Primary nasal and nasopharyngeal lymphomas: a comparative study of clinical presentation and treatment outcome

Primary nasal lymphoma (NSL) and nasopharyngeal lymphoma (NPL) are uncommon extranodal lymphomas that have often been grouped together in studies published in the literature. It is unclear whether or not NSL and NPL are biologically different entities.We reviewed the records of 25 NSL and 19 NPL patients who were managed between 1985 and 1995, to evaluate and compare their clinical characteristics and treatment outcome. Clinical variables, including age, sex, stage, tumour bulk and treatment modality, were assessed for their prognostic influence on survival.Nasal obstruction (80%), nasal discharge (64%) and epistaxis (60%) were the predominant symptoms in NSL patients; neck swelling/mass (42%), nasal obstruction (37%) and hearing impairment (32%) were common findings in NPL patients. Limited stage disease (I/II) was present in all NSL and in 80% of the NPL patients. Thirty-six per cent of the NSL and 32% of the NPL patients had bulky disease. The majority of the immunophenotyped NSLs were of T-cell lineage (75%, 12/16) and most immunophenotyped NPLs were of B-cell lineage (69%, 11/16). The patients were treated with radiotherapy alone (14%), chemotherapy alone (23%) or chemotherapy plus radiotherapy (64%). NSL patients showed a lower complete remission rate and higher relapse rate than NPL patients, although the difference did not reach statistical significance. Both the 5-year disease-free survival (36% versus 76%; P = 0.007) and overall survival (33% versus 82%; P = 0.003) were significantly worse in NSL compared with NPL patients. Advanced age (>60 years; P = 0.03) and bulky disease (P = 0.04) were also associated with inferior survival times. The analysis of sex, stage and type of therapy showed no evidence of significant impact on the survival.Despite the close anatomical relationship in origin, NSL and NPL were noted to behave as biologically distinct entities, in which the NSLs demonstrated a higher frequency of T-cell tumours and a much worse prognosis than NPL. In addition to the primary site, advanced age and bulky disease were also associated with reduced survival.     

Primary gastrointestinal lymphomas: A radiological–pathological review. Part 1: Stomach,oesophagus and colon

Primary gastrointestinal lymphomas are most common in the stomach, followed by small intestine and then colon. The most frequently used pathology classification of lymphomas is the Revised European and American Lymphoma /World Health Organization classification. The correlation of radiological morphology with histology is relatively poor, although characteristic subtypes will be discussed. In the stomach, the majority of primary lymphomas are of B‐cell origin of mucosa‐associated lymphoid tissue (MALT) type. Low‐grade MALT lymphomas are associated with Helicobacter pylori infection and often respond to eradication of this organism. Radiological features include thickened folds, nodularity, depressed lesions, ulcers, prominent areae gastricae. High‐grade (large B‐cell) tumour patterns include infiltrative, polypoid, nodular, ulcerated or a combination. Endoscopy, endoscopic ultrasound and CT are important in diagnosis and staging, although appearances on barium studies should be recognized. Primary colonic lymphomas are rare. Most are of B‐cell origin. Focal and diffuse forms are seen, the former producing polypoid or nodular or cavitating masses and the latter producing ulcerative or nodular (polyposis) patterns on imaging. Even when circumferential, lymphoma rarely causes obstruction. Small bowel lymphomas will be discussed in the forthcoming part 2 of this review.     

Primary gastrointestinal lymphomas: A radiological–pathological review. Part 2: Small intestine

Primary lymphoma of the small intestine is a heterogeneous group of diseases reflected in its protean imaging features. This review illustrates the widespread appearances seen on imaging methods (primarily CT and barium studies) with pathological correlation.     

Clinicopathological features of pyothorax-associated lymphoma; a retrospective survey involving 98 patients.

To investigate clinicopathological features of pyothorax-associated lymphoma (PAL), we examined medical records of 98 patients (88 males and 10 females) with PAL at a median age of 70 years (range 51-86). Seventy-nine patients had a history of artificial pneumothorax. Median interval between diagnosis and artificial pneumothorax was 43 years (range 19-64). At diagnosis, performance status (PS) was 0-1 (n=56) and 2-4 (n=42). Clinical stages were I (n=42), II (n=26), III (n=8) and IV (n=22). Pathological diagnosis comprised diffuse large-B-cell (n=78) and peripheral T-cell lymphoma (n=1). Seventeen were treated supportively. The other 81 received aggressive treatments; chemotherapy (n=52), radiotherapy (n=7), surgery (n=4) and combination (n=18). Five-year overall survival (OS) was 0.35 (95% confidence interval, 24% to 45%). Causes of deaths were PAL (n=39), respiratory failure (n=13) and others (n=12). Multivariate analysis identified prognostic factors for OS; lactate dehydrogenase levels [hazard ratio (HR)=2.36; P=0.013], sex (female versus male) (HR=0.15; P=0.01), PS (2-4 versus 0-1) (HR=2.20; P=0.02), clinical stages (III/IV versus I/II) (HR=1.95; P=0.037) and chemotherapy (HR=0.31; P=0.01). Most patients with PAL are elderly and have comorbidities, while some of them achieve durable remission with appropriate treatments. These findings prompt us to establish an optimal treatment strategy on the basis of risk stratification of individual patients.     

儿童侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点

 目的　分析总结中国儿童各类型侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点,为其诊断的标准化提供依据。方法　收集97例儿童侵袭性成熟B细胞淋巴瘤石蜡包埋组织标本,包括伯基特淋巴瘤（BL）81例、弥漫大B 细胞淋巴瘤（DLBCL）8例、介于BL和DLBCL间的不能分类的B细胞淋巴瘤（BL／DLBCL）8例,利用免疫组织化学技术和间期荧光原位杂交（FISH）技术检测其免疫表型和分子遗传学特征。结果　BL的bcl-2和MUM1的阳性率分别为3 %（2／66）和17 %（12／71）,DLBCL分别为50 %（4／8）和63 %（5／8）,BL／DLBCL分别为 50 %（4／8）和63 %（5／8）。BL、DLBCL 和BL／DLBCL 的Ki-67平均值分别为（93±4.4）%、（83±14.3）%和（80±11.5）%。BL、DLBCL 和BL／DLBCL 的c-myc 基因易位的比例分别为98 %（79／81）、38 %（3／8）和50 %（4／8）。38 %（3／8）的DLBCL和25 %（2／8）的BL／DLBCL 存在bcl-6基因的多拷贝,BL与DLBCL 之间、BL与BL／DLBCL之间bcl-2、MUM1和 Ki-67平均值的差异及c-myc基因易位和bcl-6基因多拷贝的差异均有统计学意义（均P＜0.05）。结论　儿童侵袭性成熟B细胞淋巴瘤的诊断和分型需要综合分析形态学、免疫表型和分子遗传学特征。儿童BL／DLBCL 可能是DLBCL 的一个亚型。CD+10、bcl-6+、bcl-2-、Ki-67＞90 %、伴有IGH／c-myc重排、不伴有bcl-2和bcl-6重排时,支持BL的诊断;bcl-2+、Ki-67 为50 %～90 %,同时伴有bcl-6基因的多拷贝时,支持 DLBCL或BL／DLBCL 的诊断。     

Primary central nervous system lymphoma in immunocompetent patients: a retrospective review of MRI features

Introduction: To define the features of primary central nervous system lymphoma (PCNSL) on MRI in immunocompetent patients. Methods: A retrospective review of the authors' institutional database was performed to identify histologically proven cases of PCNSL. Images were retrieved and reviewed with respect to location, lesion number, size, signal intensity, enhancement characteristics, oedema and necrosis. Results: Thirty‐one cases of histologically proven PCNSL had available imaging. One patient was excluded due to immunosuppression. Of the 30 remaining cases, the average age was 65.5 years, and males and females were equally represented. A total of 68 lesions (average of 2.5 per patient) were identified. With diffusion‐weighted imaging, all but two had restricted diffusion (40.3% mild and 55.6% marked) and all but one had enhancement (51.5% homogeneous, 42.6% heterogeneous and ring 4.4%). Most lesions were isointense to grey matter (75.8% on T2‐weighted image (WI) and 82.5% on T1‐WI). Oedema was mild in 43.4% and marked in 55.2%. Necrosis was seen in only five lesions (7.4%). On a per patient basis, 50% had bilateral lesions and 96.7% had lesions contacting a cerebrospinal fluid (CSF) surface. 16.7% of patients had posterior fossa involvement and 30% had lesions in the basal ganglia or thalami. Conclusion: The vast majority of cases of PCNSL in immunocompetent patients have lesions contacting a CSF surface, enhancement and restricted diffusion with no necrosis. These features should alert radiologists to the diagnosis of PCNSL.     

Incidence of gastric B-cell lymphomas: a population-based study in Germany.

BACKGROUND: While the clinical and experimental knowledge concerning gastric lymphomas is increasing, there is a scarcity of epidemiological data. PATIENTS AND METHODS: A population-based sample of patients in Franconia and Saarland in Germany was collected from a clinical trial, hospital archives and a cancer registry. RESULTS: Over a period of 3 years, 94 patients with primary gastric lymphoma were recorded out of a total population of 3.5 million. The standardised incidence rates in Saarland and Franconia were 0.7 and 0.8 cases per 100 000, respectively. Patients were predominantly from higher age groups (mean age 62.1 years) and the incidence in men was slightly more than in women (P <0.03). The distribution of histological subtypes in Franconia was as follows: marginal zone B-cell lymphomas (MZBL), 58%; diffuse large-cell B-cell lymphoma (DLBL), 33%; and mixed forms, 9%. Helicobacter pylori could be detected histologically in 84% of all cases, 95% of MZBL cases and 68% of DLBL cases. CONCLUSIONS: Incidence rates of gastric lymphoma in Germany were similar to that in other European countries, except England, where rates are lower. The subtype-specific differences of H. pylori infection rates could be due to differences in carcinogenesis or to secondary changes during malignant transformation.     

Primary gastrointestinal lymphomas: a classification of 66 cases.

Using routine histology, resin embedded sections and immunohistochemical techniques on formalin-fixed, paraffin processed tissue, 66 cases of primary gastrointestinal lymphoma have been classified. This study necessitated the development of reliable criteria to separate lymphomas of true histiocytic origin from those of lymphocytic origin. Among the morphologic properties of malignant histiocytes were complex pleomorphic nuclei, abundant well delineated cytoplasm and phagocytosis. These cells were shown to contain all major immunoglobulin chains, C3, lysozyme and in some cases alpha 1 antitrypsin. Malignant lymphomas derived from histiocytes could be divided into two groups: malignant histiocytosis of the intestine (MHI), a recently described diffuse pleomorphic lymphoma associated with villous atrophy of the small intestine, and histiocytic lymphoma (HL) which forms solid tumor masses in a similar manner to lymphocyte derived tumors. Immunohistochemical studies of lymphocyte derived tumors were negative apart from one case with plasmacytoid differentiation. Of the 66 cases, 50% were of histiocytic origin (33% MHI, 17% HL) and 41% of lymphocyte origin, there was one case of Hodgkin's disease and five cases were unclassified. The role of the histiocyte in gastrointestinal mucosa deserves further study.     

原发性腮腺非霍奇金淋巴瘤21例临床分析

目的：分析原发性腮腺非霍奇金淋巴瘤（NHL）的临床,病理,治疗及预后。方法：21例原发性腮腺NHL者均为手术后病例,T细胞NHL 1例,B细胞NHL20例,其中包括高度恶性2例,中度恶性6例,低度恶性12例[含粘膜相关淋巴组织（MALT）型NHL7例]。按Ann Anbor分期法,ⅠE16例,ⅡE5例,治疗均以放射治疗为主,放射治疗前后有11例行2－6周期化疗。结果：本组5年生存率为77．0％,共有5例死亡,均死于远地受累,结论：本组提示腮腺NHL有一定比例MALT型,该型在传统的工作分型中未列入,低剂量单纯放射治疗可作为此类患者的首选治疗,中高度恶性NHL则应行综合治疗。     

肺类癌12例临床分析

为了探讨肺类癌的临床特点、诊疗方法与预后的影响因素，对12例肺类癌病例进行回顾性研究。12例惠者中2例伴有类癌综合征，2例术前经纤支镜确诊。术式包括肺叶切除9例，开胸探查1例,全肺切除2例，无手术期死亡和并发症。全组5年生存率为72．65％．类癌5年生存率为84．23％，不典型类癌为66．68％。初步研究结果提示，肺类癌属低度恶性肿瘤；影像学及纤维支气管镜检查对诊断具有重要价值；光镜HE染色常可诊断，但嗜银染色及电镜确定特征性嗜银性神经内分泌颗粒是确诊的可靠标准；治疗应以保守性外科手术切除为主，尽量保留肺功能为手术原则；手术切除后预后良好，远期疗效明显优于肺癌。     

Primary central nervous system lymphomas (PCNSL): MRI features at presentation in 100 patients

To avoid an unnecessary extend of surgery in primary central nervous system lymphoma (PCNSL), the diagnosis should be suspected after MRI.Pre-treatment MRI examinations of 100 immunologically competent patients with biopsy-proven PCNSL were evaluated. All patients had T2- and T1-weighted images with contrast enhancement. Diffusion-weighted MRI (DW-MRI) was available in 15, proton-MR-spectroscopy (¹H-MRS) in four patients.The number of lesions ranged from one (n=65 patients) to eight (n=1) with a mean of 1.7. The most frequent locations were the cerebral hemispheres (n=66), the basal ganglia (n=27) and the corpus callosum (n=24). In the 65 patients with a solitary lesion, hemispheric lesions were most frequent (n=23) followed by corpus callosum (n=18). Contrast enhancement was found in all but one patient. ¹H-MRS revealed a uniformly pathologic pattern of metabolite concentrations in all patients.Characteristic imaging features of PCNSL are contrast-enhancing lesions with a diameter of at least 15 mm in contact with the subarachnoid space. DW-MRI and proton spectroscopy may aid in differential diagnosis.     

原发胃肠与原发结内弥漫大B细胞淋巴瘤临床特点及治疗预后的对比分析

目的:对原发胃肠弥漫大B细胞淋巴瘤(PGI-DLBCL)与原发结内弥漫大B细胞淋巴瘤(PN-DLBCL)的临床病理特点及治疗预后对比分析。方法:回顾性分析我院肿瘤中心2013年6月至2018年12月间收治确诊为PGI-DLBCL 70例和PN-DLBCL 80例,卡方检验比较原发胃肠与结内的DLBCL临床病理特征、治疗特点,应用Kaplan-Meier法进行生存分析,并行log-rank检验,多因素分析采用COX比例风险模型。结果:PGI-DLBCL较PN-DLBCL分期偏晚,LDH增高多,联合有手术治疗多,放疗相对较少。单因素分析提示,PGI-DLBCL的年龄、LDH、IPI积分这3项是预后的影响因素;结内DLBCL患者组年龄、双表达、LDH、IPI积分、KI67%值、分期这6项是预后的影响因素,COX多因素分析提示PGI-DLBCL的年龄≥60岁、LDH升高是其生存的独立危险因素;IPI≥3分、KI67≥70%是影响结内DLBCL生存的独立危险因素。结论:PGI-DLBCL患者应提高早期诊断率,治疗上可根据年龄、LDH独立的预后危险因素制定综合治疗方案,目标超过结内DLBCL的预后。     

Participants' uptake of clinical trial results: a randomised experiment

Background:

It is unknown whether breast cancer (BC) characteristics among young women treated with radiotherapy (RT) for Hodgkin''s lymphoma (HL) differ from sporadic BC.

Methods:

Using population-based data, we calculated BC risk following HL according to clinicopathologic features.

Results:

Compared with BC in the general population, risks of oestrogen receptor (ER)-positive/progesterone receptor (PR)-positive and ER-negative/PR-negative BC in young, irradiated HL survivors were increased five-fold (95% confidence interval (CI)=3.81–6.35) and nine-fold (95% CI=6.93–12.25), respectively. Among 15-year survivors, relative risk of ER-negative/PR-negative BC exceeded by two-fold (P=0.002) than that of ER-positive/PR-positive BC.

Conclusion:

Radiotherapy may disproportionately contribute to the development of BC with adverse prognostic features among young HL survivors.     

肺类癌12例临床分析

为了探讨肺类癌的临床特点、诊疗方法与预后的影响因素,对12例肺类癌病例进行回顾性研究。12例患者中2例伴有类癌综合征,2例术前经纤支镜确诊。术式包括肺叶切除9例,开胸探查1例,全肺切除2例,无手术期死亡和并发症。全组5年生存率为72.65%,类癌5年生存率为84.23%,不典型类癌为66.68%。初步研究结果提示,肺类癌属低度恶性肿瘤;影像学及纤维支气管镜检查对诊断具有重要价值;光镜HE染色常可诊断,但嗜银染色及电镜确定特征性嗜银性神经内分泌颗粒是确诊的可靠标准;治疗应以保守性外科手术切除为主,尽量保留肺功能为手术原则;手术切除后预后良好,远期疗效明显优于肺癌。     

鼻腔及鼻咽部恶性淋巴瘤60例临床病理观察

 报告鼻腔及鼻咽部恶性淋巴瘤60例，其中属中线恶组（MMH）41例，其中24例作免疫组化，证实为T细胞型淋巴瘤；中线非何杰金氏淋巴瘤（MNHL）19例，其中10例作免疫组化，证实为T细胞型4例，B细胞型6例。MMH与MNHL对比观察，前组有肉芽组织结构，其中有异型细胞，并常见淋巴上皮病表现及容易侵犯血管壁等特点，后组形态与淋巴结内淋巴瘤无异；据临床分析，前组以局部破坏及发烧为主，后者以鼻塞、耳聋及局部肿块为主，因此我们认为：MMH可看作一种特殊类型的淋巴瘤，有其独立的临床病理特点。     

Primary lymphoma of the thyroid: clinical features, prognostic factors, and results of treatment

This study analyzes the results of 46 cases of biopsy-proven primary lymphoma of the thyroid, localized at diagnosis and treated at the Royal Marsden Hospital between 1948-1980. In all cases, disease was limited to the thyroid and cervical lymph nodes. There were 43 females and 3 males, with a mean age of 64.4 years (median 66 years; range 17-86). A short history (less than 3 months) of a neck mass and local compressive symptoms was the typical presentation. The majority of patients (91%) had adverse histologies, with diffuse histiocytic lymphomas being most common (78%). The overall crude 5-year survival rate was 40%, with 30% of patients surviving beyond 10 years. Disease-free and overall survival were virtually identical, indicating the ineffectiveness of salvage therapy. The important prognostic factors were: size of tumor, fixation, extra-capsular extension, and retrosternal involvement. Survival was not correlated with age, sex, histology, thyroid status, or stage of disease, as defined by the Ann Arbor classification. Patients who had total macroscopic removal of tumor had the highest rate of local control and long survival (p less than 0.005). No significant difference was seen between lobectomy, subtotal or total thyroidectomy, but 7 of 9 long-term survivors (58-129 months) have had total thyroidectomy. A radiotherapy dose to the tumor, of at least 40 Gy, was required to produce consistent local control in patients with unresected disease. The majority of patients who died had generalized disease. Only 52% of these patients were free of local recurrence. In view of the frequency of occult disseminated disease, adjuvant chemotherapy is one recommendation for patients with poor prognostic factors. For optimal local control, complete surgical eradication is recommended followed by high-dose local radiotherapy. The use of aggressive cytoreductive chemotherapy prior to radiotherapy is a possible alternative option which needs to be explored.     

Primary cardiac lymphoma: an analysis of presentation, treatment, and outcome patterns

BACKGROUND:

Primary cardiac lymphoma (PCL) represents a rare subset of non‐Hodgkin lymphoma, characterized by poor outcomes. The authors aimed to construct a framework of known clinical presentations, diagnostic features, disease complications, treatment, and outcomes to improve prognostication.

METHODS:

Individual patient data were obtained from defined cases of PCL (1949‐2009) and systematically analyzed.

RESULTS:

The authors report results of a review of 197 cases of PCL, with half of all cases reported since 1995. Survival was affected by 4 factors: immune status, left ventricular involvement, presence of extra‐cardiac disease, and arrhythmia. Median overall survival (OS) for immunocompromised and immunocompetent was 3.5 months (m) and not reached, respectively (HR 0.29, 95% CI, 0.13‐0.68; P = .004). LV involvement was uncommon (26%) and associated with an OS of only 1m, whereas patients free of LV involvement had a median OS of 22m (HR 0.28, 95% CI, 0.12‐0.64; P = .002). Patients with extracardiac disease had shorter median OS compared with those without (6m vs 22m, HR 0.49, 95% CI, 0.26‐0.91; P = .02). Those patients with an arrhythmia of any type had a median OS that was not reached (n = 55), whereas those without rhythm disturbances (n = 41) had median OS of 6m (HR 0.51, 95% CI, 0.29‐0.91; P = .024). Overall response rate to therapy was 84%, with long‐term OS over 40%.

CONCLUSIONS:

The current study presents the largest analysis of PCL to date. The data demonstrate that PCL is now more frequently diagnosed premortem and appears to have reasonable response rates. Lack of LV involvement and the presence of arrhythmias are associated with improved survival. Cancer 2011. © 2010 American Cancer Society.     

Adenoid cystic carcinoma: a retrospective clinical review.

Adenoid cystic carcinoma (ACC) are uncommon tumors, representing about 10% to 15% of head and neck tumors. We compare the survival and control rates at our institution with those reported in the literature, and examine putative predictors of outcome. All patients registered with the tumor registry as having had ACC were identified. Demographic and survival variables were retrieved from the database. Additionally, a chart review of all patients was done to obtain specific information. Minor gland tumors were staged using the American Joint Committee on Cancer's criteria for squamous cell carcinomas in identical sites. Histopathologic variables retrieved included grade of the tumor, margins, and perineural invasion. Treatment modalities, field sizes, and radiation doses were recorded in applicable cases. An effort to retrieve archival tumor specimens for immunohistochemical analysis was undertaken. A total of 69 patients were treated for ACC from 1955 to 1999. One patient, who presented with fatal brain metastasis, was excluded from further analysis. Of the remaining 68 patients, 30 were men and 38 were women. The average age at diagnosis was 52 years, and mean follow-up was 13.2 years. Mean survival was 7.7 years. Overall survival (OS) rates at 5, 10, and 15 years were 72%, 44%, and 34%, and cause-specific survival was 83%, 71%, and 55%, respectively. Recurrence-free survival rates were 65%, 52%, and 30% at 5, 10, and 15 years, with a total of 29 of 68 (43%) eventually suffering a recurrence. Overall survival was adversely affected by advancing T and AJCC stage. Higher tumor grades were also associated with decreased OS, although the numbers compared were small. Primaries of the nasosinal region fared poorly when compared with other locations. Total recurrence-free survival, local and distant recurrence rates were distinctly better in primaries of the oral cavity/oropharynx when compared with those in other locations. Reduced distant recurrence-free survival was significantly associated with increasing stage. No other variables were predictive for recurrence. Additionally, we found that nasosinal tumors were more likely to display higher stage at presentation, and were more often associated with perineural invasion. Also of interest was the association of perineural invasion with margin status, with 15 of 20 patients with positive margins displaying perineural invasion, while only 5 of 17 with negative margins showed nerve invasion (P = 0.02). On immunohistochemistry, 2 cases of the 29 (7%) tumor specimens found displayed HER-2/neu positivity. No correlation between clinical behavior and positive staining could be demonstrated. Our data concur with previous reports on ACC in terms of survival and recurrence statistics. Stage and site of primary were important determinants of outcome. Grade may still serve a role in decision making. We could not demonstrate any differences attributable to primary modality of therapy, perhaps due to the nonrandomization of patients into the various treatment tracks and the inclusion of palliative cases. Similarly, perineural invasion, radiation dose and field size, and HER-2/neu positivity did not prove to be important factors in our experience.     

Primary lymphoepithelioma-like carcinoma of ocular adnexa: clinicopathologic features and treatment

Background and Methods

Lymphoepithelioma-like carcinoma (lelc) is a rare malignancy in ocular adnexa.Here, we report 4 patients with lelc and review 11 patients reported in the literature. Clinical profiles, association with Epstein–Barr virus (ebv), treatment, and outcomes are analyzed.

Results

Lacrimal glands and the lacrimal drainage system, eyelid, and conjunctiva are potential primary sites for lelc. The tumours are characterized histologically by nests of undifferentiated malignant cells surrounded by lymphoid infiltrates. Infection with ebv was confirmed in lelc of ocular adnexa, and that association seemed to be restricted to Asian populations. Results from our centre uniformly showed expression of ebv-encoded small rnas in primary tumour, locally recurrent tumour, and metastatic lymph nodes. This disease had a tendency to relapse regionally. Postoperative radiotherapy seems to improve disease-free survival. Tumours appear to be sensitive to radiotherapy and chemotherapy based on cisplatin and 5-fluorouracil. At our centre, 3 patients were still living at 22, 33, and 76 months after surgery. One patient died of distant metastasis after a survival of 38 months.

Conclusions

Lymphoepithelioma-like carcinoma is a heterogenous entity among ocular adnexal malignancies. Multimodality treatment provides a better chance at survival. Further investigation is required to achieve a better understanding of the biologic behavior of this entity and of its optimal treatment.