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.

Association of T and N Categories of the American Joint Commission on Cancer, 8th Edition, With Metastasis and Survival in Patients With Orbital Sarcoma.

Importance: No previous studies to date have validated the American Joint Committee on Cancer (AJCC) 8th edition of the TNM classification for orbital sarcoma. Objectives: To determine the prognostic performance of the most recent TNM classification for orbital sarcoma and to identify other prognostic factors for local recurrence, lymph node metastasis, distant metastasis, and death due to disease. Design, Setting, and Participants: This single-center retrospective cohort study included 73 consecutive patients treated for orbital sarcoma from March 1, 2003, through June 30, 2018. Data were analyzed from November 1 to December 31, 2018. Main Outcomes and Measures: T and N categories at presentation and disease-related outcomes, including local recurrence, lymph node metastasis, distant metastasis (DM), and death due to disease (DD). Results: The 73 participants included 43 men (59%), and the median age was 21 (range, 0-77) years. The common histologic types were rhabdomyosarcoma (RMS) (35 [48%]), solitary fibrous tumor/hemangiopericytoma (10 [14%]), and Ewing sarcoma (8 [11%]). The most common TNM designations were T2 N0 M0 (26 [36%]) and T4 N0 M0 (24 [33%]). T category was associated with the risk of all disease-related outcomes, including local recurrence (hazard ratio [HR] for T2 vs T4, 0.22 [95% CI, 0.06-0.81]; HR for T3 vs T4, 0.59 [95% CI, 0.13-2.65]; P = .03), lymph node metastasis by the last follow-up (T1, 1 [14%]; T2, 0; T3, 0; T4, 12 [35%]; P = .001), DM (HR for T2 vs T4, 0.29 [95% CI, 0.08-1.07]; P = .04), and DD (HR of T2 vs T4, 0.16 [95% CI, 0.04-0.73]; HR of T3 vs T4, 0.30 [95% CI, 0.04-2.34]; P = .02). Higher risk of DM and higher risk of DD were associated with disease category of at least T3 (HR for DM, 3.24 [95% CI, 0.89-11.72; P = .06]; HR for DD, 6.32 [95% CI, 1.43-27.95; P = .005]), N1 disease (HR for DM, 13.33 [95% CI, 4.07-43.65; P < .001]; HR for DD, 7.07 [95% CI, 2.45-20.44; P < .001]), tumor size larger than 3 cm (HR for DM, 2.72 [95% CI, 0.92-8.05; P = .06]; HR for DD, 5.79 [95% CI, 1.85-18.14; P < .001]), and age of patient with RMS younger than 1 year or 10 years or older (HR for DM, 6.85 [95% CI, 0.83-56.53; P = .04]; HR for DD, 7.03 [95% CI, 0.85-57.83; P = .04]). Higher risk of local recurrence was associated with disease category of at least T3 (HR for3 cm, 0.27 [95% CI, 0.09-0.77]; P = .009). Higher risk of lymph node metastasis was associated with disease category of at least T3 (odds ratio [OR], 13.33 [95% CI, 1.77-602.30]; P = .004), alveolar RMS (OR, 9.98 [95% CI, 2.13-51.55]; P = .001), and age of patient with RMS younger than 1 year or 10 years or older (OR, 9.20 [95% CI, 1.01-458.29] P = .03). Conclusions and Relevance: In patients with orbital sarcoma, T and N categories at presentation (defined by the AJCC 8th edition classification) correlate with metastasis and survival. These findings appear to support consideration of strict surveillance testing for regional nodal and systemic metastases in patients with orbital sarcoma with disease category of at least T3 and/or N1 disease.

Evaluation of cytology and histopathology for the diagnosis of canine orbital neoplasia: 112 cases (2004-2019) and review of the literature.

OBJECTIVE: To provide an updated overview of canine orbital neoplasia, to compare diagnostic utility of cytology and histopathology, and to evaluate alternative sampling modalities, particularly image-guided core needle biopsy. PROCEDURES: A medical records search was performed to identify dogs with orbital neoplasia. Data were collected regarding signalment, diagnosis, vision status, imaging modalities, and sample collection methods. A reference population with orbital neoplasia was also identified via literature search for comparison with regard to final diagnosis. RESULTS: One hundred and twelve dogs met selection criteria. In the study and reference populations, respectively, diagnoses were grouped as follows: mesenchymal tumors 40% and 35%, epithelial tumors 35% and 18%, tumors of neural origin 8% and 37%, and round cell 17% and 10%. The most common diagnoses in the study group were nasal adenocarcinoma, osteosarcoma, lymphoma, and meningioma. Cytology results were available for 47 dogs and histopathology results were available for 95 dogs. Both cytology and histopathology results were available for 30 dogs, in 53% of which results were discordant. Cytology samples were nondiagnostic or provided a diagnosis that was later overturned in 32% of cases in which they were obtained. Results from core needle biopsy samples were nondiagnostic or overturned by surgical biopsy results in only 13% of cases. No significant complications were associated with any sampling method. CONCLUSIONS: Orbital neoplasia is common in dogs. Histopathology is superior to cytology in providing a definitive diagnosis. Image-guided core needle biopsy appears to be a safe and effective means of obtaining samples.

Spectrum of Biopsy Proven Extraocular Muscle Tumours of Non-Thyroid Origin.

OBJECTIVE: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017. METHODOLOGY: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly. RESULTS: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy. CONCLUSION: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.

Orbital Lymphoma-An International Multicenter Retrospective Study.

PURPOSE: To investigate and characterize the clinical features of subtype-specific orbital lymphoma. DESIGN: Retrospective, interventional case series. METHODS: The study included 7 international eye cancer centers. Patient data were collected from January 1, 1980 through December 31, 2017. A total of 797 patients with a histologically verified orbital lymphoma were included. The primary endpoints were overall survival, disease-specific survival, and progression-free survival. RESULTS: The median age was 64 years, and 51% of patients (n = 407) were male. The majority of lymphomas were of B-cell origin (98%, n = 779). Extranodal marginal zone B-cell lymphoma (EMZL) was the most frequent subtype (57%, n = 452), followed by diffuse large B-cell lymphoma (DLBCL) (15%, n = 118), follicular lymphoma (FL) (11%, n = 91), and mantle cell lymphoma (MCL) (8%, n = 66). Localized Ann Arbor stage IE EMZL and FL were frequently treated with external beam radiation therapy. DLBCL, MCL, and disseminated EMZL and FL were primarily treated with chemotherapy. EMZL and FL patients had a markedly better prognosis (10-year disease-specific survival of 92% and 71%, respectively) than DLBCL and MCL patients (10-year disease-specific survival of 41% and 32%, respectively). CONCLUSIONS: Four lymphoma subtypes were primarily found in patients with orbital lymphoma: EMZL, DLBCL, FL, and MCL. The histologic subtype was found to be the main predictor for outcome, with EMZL and FL patients having a markedly better prognosis than DLBCL and MCL.

Vascular Anomalies of the Orbit--A Reappraisal.

The recent International Society for the Study of Vascular Anomalies (ISSVA) classification of vascular anomalies can be applied to orbital lesions, dividing them into vascular tumors and vascular malformations. Orbital cavernous hemangiomas are probably best considered cavernous venous malformations under this classification. Management of symptomatic lesions can be with surgical excision or stereotactic fractionated radiotherapy in selected cases. Beta-blockers including propranolol and topical timolol maleate represent first-line therapy for infantile hemangiomas, although surgery has a role in selected cases. Orbital venous-lymphatic malformations are problematic but with improved imaging, neuroradiological intervention, and a multidisciplinary approach to management, outcomes are improving.

Types of orbital osteoma - A descriptive analysis.

AIMS: This study describes different types of orbital osteoma based on clinical per-operative morphology and radiological findings to facilitate communication between ophthalmologists and surgical management for a better patient outcome. MATERIALS AND METHODS: The study was conducted in the Orbit and Oculoplastics Department of Al-Shifa Trust Eye Hospital, Rawalpindi, Pakistan, from July 1, 2001 to June 30, 2014. A retrospective analysis of 520 diagnosed orbital tumours who presented to us was carried out and the prevalence of orbital osteoma was determined. The sampling technique was non-randomized sampling. Based on the clinical morphology observed during surgical intervention as well as the radiological findings of the orbital osteoma, a simple yet useful description of orbital osteoma was introduced. RESULTS: The retrospective analysis of 520 diagnosed orbital tumours revealed a prevalence of 2.3% (n = 12) of orbital osteoma. Based on our observation of the 12 (n = 12) cases of orbital osteoma, we have classified orbital osteoma into a combination of seven types: "sessile" or broad-based osteoma; "pedunculated" or mushroom osteoma with a thin cylindrical stalk of origin; "dumb-bell"-shaped osteoma with the simultaneous presence in orbital and adjoining sinus/nasal cavity; "wrapped" osteoma, covered by a thin cartilaginous layer; "naked" osteoma, without any such covering; "homogenous" or uniformly dense osteoma; and "heterogeneous", soft-dense osteoma with varying areas of density. CONCLUSION: This study attempts to introduce a simple description of different types of orbital osteoma based on clinical per-operative morphology and radiological findings for the first time to facilitate the surgical removal of orbital osteoma.

Orbital Dermoid Cyst: Classification and its Impact on Surgical Management.

PURPOSE: Analysis of different clinical types of orbital dermoid cyst, and studying the impact of preoperative classification on the proposed surgical procedure. METHODS: A retrospective study of orbital dermoid cyst cases. The clinical and imaging data were reviewed. The different types of dermoid were recorded as superficial or deep, lateral or medial, exophytic or endophytic. The deep dermoids were classified according to their site and continuity of the cyst wall. The operative approach, intraoperative, and/or postoperative complications were recorded. RESULTS: The study included 153 cases of orbital dermoid. The superficial lesions represented 68.6%, of which 74.3% were lateral and 61.0% were endophytic. Intraoperative cyst perforation was recorded in 56.3% of exophytic cases approached through crease incision, and recurrence occurred in 6.3%. Defective cyst wall was found in 31.25% of deep orbital dermoid. Surgery was complicated by cyst rupture when excision was attempted, and recurrence was recorded in one case. Exposure, intended evacuation, and dissection of the cyst wall were better surgical approaches for these cases without reported complications. CONCLUSION: Preoperative classification of orbital dermoid had an important effect on the surgical procedure. Endophytic superficial lesions were approached through crease incision without complications, while exophytic lesions were better approached through infrabrow incision. Deep orbital lesions with defective wall were better approached through exposure, intended evacuation, dissection, and excision of the remaining cyst wall.

Spheno-Orbital Meningiomas: An Analysis Based on World Health Organization Classification and Ki-67 Proliferative Index.

PURPOSE: To evaluate the clinical behavior of spheno-orbital meningiomas with regard to World Health Organization (WHO) tumor grade and Ki-67, a cellular marker of proliferation. METHODS: A retrospective review over a 16-year period of the demographic, clinical, radiographic, and surgical data of all patients with spheno-orbital meningioma who underwent surgical resection. Tumor specimens were examined histologically using the current WHO 2016 classification and immunohistochemically using Ki-67/MIB-1 monoclonal antibody. RESULTS: Thirty-eight patients met all inclusion criteria: 78.9% of tumors were WHO grade I with a mean Ki-67 of 3.76, and 93% of patients were clinically stable at last follow up; 10.5% of lesions were WHO grade II (atypical) with a mean Ki-67 of 14.93, and 10.5% of lesions were WHO grade III (anaplastic) with a mean Ki-67 of 58.3. All grade II and III meningiomas exhibited an aggressive clinical course. There were statistically significant correlations between disease clinical progression and WHO tumor grade (p < 0.001), between disease clinical progression and Ki-67 (p < 0.001), and between increasing Ki-67 index and higher WHO grade (p < 0.001). For WHO grade I lesions, a Ki-67 of ≥3.3 correlated with recurrence (p = 0.0256). Overall, disease-specific mortality occurred in 5 (13%) patients. CONCLUSIONS: Ki-67 index is a valuable marker to use in conjunction with WHO grade to predict meningioma behavior, particularly in histologically borderline lesions, and possibly to identify a subset of WHO grade I tumors at risk of recurrence. This combination of methods can aid in tailoring treatment and surveillance strategies.

Recent Advances in Orbital Tumors--A Review of Publications from 2014-2016.

The aim of this review article is to provide an update of the current literature on orbital tumors. The authors conducted a PubMed literature search of English language articles published between January 2014 and December 2016 using the following search items: orbit, tumors, lacrimal gland, lymphoma, hemangioma, lymphangioma. The authors included reviews, original articles, case series, and case reports with relevant new information. There is new information about the clinical spectrum of orbital tumors, capillary hemangioma, cavernous hemangioma, lymphangioma, orbital venous malformation, lacrimal gland tumors, and orbital lymphoma. This review highlights the current understanding, practice, and guidelines in the diagnosis and management of common tumors of the orbit.

[Transnasal endoscopic surgery of communicating neoplasm outside cerebral dura mater and orbital fasciae].

Objective:This paper is intend to observe the feasibility of surgical approach with which communicating neoplasm outside cerebral dura mater and orbital fasciae was removed by transnasal endoscopic surgery.Method:Nine patients suffered from communicating neoplasm in the area of naso-orbital-cranio complex were treated with transnasal endoscopic surgery. The size of skull base bone defect was from 1.25 cm×1.13 cm to 3.25 cm×2.69 cm. No eye enucleation was done. No reconstructions of skull base and medial wall of orbital cavity were done. Iodoform gauze was packed in paranasal sinuses fourteen days. Patients with malignant tumor were treated radiation therapy and chemical therapy after surgical treatment. The degree of resection, complications of intracranial and orbital cavities, survival condition were the focus of this study.Result:The tumor were completely removed and all operations were successfully completed by trasnasal endoscopic surgery. Only one patient was required blood transfusion due to seriously bleeding from tumor during the operation. There was no orbital, intracranial, and vascular complications. The defect of bone at skull base and lamina papyracea were automatic reconstructed. Three patients have showed recurrence of neoplasm, and two of them died during two years of following up time.Conclusion:Transnasal endoscopic surgery is a safe, reliable and effective approach to treat communicating neoplasm in the area of naso-orbital-cranio complex, of which main part of tumor exists in the sinunasal cavity.

Fine-Needle Aspiration Biopsy in Orbital Lesions: A Retrospective Study of 225 Cases.

PURPOSE: To describe and analyze results from the fine-needle aspiration biopsy (FNAB) technique, used as a diagnostic tool, in patients with orbital lesions. DESIGN: Retrospective case series. METHODS: setting: Institutional (Karolinska Hospital, Stockholm). STUDY POPULATION: 207 patients with 210 orbital lesions. INTERVENTION: 225 FNABs of the orbit. MAIN OUTCOME MEASURES: Successful diagnosis from FNAB. RESULTS: Of the 210 orbital lesions evaluated with FNAB, a successful cytologic diagnosis was achieved in 176 (84%). In more than half of the orbital lesions (54%), the FNAB diagnosis in addition to imaging appearance, clinical appearance, and clinical history provided sufficient information for treatment, and the patient did not require an incisional or excisional biopsy. Ninety-seven patients underwent additional excisional or incisional biopsy; FNAB diagnoses and the histopathologic diagnoses corresponded in 87% of the cases with an intraclass correlation coefficient of 0.84 (95% confidence interval [CI] 0.76-0.90). The difference was statistically significant between the ability to make a successful cytologic FNAB diagnosis in palpable lesions vs nonpalpable lesions (successful diagnosis in 90% [CI = 85%-95%] vs 75% [CI = 66%-84%]; P < .01). Neither the orbital quadrant location, nor the radiologic appearance (diffuse vs encapsulated), nor size of the lesion affected the success of FNAB diagnoses (all P > .7). There was a complication in 6 cases (3%). All complications were temporary and none led to permanent damage. CONCLUSIONS: FNAB proved effective and exceedingly safe. With the current healthcare climate of minimally invasive surgery and cost control, FNAB should be considered as a valid alternative to open surgery in the evaluation and management of orbital lesions.

A simple classification of cranial-nasal-orbital communicating tumors that facilitate choice of surgical approaches: analysis of a series of 32 cases.

Cranial-nasal-orbital communicating tumors involving the anterior and middle skull base are among the most challenging to treat surgically, with high rates of incomplete resection and surgical complications. Currently, there is no recognized classification of tumors with regard to the choice of surgical approaches. From January 2004 to January 2014, we classified 32 cranial-nasal-orbital communicating tumors treated in our center into three types according to the tumor body location, scope of extension and direction of invasion: lateral (type I), central (type II) and extensive (type III). This classification considerably facilitated the choice of surgical routes and significantly influenced the surgical time and amount of hemorrhage during operation. In addition, we emphasized the use of transnasal endoscopy for large and extensive tumors, individualized treatment strategies drafted by a group of multidisciplinary collaborators, and careful reconstruction of the skull base defects. Our treatment strategies achieved good surgical outcomes, with a high ratio of total resection (87.5 %, 28/32, including 16 cases of benign tumors and 12 cases of malignant tumors) and a low percentage of surgical complications (18.8 %, 6/32). Original symptoms were alleviated in 29 patients. The average KPS score improved from 81.25 % preoperatively to 91.25 % at 3 months after surgery. No serious perioperative complications occurred. During the follow-up of 3 years on average, four patients with malignant tumors died, including three who had subtotal resections. The 3-year survival rate of patients with malignant tumors was 78.6 %, and the overall 3-year survival rate was 87.5 %. Our data indicate that the simple classification method has practical significance in guiding the choice of surgical approaches for cranial-nasal-orbital communicating tumors and may be extended to other types of skull base tumors.

Genetic profiling by single-nucleotide polymorphism-based array analysis defines three distinct subtypes of orbital meningioma.

Orbital meningiomas can be classified as primary optic nerve sheath (ON) meningiomas, primary intraorbital ectopic (Ob) meningiomas and spheno-orbital (Sph-Ob) meningiomas based on anatomic site. Single-nucleotide polymorphism (SNP)-based array analysis with the Illumina 300K platform was performed on formalin-fixed, paraffin-embedded tissue from 19 orbital meningiomas (5 ON, 4 Ob and 10 Sph-Ob meningiomas). Tumors were World Health Organization (WHO) grade I except for two grade II meningiomas, and one was NF2-associated. We found genomic alterations in 68% (13 of 19) of orbital meningiomas. Sph-Ob tumors frequently exhibited monosomy 22/22q loss (70%; 7/10) and deletion of chromosome 1p, 6q and 19p (50% each; 5/10). Among genetic alterations, loss of chromosome 1p and 6q were more frequent in clinically progressive tumors. Chromosome 22q loss also was detected in the majority of Ob meningiomas (75%; 3/4) but was infrequent in ON meningiomas (20%; 1/5). In general, Ob tumors had fewer chromosome alterations than Sph-Ob and ON tumors. Unlike Sph-Ob meningiomas, most of the Ob and ON meningiomas did not progress even after incomplete excision, although follow-up was limited in some cases. Our study suggests that ON, Ob and Sph-Ob meningiomas are three molecularly distinct entities. Our results also suggest that molecular subclassification may have prognostic implications.

Undifferentiated sarcoma of the orbit with angiomyxoid features.

A 7-month-old female infant developed a grade 3 un-differentiated sarcoma with angiomyxoid features of the right orbit. The tumor expanded rapidly 2 months after an initial sub-total resection and extended posteriorly toward the right cavernous sinus. After treatment with ifosphamide, doxorubicin, and proton beam radiation, there was no discernible residual tumor 20 months after starting chemotherapy.

Primary orbital tumors: a review of 122 cases during a 23-year period: a histo-clinical study in material from the ENT Department of the Medical University of Silesia.

BACKGROUND: The aim of this study was to investigate the distribution of different types of primary orbital tumors, histopathological diagnosis, and postoperative complications. MATERIAL AND METHODS: We analyzed 122 patients (68 women and 54 men) with orbital tumors, hospitalized in the ENT Department of the Medical University of Silesia in Katowice during 1990-2013. The patients were characterized in terms of anatomic, topographical, histopathological, and clinical parameters. The role of diagnostic imagining such as CT, NMR, and fine-needle aspiration (FNB) in preoperative diagnostics is discussed. RESULTS: of FNB, cytological, and histopathological examination of the postoperative specimens were compared. Results There were 56 (46%) patients with malignant tumors, 42 (34%) with benign tumors, 19 (16%) with inflammatory tumors, and 5 patients (4%) had other tumors. In cases of malignant tumors, local recurrence up to 5 years was found in 36 (64.3%) cases. In the other 20 (35.7%) cases of malignant tumors, the patients remained under close follow-up in the outpatient clinic, without signs of local recurrence (follow-up 1-17 years). According to histopathological examination, malignant tumors were detected in 45.9% of patients and non-malignant tumor in 34.4% of patients. In 19.7% of patients, inflammatory and other types of tumors were diagnosed. CONCLUSIONS: We characterized the occurrence and pathological profiles of orbital tumors. The tumor location, histopathological diagnosis, and postoperative complications give us important information for the diagnosis of tumor prior to biopsy or tumor resection and for the determination of the treatment strategy and possible complications after surgery.

Comparison of American Joint Committee on Cancer TNM-based staging system (7th edition) and Ann Arbor classification for predicting outcome in ocular adnexal lymphoma.

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

Cancer of the orbit and adnexa.

The diagnosis and treatment of orbital and periorbital malignancies are challenging. These tumors can result in blindness, death, and significant cosmetic deformities. Herein, we present the most common ocular malignancies and a systematic approach to diagnosis. Further, we integrate the seventh edition American Joint Committee on Cancer staging system, biomarkers, and multidisciplinary approaches to treatment.

[Orbital neoplasia. Part II: mesenchymal, leukemic, lymphoproliferative and bony tumors, metastasis]. / Orbitale Neoplasien. Teil II: Mesenchymale, leukämische, lymphoproliferative und Knochentumoren, Metastasen.

With reference to the first article on the topic orbital neoplasias this second part focuses on the main characteristics of mesenchymal, leukemic, lymphoproliferative, osseous as well as secondary neoplasias of the orbit. For each entity the therapeutic approaches are presented. A large number of case report examples underline once more the importance of an efficient diagnostic procedure to ensure sufficient treatment of the lesion itself as well as the relatively common underlying systemic disease.