive staging and histological diagnosis was made by a reference pathologist according to the WHO classification., Results: The majority of patients presented with stage IE (n = 40/60, 67%), three had IIE/IIIE and the remaining 17 stage IVE. Seven patients with IVE had bilateral orbital disease whereas the others showed involvement of further organs. Treatment data were available in 58 patients. Local treatment with radiotherapy (14/58, 24%) or surgery (3/58, 5%) resulted in response in 82% of patients. A total of 26 patients (45%) received systemic treatment with a response rate of 85%. Nine patients received antibiotics as initial therapy; response rate was 38%. Watchful-waiting was the initial approach in 6/58 patients. In total 28/58 patients (48%) progressed and were given further therapy. Median time-to-progression in this cohort was 20 months (IQR 9-39). There was no difference in time-to-progression after first-line therapy between the different therapy arms (p = 0.14). Elevated beta-2-microglobulin, plasmacytic differentiation, autoimmune disorder and site of lymphoma were not associated with a higher risk for progress., Conclusion: Our data underscore the excellent prognosis of OAML irrespective of initial therapy, as there was no significant difference in time-to-progression and response between local or systemic therapy. In the absence of randomized trials, the least toxic individual approach should be chosen for OAML.

Unlabelled: Diffuse large B-cell lymphoma (DLBCL) and mantle cell lymphoma (MCL) constitute two distinct subtypes of non-Hodgkin lymphoma (NHL) associated with considerable morbidity and mortality. Marked diversities with regard to molecular biology and clinical features are recognized in different subsets of the two lymphomas. Because these differences could be related to the location of the lymphoma, it is of interest to investigate the clinical and histopathological features of DLBCL and MCL involving the ocular adnexal region (i.e. the orbit, eyelids, conjunctiva, lacrimal gland and lacrimal sac). Similarly, the lacrimal gland is the only glandular structure within the orbit. Because the lacrimal gland represents an important part of the immunological system, it is of interest to investigate lymphomas involving this location with regard to clinical and histological characteristics., Purpose:   To characterize the clinical and histopathological features of Danish patients with DLBCL of the ocular adnexal region between 1980 and 2009 and of Danish ocular adnexal MCL patients from 1980 to 2005. Furthermore, the aim of this PhD was to review all specimens from patients with lymphoma of the lacrimal gland in Denmark between 1975 and 2009 to determine the distribution of lymphoma subtypes of the lacrimal gland and to describe the clinicopathological features of these patients., Results:   A total of 34 patients with DLBCL and 21 with MCL of the ocular adnexal region were identified. Twenty-seven patients had lacrimal gland lymphoma, including four DLBCLs and three MCLs from studies I and II. Elderly patients predominated in all three groups, with median ages of 78, 75 and 69 years in the DLBCL, the MCL and the lacrimal gland lymphoma groups, respectively. MCL patients had a preponderance of males, whereas females prevailed among lacrimal gland lymphoma patients. The orbit was the most common site of involvement in DLBCL and MCL. Most DLBCL patients had unilateral involvement, while MCL patients had a high frequency of bilateral involvement. Similarly, localized lymphoma was relatively frequently seen in DLBCL patients in contrast to the predominance of disseminated lymphoma in the MCL group. The majority of lacrimal gland lymphomas were low grade, and the distribution of subtypes was as follows: extranodal marginal zone lymphoma, 10 (37%); follicular lymphoma, 5 (19%); DLBCL, 4 (15%); MCL, 3 (11%); chronic lymphocytic leukaemia/small lymphatic lymphoma, 2 (7%); and unclassified B-cell lymphoma, 3 (11%). The overall survival rates at 3 and 5 years for the entire study group of DLBCL were 42% and 20%, whereas 58% and 22% of MCL patients were alive 3 and 5 years after the time of diagnosis. The 5-year overall survival rate of lacrimal gland lymphoma patients was 70%. Concordant bone marrow involvement and the International Prognostic Index score were predictive factors for the overall survival in the DLBCL group in Cox regression analysis. Rituximab-containing chemotherapy was associated with an improved survival rate in MCL patients., Conclusions:   Diffuse large B-cell lymphoma and MCL involving the ocular adnexal region and lymphoma of the lacrimal gland are prevalent among elderly patients. The overall prognosis in DLBCL and MCL was poor, whereas the prognosis for lacrimal gland lymphoma patients was relatively good. Concordant bone marrow involvement and the International Prognostic Index score were independent predictive factors for mortality in the DLBCL group. Chemotherapy containing rituximab significantly improved survival in the MCL group., (© 2013 Acta Ophthalmologica Scandinavica Foundation.)

Background: Ocular adnexal mucosal-associated lymphoid tissue lymphomas (MALTomas) are rare, and there are no phase III trials to guide treatment. Primary radiation therapy has been the typical management. This retrospective series reports the experience of a single institution and adds to the current literature., Methods: Our electronic medical record system and available paper charts were used to identify patients with MALTomas of the lacrimal gland or sac, conjunctiva, and orbital structures, including extraocular muscles. In order to determine pathology, staging, treatment information, local and distant control, salvage treatments, and late toxicity, records were reviewed., Results: Sixteen patients with ocular adnexal MALTomas had local radiation between 1992 and 2011 for primary or recurrent disease. Fifty percent of patients had lymphoma in the conjunctiva, 25% had lymphoma in the lacrimal sac/gland, and 25% of patients had lymphoma in the posterior orbit. Stage IAE disease occurred in 75% of patients, 6% had stage IIAE disease, and 19% of patients had a positive bone marrow biopsy. One patient received chemotherapy as part of initial therapy. The median radiation dose was 30 Gy (25.5-36 Gy) delivered with electrons (31%) or photons (69%). After a mean follow-up of 62.8 months, 2 patients had residual/progressive disease, 2 had contralateral recurrence, and 1 patient had a distant failure, for local control of 87.5% and overall disease control of 68.75%. Recurrence/progression occurred at a median of 35.45 months. Two patients with residual/progressive disease and 1 patient with a contralateral recurrence were followed, successfully salvaged, and have no evidence of disease. Fourteen patients are still alive, and there were no disease-related/toxicity deaths. Seven patients developed cataracts in the treated eye, 2 patients had radiation retinopathy, 2 had permanent dry eye syndrome, and 1 patient had severe keratopathy requiring enucleation. Six patients (3.75%) had worsening visual acuity of unclear etiology., Conclusions: Primary radiation therapy for ocular adnexal MALTomas with a median dose of 30 Gy led to excellent local control. Patients who did recur were successfully salvaged. Radiation was generally well tolerated, with expected cataractogenesis, given the dose required to achieve local control (with only 1 patient developing severe keratopathy after receiving the highest dose in this series).

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 explore clinical experience and propose new ideas for treating children diagnosed with orbital rhabdomyosarcoma (RMS)., Methods: We retrospectively analyzed the clinical data for30 patients (16 males and 14 females, with a median age of 6.2 years) with primary orbital RMS who were enrolled in the Department of Eye Oncology and Pediatrics of our hospital from November 2004 to December 2012. International Rhabdomyosarcoma Organization Staging Standards indicated that among the 30 patients, 4 cases were in phase II, 20 were in phase III, and 6 were in phase IV. All patients underwent a multidisciplinary collaborative model of comprehensive treatment (surgery, chemotherapy, external radiotherapy, 125I radioactive particle implantation, and autologous peripheral blood stem-cell transplantation)., Results: Follow-up was conducted until March 2013, with a median follow-up time of 47.2 months (5 to 95 months), and 7 deaths occurred. The 2-year estimated survival rate reached 86.1%, the ≥3-year estimated survival rate was 77%, and the 5-year estimated survival rate was 70.6%., Conclusions: The multidisciplinary collaborative model can be a safe and effective approach to the comprehensive treatment of children with orbital RMS. It has clinical significance in improving the tumor remission rate.

Background: Solitary fibrous tumor (SFT) is a rare spindle-cell benign neoplasm and located in orbit. The present research represents case reports of ten patients with orbital SFT., Method: Prognosis was assessed by clinic service and telephone interview. Clinical data were retrieved from the medical records. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, GFAP, S-100, MBP, CK, and MIB-1 was performed. Distributions of time to progression and recurrence between different operative methods were estimated using the Kaplan-Meier method and compared using the log-rank test., Findings: The ten patients included six men and four women (range: 19-75 years). The most common initial symptom was painless proptosis (n = 6). After the ten initial surgeries, with four requiring gross total resection (GRT) and six requiring subtotal resection (STR), an additional nine were required to remove recurrent tumors. Eighteen samples were obtained for pathological examination (the third sample for case 4 was not achieved). Cellular areas with partial hemangiopericytoma pattern were noted in four samples (4/18); scarce mitosis was noted in six samples (6/18). All SFTs (18/18) were positive for CD34, CD99 and vimentin, while all SFTs (18/18) were negative for EMA, CK, MBP, HBM-45 and GFAP. Bcl-2 was positive in 13/18 cases, while S-100 was negative in 14/18 cases. The MIB-1 labeling index varied from 1% to 3%. Follow-up was available for nine patients with a median of 88 months. All four patients who underwent GTR on initial operation did not recur; while the five patients who underwent STR recurred. The log-rank test showed that the incomplete surgical resection was significantly associated with recurrence (p = 0.015)., Conclusions: Orbital SFT is a rare mesenchymal tumor and painless proptosis is the most common initial symptom. Immunohistochemistry should be used to differentiate SFT from other tumors. Orbital SFT usually has a non-aggressive histological characteristic, and has a good prognosis if GTR is performed. Postoperative regular and long-term follow-up remains mandatory to monitor recurrence.

Aim: The value of neuronavigation in cranioorbital neurosurgery is controversial and relatively unstudied. The aim of this study was to evaluate the application, the usefulness and the reliability of neuronavigation in the neurosurgical treatment of orbital tumours., Material and Methods: A frameless armless infrared-based neuronavigation system was applied in the microsurgical removal of 7 orbital tumors. Image guidance was CT-based in 3 cases, MRI-based in another 3 cases and based on image fusion between CT and MRI image sets in one patient. The extradural fronto-orbital approach was performed in 3 cases, lateral orbitotomy in 2 cases, trans-supraciliar approach in 1 case and inferomedial orbitotomy in 1 case., Results: The surgical procedures were successful in all cases. The procedure-related morbidity and mortality rate in the series was zero. The registration accuracy of the neuronavigation ranged between 1.0 and 1.7 mm, with an average of 1.3 mm. Neuronavigated image guidance was evaluated as useful in all patients. Total tumour removal was achieved in 5 patients and partial tumour excision in 1 case. One patient was only biopsied., Conclusion: Neuronavigation is not a substitute for surgical knowledge and experience, but it is a valuable complement with significant intraoperative potential in cranioorbital surgery.

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

Background: Primary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkin's lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma., Methods: Forty-seven consecutive patients having primary orbital lymphoma treated in our department between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co gamma rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3 mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall survival (OS) and late side effects were evaluated in all patients., Results: With a median follow up of 45 months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and 55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal. Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12 (25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients., Conclusion: Radiotherapy is an effective and at the same time well tolerated treatment for primary orbital lymphoma.

Orbital exenteration is a disfiguring procedure which typically involves removal of the entire contents of the orbit including the periorbita, appendages, eyelids and, sometimes, a varying amount of surrounding skin. This operation is reserved for the treatment of potentially life-threatening malignancies arising from the orbit, paranasal sinuses or periocular skin. The marked increase in the average life span and resulting greater incidence of invasive malignant skin tumours of the face, typical of old age, is the reason for the increased rate of exenterations in elderly patients. The purpose of this report is to describe personal experience regarding 8 operations of orbital exenteration carried out on elderly patients, 6 males and 2 females, age range 66-85 years (mean 75), who came to our observation, from January 2002 to December 2007, on account of cancer (7 cases: 4 basal cell carcinomas; 1 squamous cell carcinoma; 1 fibrosarcoma; 1 melanoma) or infectious inflammatory disease (1 case of rhinocerebral mucormycosis) and were treated with type III orbital exenteration (2 cases) and type IV orbital exenteration (6 cases according to Meyer and Zaoli's classification). The methods used to reconstruct the eye-socket consisted of a full-thickness skin graft in 5 cases, pedicled myocutaneous flaps in 2 cases--a latissimus dorsi muscle flap alone, in one patient, and combined with a pectoralis major muscle flap in another - and a combined lateral-based frontal fasciocutaneous pedicled flap and full-thickness skin graft in the oldest patient. Regarding survival and the local clinical situation, 3 of the 4 patients with basal cell carcinomas are alive and disease-free after 6 years, 2 years and 20 months, respectively, while the oldest patient died of the disease after 10 months. The subject who underwent surgery for squamous cell carcinoma is alive and disease-free after 2 years. The patients with melanoma, fibrosarcoma and mucormycosis died. Although there are various options available for reconstruction, full-thickness skin graft or a pedicled muscolocutaneous flap provide the simplest solution in the elderly population with significant co-morbidities. The final outcome is, in our experience, comparable to that of more complex flap reconstruction, obtaining very good final results with minimal donor site morbility and a reduced operation time.

Purpose: To investigate the association between presence of orbital or ocular lesions and type and stage of leukemia and to investigate whether orbital and ocular lesions are significant in predicting leukemia prognosis., Methods: The authors evaluated 180 patients with acute childhood leukemia. Lesions associated with leukemia may be classified as specific (due to leukemic infiltration of various ocular tissues), nonspecific (due to one of the secondary complications), or iatrogenic manifestations caused by chemotherapy. Risk-based treatment assignment is based on clinical and laboratory features at diagnosis. Children with presenting white blood cell count below 50,000 mm3 are considered at standard risk for treatment failure, while all others are considered at high risk for treatment failure., Results: Specific lesions were noted in 66% of patients with acute myeloid leukemia (AML) and 11.5% patients with acute lymphocytic leukemia (ALL) (p<0.05), and were more severe in patients with high risk leukemia than in patients with standard risk leukemia. Orbital or ocular lesions were noted more commonly in patients with AML (66.6%) compared to patients with ALL (15.1%). In both the AML and ALL groups, there was a higher frequency of leukemic relapses in the bone marrow and/or central nervous system in patients with specific lesions (63.1%) compared to patients with nonspecific lesions (42%), and in patients without orbital or ocular lesions (29.2%) (p<0.05)., Conclusions: In both the AML and ALL groups, the presence of specific orbital or ocular lesions was associated with a higher frequency of bone marrow relapses and CNS involvement (p<0.05), leading to a lower survival rate.

Purpose: The aim of this study was to evaluate the efficacy and toxicity of moderate dose radiation therapy (RT) for mucosa-associated lymphoid tissue (MALT) lymphoma in a prospective multicenter phase II trial., Methods and Materials: The subjects in this study were 37 patients with MALT lymphoma between April 2002 and November 2004. There were 16 male and 21 female patients, ranging in age from 24 to 82 years, with a median of 56 years. The primary tumor originated in the orbit in 24 patients, in the thyroid and salivary gland in 4 patients each, and 5 in the others. The median tumor dose was 30.6 Gy (range, 30.6-39.6 Gy), depending on the primary site and maximal tumor diameter. The median follow-up was 37.3 months., Results: Complete remission (CR) or CR/unconfirmed was achieved in 34 patients (92%). The 3-year overall survival, progression-free survival, and local control probability were 100%, 91.9%, and 97.3%, respectively. Thirteen patients experienced Grade 1 acute toxicities including dermatitis, mucositis, and conjunctivitis. One patient developed Grade 2 taste loss. Regarding late toxicities, Grade 2 reactions including hypothyroidism, and radiation pneumonitis were observed in three patients, and Grade 3 cataract was seen in three patients., Conclusions: This prospective phase II study demonstrated that moderate dose RT was highly effective in achieving local control with acceptable morbidity in 37 patients with MALT lymphoma.

Background: Although primary ocular adnexal MALT (mucosa-associated lymphoid tissue) lymphoma (POAML) is a recently recognized unique entity, its natural history, prognostic factors, behavior of progression and death, and standard initial management have not been fully elucidated., Methods: The data of 114 patients with histologically verified POAML who were treated at our institution between 1970 and 2003 were retrospectively analyzed., Results: With a median follow-up duration of 5.7 years (0.6-34.0), estimated overall survival (OS) rate and progression-free survival (PFS) rate at 10 years was 89% and 57%, respectively. Thirteen (11%) patients died, but only three (3%) of them due to progressive lymphoma. Thirty-one (27%) patients progressed: eight who progressed at contra lateral sites were limited to those who had initially involved in the orbit (P = 0.036) and their OS and PFS were significantly longer (P = 0.035 and 0.039, respectively). Patients who initially received radiation-containing therapy were superior in PFS but not in OS to those initially treated with other modalities (P = 0.016 and 0.091, respectively). When we compared the outcomes of the observation cohort and the immediate therapy cohort, there were no significant differences in OS and PFS (P = 0.499 and 0.073, respectively)., Conclusions: The majority of patients with POAML showed the behaviors of localized and indolent diseases. Our preliminary observation that no initial therapy is an acceptable approach for selected patients was confirmed. Considering the possible heterogeneity of POAML among initial sites, further investigations are warranted.

Purpose: To elucidate the clinical behavior and treatment outcome of low-grade primary orbital lymphoma arising from mucosa-associated lymphoid tissue (MALT)., Methods and Materials: Forty-eight patients with pathologically confirmed marginal zone B-cell lymphoma of MALT were treated with radiotherapy (RT). Thirty-eight patients (79.1%) received thorough staging workup studies including bone marrow biopsy. Radiation doses ranged from 5.4 to 30.6 Gy (median, 30.6 Gy). Median follow-up period was 70 months., Results: Only 2 patients revealed extraorbital lymphoma involvement (bone marrow, skin). Forty-six of 52 eye lesions showed complete response to RT. Six lesions demonstrated a partial response and showed gradual regression during the follow-up period of 39-72 months. Three patients experienced local recurrences at 34, 48, and 52 months after RT, which seemed to be related to improper use of the lens shield. Salvage re-RT was successful. The 10-year actuarial relapse-free survival, cause-specific survival, and overall survival rates were 93.1%, 97.9%, and 86.9%, respectively., Conclusion: Most of the MALT lymphoma of the orbit was localized at diagnosis and extraorbital relapse rarely occurred. Therefore, extensive staging workup at the time of diagnosis and follow-up studies to detect distant relapse may not be obligatory. Low-dose RT alone with proper lens shielding is the optimum treatment modality for orbital MALT lymphoma.

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.

Purpose: To assess the outcome and prognostic factors in patients with orbital lymphoma treated by radiotherapy (RT)., Methods and Materials: Between 1980 and 1999, 90 consecutive patients with primary orbital lymphoma were treated in 13 member institutions of the Rare Cancer Network. A full staging workup was completed in 56 patients. Seventy-eight patients had low-, 6 intermediate-, and 6 high-grade lymphoma, and 75 had a single orbital localization. All patients underwent RT with a median dose of 34.2 Gy (range 4.0-50.4). Eleven patients received chemotherapy in addition to RT., Results: After RT, local control was achieved in 97% of the patients. Local progression occurred in 2% and local relapse 1%. The rate of systemic relapse was 20%, and 9% of the patients developed metachronous contralateral eye involvement. The 5-year disease-free survival, overall survival, and cause-specific survival rate was 65%, 78%, and 87%, respectively. In univariate analyses, the statistically significant favorable prognostic factors were younger age, low grade, normal erythrocyte sedimentation rate, absence of muscular infiltration, complete response to treatment, conjunctival localization, and normal lactate dehydrogenase value for overall survival, disease-free survival, and freedom from treatment failure. In multivariate analysis, the favorable factors were younger age and low grade for overall and disease-free survival; a favorable response, conjunctival localization, and complete staging were highly significant for disease-free survival and freedom from treatment failure. Neither the RT technique nor the total dose influenced the outcome. Cataract and xerophthalmia were the most prominent late toxicities., Conclusion: Moderate- to low-dose RT alone is able to control primary orbital lymphoma with low morbidity. A full staging workup is warranted in these patients. Prognostic factors were identified that could be useful in the overall management of this uncommon site of primary lymphoma.

Purpose: To review our institutional experience with primary orbital lymphoma and determine the prognostic factors for survival, local control, and distant metastases. In addition, we also analyzed the risk factors for complications in the radiotherapeutic management of this tumor., Methods and Materials: Between 1973 and 1998, 47 patients (29 women [62%] and 18 men [38%], median age 69 years, range 32-89) with Stage IAE orbital lymphoma were treated with curative intent at one department. Five had bilateral orbital involvement. The tumor was located in the eyelid and extraocular muscles in 23 (44%), conjunctiva in 17 (33%), and lacrimal apparatus in 12 (23%). The histologic features according to the World Heath Organization classification of lymphoid neoplasms was follicular lymphoma in 25, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type in 8, diffuse large B-cell lymphoma in 12, mantle cell lymphoma in 6, and peripheral T-cell lymphoma in 1. For the purposes of comparison with the existing literature on orbital lymphomas, the grading system according to the Working Formulation was also recorded. The histologic grade was low in 33 (63%), intermediate in 18 (35%), and high in 1 (2%). All patients were treated with primary radiotherapy alone. The median dose for low-grade tumors was 3000 cGy (range 2000-4020); the median dose for intermediate and high-grade tumors was 4000 cGy (range 3000-5100). A lens-sparing approach was used in 19 patients (37%). Late complications for the lens and cornea were scored according to the subjective, objective, management, and analytic (SOMA) scale of the Late Effects of Normal Tissue (LENT) scoring system. The median follow-up was 55 months (range 6-232)., Results: The local control rate was 100% in the 52 orbits treated. The 5-year overall survival and relapse-free survival rate was 73.6% and 65.5%, respectively. Tumor grade and location did not predict for overall survival or relapse-free survival. Seven patients (15%) developed distant recurrence (brain 2, extremity 2, mediastinum 1, liver 1, and retroperitoneum 1). One patient (2%) developed cervical node metastasis. The 5- and 10-year cataract-free survival rate was 56.7% and 32.9%, respectively. Of the 12 lens complications, 8 were LENT Grade 1 and 4 were Grade 3 toxicity. Only male gender predicted for an increased risk of cataract formation. Radiotherapy dose and technique did not predict for cataract formation; however, none of the patients who underwent the lens-sparing technique developed Grade 3 lens toxicity or required surgical correction. Of the nine corneal events, two were Grade 1, four Grade 2, and three were Grade 3 toxicity. Ten dry eyes were recorded; all were mild, and no patient had severe dry eye syndrome. Neovascular glaucoma was seen in 1 patient. No injury to the retina or optic nerve was reported., Conclusion: Radiotherapy alone is a highly effective modality in the curative management of primary orbital lymphoma. Most complications were minimal and did not require medical or surgical intervention. Although the use of the lens-sparing technique did not influence the incidence of cataractogenesis, we continue to recommend this approach whenever possible, because our experience indicates a higher grade of toxicity occurs and a higher incidence of corrective surgery is needed in patients treated without lens protection.

Purpose: To evaluate the treatment outcome in patients with mucosa-associated lymphoid tissue (MALT)/marginal zone (MZ) non-Hodgkin's lymphoma (NHL)., Materials and Methods: Between 1986 and 2000, 66 patients with clinical stage (CS) I-IV MALT/MZ NHL were treated; these comprise the study population. The progression-free survival (PFS) and overall survival (OS) were calculated using the Kaplan-Meier technique. Forty-five patients (68%) had CS I-II and 21 (32%) had CS III-IV disease. Twenty-nine of the 45 CS I-II patients received radiation therapy (RT) alone, and 6 patients had surgery and RT. The median RT dose was 3350 cGy. Among the 21 CS III-IV patients, treatment included chemotherapy alone (15), chemotherapy + RT (3), surgery (1), surgery + chemotherapy (1), and RT alone (1). Median follow-up was 48 months., Results: All 35 early-stage and all 4 advanced-stage patients who received RT as part of initial treatment achieved local control. Among the 63 evaluable patients, the 5-year OS and PFS were 90 and 57%, respectively. The 5-year OS was 93% and PFS was 75% among CS I-II patients; the corresponding estimates in CS III-IV patients were 83% and 14%, respectively., Conclusion: Modest doses of RT provide excellent local control in patients with MALT/MZ NHL. The poor PFS in advanced-stage patients suggests the need to develop alternative systemic treatment strategies for this disease.

Purpose: To review the findings, management, and outcome in 33 cases of primary ophthalmic rhabdomyosarcoma., Methods: The records of 33 consecutive patients from a single ocular oncology center were analyzed retrospectively for outcomes of final visual acuity, local recurrence, and distant metastasis., Results: Rhabdomyosarcoma was primarily located in the orbit m 25 cases (76%), conjunctiva in 4 (12%), eyelid in 1 (3%), and uveal tract in 3 (9%). Findings had been present for a mean of 5 weeks and included proptosis in 10 patients (30%), eyelid swelling in 7 (21%), and blepharoptosis in 6 (18%). The initial diagnoses before referral to us included primarily rhabdomyosarcoma in 8 cases (24%), conjunctivitis in 5 (15%), cellulitis in 5 (15%), and pseudotumor in 4 (12%). Tumors were classified according to the Intergroup Rhabdomyosarcoma Study Group staging and treatment protocols as group I in 4 cases (12%), group II in 12 (36%), group III in 16 (48%), and group IV in 1 case (3%). Treatment included surgical debulking, chemotherapy, and radiotherapy. Local tumor recurrence was detected in 6 patients (18%), lymph node spread in 2 (6%), and distant metastasis in 2 (6%). Long-term visual outcome of the 28 patients who maintained their globe was 20/20 to 20/40 in 11 patients (39%), 20/50 to 20/100 in 5 (18%), and 20/200 or worse in 12 (43%). Mean follow-up was 8.3 years; tumor-related death occurred in 1 patients (3%)., Conclusions: Rhabdomyosarcoma can present in the orbit, eyelid, conjunctiva, and uveal tract. Following treatment, local tumor recurrence occurs in 18% of cases, metastasis in 6%, and death in 3%.

Purpose: In order to further assess the survival value of orbital exenteration in malignant orbital and periorbital tumors., Methods: The charts of 44 patients exenterated for a neoplasm of the orbit or periorbit were reviewed in a retrospective study., Results: The overall 4-year survival was 45%. 26 patients had free margins on histological examination and 24 patients had developed local recurrence or metastasis. Local recurrence or metastasis were significantly more common in the group with transected margins than in the group with free-margins (p= 0.01). Survival between the group of patients with local recurrence or metastasis and the group without local recurrence or metastasis showed statistically significant difference (p=0.0025). In contrast, survival between the group of patients with free margins and the group with transected margins did not show statistically significant difference (p=0.13)., Conclusion: Surgical free margins section is a key element in successful cancer surgery but seems not the only prognosis variable.

Purpose: The influence of tumor size, grade, thoroughness of staging workup, and radiation dose on disease control, radiation-related complications, and incidence of systemic progression of primary orbital lymphoma is analyzed., Methods and Materials: Twenty patients with Stage I primary orbital lymphoma were treated from August 1976 through August 1991 at Mallinckrodt Institute of Radiology. Staging workups included physical examination, chest x-ray, complete blood count (CBC), liver function test, and computerized tomography (CT) scan of the orbit, abdomen, and pelvis. Nineteen patients had bone marrow biopsy. The histological types based on the National Cancer Institute working formulation were 9 low-grade and 11 intermediate-grade, including five lymphocytic lymphomas of intermediate differentiation. The extension of disease and the volume of tumor were evaluated by CT scan of the orbit. The most commonly used radiation therapy technique was single anterior direct field with 4 MV or 6 MV photons. Lens was shielded or not treated in eight patients. Dose ranged from 20 to 43.2 Gy. Thirteen of 20 patients received 30 Gy. Minimum follow-up was 24 months (median, 4 years)., Results: Local control was achieved in all 20 patients. One patient with lymphocytic lymphoma with intermediate differentiation developed disseminated disease. Actuarial disease-free survival (DFS) was 100% and 90% at 2 and 5 years, respectively. No retinopathy was observed. Cataracts were noted in seven patients at 1 to 10 years following irradiation (median, 2 years). Three patients developed lacrimal function disorder; however, no corneal ulceration occurred., Conclusions: Thirty Gy in 15 fractions appears to be a sufficient dose for local control with acceptable morbidity, especially for low-grade, as well as certain types of intermediate-grade lymphomas, such as diffuse small cleaved cell and lymphocytic lymphoma of intermediate differentiation. Systemic dissemination is minimal, provided local control is achieved and initial staging workups are adequate.

Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and lymphoma. No clinical or radiologic criteria facilitate a distinction among these lymphoproliferative lesions. The two hyperplastic processes may evolve to localized or systemic lymphoma. A similar pattern is evident in other mucosa-associated lymphoid tumors elsewhere in the body. Most ocular adnexal lymphomas are small lymphocytic non-Hodgkin's tumors with an indolent course; frequently, they remain localized to the ocular adnexa. In comparison, intermediate- and high-grade lymphomas are less common in the ocular adnexa but more aggressive. An approach to the diagnosis and treatment of these complex entities is suggested. Despite new pathologic classification schemes, immunophenotypic labeling, and molecular genetic analysis, the prognosis for patients with small-cell lymphoma in the ocular adnexa is difficult to predict.

Teamwork between neurosurgeons and head and neck surgeons has increased the possibility of managing tumours involving the skull base. The authors distinguish the anterior half of the skull base into a median and two lateral portions and describe the surgical approaches utilized to expose it. The authors present their clinical experience in 27 patients with extensive lesions of the anterior half of the skull base and their results.

This report documents the long-term clinical and histopathological behaviour of eight intraorbital and 16 intracranial optic nerve gliomas and relates the therapeutic data to the prognosis for both visual acuity and survival. The mean age at onset of symptoms was 8.6 years and at the diagnosis 10.9 years. It is generally held that proptosis is mild in intraorbital glioma, but we encountered marked proptosis ranging from 7 to 12 mm in six of the eight intraorbital gliomas, which contained abundant Alcian-blue-positive mucoid material. Of the patients with intracranial optic nerve gliomas 37.5% survived for a mean of eight years after treatment with radiotherapy or surgery combined with radiotherapy. At follow-up ranging from five months to 11 years only one of the six patients with intracranial gliomas had full visual acuity. Our observations emphasise that, although optic nerve gliomas are benign hamartomas, the prognosis for visual acuity and survival is unfavourable in cases which are diagnosed and treated late. Histopathological and histochemical observations suggest that increase in the amount of mucoid material may contribute to rapid enlargement of intraorbital and intracranial optic gliomas.