Ocular adnexal lymphoma: Five case reports and a literature review

This article reports the clinical course and treatment of ocular adnexal lymphoma based on a retrospective review of five cases with a histologically approved ocular adnexal lymphoma at Kaohsiung Veterans General Hospital over 10 years. Extranodal B-cell lymphoma in the orbit, lacrimal gland, eyelid, or conjunctiva was found in these patients. Four of them were female, and they were aged 45–64 years. All patients were also consulted with hematologists for possible systemic involvement and therapeutic plan. The patient with retrobulbar and orbital apex involvement received systemic chemotherapy. The patient with lacrimal gland involvement experienced tumor recurrence after local excision, and therefore received adjuvant radiotherapy. The remaining three patients had localized lymphoma on the eyelid or bulbar conjunctiva, and they all showed no recurrence after surgical excision. The incidence of ocular adnexal lymphoma has risen worldwide over the last few decades. Although most cases are confined to ocular adnexal, some may also be associated with disseminated lymphoma. Accurate diagnosis and staging is mandatory for appropriate treatment. Generally speaking, localized and low-grade ocular adnexal lymphoma involved eyelid or conjunctiva seem to have good outcome after surgical excision only. Systemic chemotherapy should be considered in patients with advanced disease or systemic manifestations, and radiotherapy also offers a good choice for lacrimal gland lymphoma.     

Hodgkin's Disease Involving Waldeyer's Ring: A Study of Four Cases

We report four cases of Hodgkin's disease who presented with involvement of Waldeyer's ring. Their clinical, morphological and immunohistochemical features are discussed. The patients were immuno-competent, were between 17 and 65 years of age and presented with symptoms related to swelling in the nasopharynx or oropharynx and cervical lymphadenopathy. The nasopharyngeal biopsy and the cervical lymph node in all four cases showed features of classical Hodgkin's disease. The Reed Sternberg cells expressed CD15 and CD30, and in three cases, CD20 and Epstein-Barr virus-latent membrane protein-1 (EBV-LMP-1). Extranodal involvement by Hodgkin's disease which is not in continuity with nodal disease is rare in immunocompetent patients. Morphologically, such extranodal lesions, especially in locations like the oropharynx and nasopharynx, should be differentiated from EBV-associated lymphoproliferations.     

结外鼻型NK/T细胞淋巴瘤117例临床病理分析

 目的　分析结外鼻型NK／T细胞淋巴瘤（NKTL）的临床病理特点,探讨其与疾病预后的关系,为临床个体化治疗提供依据。方法　回顾117例确诊为NKTL患者的病理临床资料。病理诊断均采用常规组织学、免疫组织化学、T细胞重排和原位杂交方法检测EB病毒（EBV）编码小RNA（EBER）。EBV DNA拷贝数检测采用PCR方法。所有患者经过国际预后指数（IPI）和Ki-67等指标临床评估后均采用化疗和放疗联合治疗。随访患者并对临床指标与2年总生存（OS）率和无进展生存（PFS）率的关系进行单因素分析。结果　免疫组织化学结果提示各指标阳性率：CD3为90.6 %,CD56为94.0 %,CD45RO为92.9 %,TIA为97.9 %,Granzyme B为97.7 %,EBER为100.0 %。117例患者中位年龄为43.2岁（14～77岁）,原发鼻者95例（81.2 %）,Ki-67平均值为（48.3±2.6）%;原发非鼻者22例（18.8 %）,包括原发咽喉、舌、扁桃体、淋巴结、皮肤、肝、肠、中枢神经系统和睾丸。与原发鼻的NKTL患者相比,原发肝和肠者Ki-67值较高,且Ki-67值大于80 %的NKTL患者均在1年内死亡。Ki-67值为60 %～80 %的45例2年OS率为60.0 %,与Ki-67值为30 %～60 %的33例OS率（86.3 %）和Ki-67值小于30 %的8例OS率（100.0 %）分别相比,差异均有统计学意义（P＝0.047、0.011）。Ki-67值为60 %～80 %者2年PFS率为36.0 %,与Ki-67值为30 %～60 %者PFS率（57.5 %）相比,差异无统计学意义（P＝0.07）,与Ki-67值小于30 %者PFS率（78.0 %）相比,差异有统计学意义（P＝0.02）。CD56阴性的8例CD3表达阳性,TCR重排均为阳性,提示为T细胞来源,原发部位均为鼻部,无全身症状,2年OS率和PFS率分别为100.0 %和70.0 %,高于CD56阳性患者,差异均有统计学意义（P＝0.03、0.02）。13例检测了EBV DNA拷贝数,5例高于正常值者OS率为60.0 %,余8例OS率为100.0 %。结论　除了IPI外,NKTL原发部位和病理特征,尤其是Ki-67、CD56、EBER和EBV DNA拷贝数与其临床预后的关系不容忽视,也是NKTL预后判断的独立因素。     

Initial features and outcome of cutaneous and non-cutaneous primary extranodal follicular lymphoma

Follicular lymphoma (FL), a typically nodal disease, can arise in extranodal sites in about 10% of cases. The present study aimed to analyse the main differential features of patients with primary extranodal FL. Thirty-nine patients with primary extranodal FL were identified from a series of 354 patients with FL diagnosed at a single institution and their main clinicobiological features were analysed. Twenty patients (5·6%) had a primary extranodal non-cutaneous FL, and 19 (5·4%) a cutaneous FL. BCL2(+) and CD10(+) expression and BCL2/IGHJ@ rearrangement were less frequently observed in cutaneous FL. Absence of 'B'-symptoms, early stage, absence of bone marrow involvement and low-risk Follicular Lymphoma International Prognostic Index (FLIPI) were more frequent in extranodal FL. Five-year overall survival (OS) was 100%, 83% and 78% for cutaneous, non-cutaneous and nodal FL, respectively. When stage I patients were analysed separately, no differences were seen in terms of OS. In multivariate analysis, FLIPI was the most important variable to predict outcome. In conclusion, extranodal FLs, particularly cutaneous, have particular clinico-biological features, which differentiate them from nodal cases. Nevertheless, primary site of the disease is not the main issue to predict outcome.     

47例结外NK/T细胞淋巴瘤治疗和预后分析

本研究探讨结外NK/T细胞淋巴瘤的临床特征、治疗和预后情况。对中国人民解放军总医院血液科自1995年10月到2008年12月收治的47例结外NK/T细胞淋巴瘤患者进行回顾性分析。运用Kaplan-Meier方法行生存分析,COX回归分析模型对预后进行多变量分析。采用的临床参数分别为CD56、Ann Arbor分期、国际预后指标和B组症状。结果表明,全组2年、5年总生存(OS)率分别为91%和71%。单纯放疗患者的累积生存率较单纯化疗患者明显提高,二者相比有统计学差异;而放疗与放化疗结合治疗之间无统计学差异。COX回归分析模型多变量分析显示,CD56、Ann Arbor分期是独立的预后因素;单纯化疗中使用CHOP方案治疗的患者以分期为单因素分析显示,分期≥ⅢE期为预后的因素之一;以B症状为单因素分析3种疗法的生存曲线显示,B症状亦为预后因素之一。结论:单纯放疗的近期疗效明显优于单纯化疗,放化疗结合对近期生存率没有显著提高;CD56阴性和Ann Arbor分期较早是患者预后良好的指标,改善病人的一般状况可以使患者的预后更佳。     

Extranodal spread in the neck: MRI detection on the basis of pixel-based time-signal intensity curve analysis

Purpose

We evaluated dynamic contrast‐enhanced (DCE) magnetic resonance imaging (MRI) for the preoperative detection of extranodal spread (ENS) in metastatic nodes in the neck.

Materials and Methods

The time‐signal intensity curve (TIC) profiles of 54 histologically proven metastatic nodes (26 ENS‐positive and 28 ENS‐negative) from 43 patients with head and neck squamous cell carcinoma (SCC) were retrospectively analyzed to determine the effective TIC criteria for ENS‐positive nodes. The TICs were semiautomatically classified into four distinctive patterns (flat, slow uptake, rapid uptake with low washout ratio, and rapid uptake with high washout ratio) on a pixel‐by‐pixel basis.

Results

A number of the MRI findings were significantly correlated with ENS. However, multivariate logistic regression analysis revealed that only a short‐axis diameter and an area with slow uptake TIC patterns were significantly and independently indicative of the presence of ENS. The combined MRI criteria of nodal size (>25 mm) or TIC profile (>44% nodal areas with slow‐uptake TIC patterns) yielded the best results for differentiation between ENS‐positive and ENS‐negative nodes, providing 96% sensitivity, 100% specificity, 98% accuracy, and 100% positive, and 97% negative predictive values.

Conclusion

When combined with size criteria, pixel‐based MR factor analysis may be a promising tool for detecting ENS. J. Magn. Reson. Imaging 2011;33:830–838. © 2011 Wiley‐Liss, Inc.     

Diagnostic accuracy of PET/CT in patients with extranodal marginal zone MALT lymphoma

BACKGROUND: (18)Fluoro-2-deoxyglucose ((18)FDG) positron emission tomography (PET) is widely used for initial staging and follow-up in patients with malignant lymphoma. While earlier studies suggested a limited role for PET in extranodal marginal zone mucosa-associated lymphoid tissue (MALT) lymphoma patients due to their non-FDG avidity, more recent reports have suggested that the issue is controversial. In the present study, we evaluated the diagnostic accuracy of PET integrated with CT (PETCT) in patients with MALT lymphoma and assessed its reliability in clinical staging and monitoring response. METHODS: Thirty-three patients with biopsy proven MALT lymphoma in 37 sites, who underwent PET/CT at diagnosis, were enrolled. Medical records, PET/CT findings and data obtained by other diagnostic procedures were reviewed. RESULTS: Common sites of MALT lymphoma were the stomach (18), lung (5), orbit (4), and parotid gland (3). PET/CT detected active disease in 18 of 33 patients (54.5%) at diagnosis. Sensitivity in gastric MALT (38.9%) was lower when compared with non-gastric MALT (75%). PET/CT detected active disease in 100% patients with advanced disease (stage III-IV) but only in 42.3% with early stage disease (I-II). The incidence of gastric FDG uptake was higher in patients showing gastric ulcer on gastroscopy than in subjects with minimal or no macroscopic findings. Of the 33 patients in the study cohort, 12 had a follow-up PET/CT which detected relapse in three patients. CONCLUSIONS: These data suggest that PET/CT is a useful tool for both, initial staging and follow-up after therapy in patients with MALT lymphoma. Its sensitivity depends on disease location and stage at initial diagnosis.     

淋巴结外Rosai-Dorfman病7例临床病理分析

目的探讨淋巴结外Rosai-Dorfman病(Rosai-Dorfman disease,RDD)的临床病理特征、诊断及鉴别诊断。方法对7例结外RDD患者进行HE染色和免疫组化检查,并复习相关文献。结果 7例结外RDD患者中男性4例,女性3例,年龄21～57岁(平均41岁),4例位于中枢神经系统,胫骨、甲状软骨和皮下组织各1例,均为结外单发病变。组织学改变:病变组织在低倍镜下呈大小不一的不规则结节样结构,结节内"明暗"相间。高倍镜下"暗区"为浸润的淋巴细胞、浆细胞等;"明区"是散在、成簇或片状分布的组织细胞。后者体积大,胞质丰富,泡状核,可见小核仁。部分组织细胞胞质内见吞噬完整的淋巴细胞和浆细胞等("伸入"现象)。免疫表型:组织细胞S-100蛋白和CD68均阳性,CD1a阴性。结论结外RDD少见,因组织细胞吞噬现象不明显且临床及影像学特征缺乏特异性,易被误诊。结节状分布的结构特点以及低倍镜下明暗相间的组织学特征是诊断RDD的重要线索。免疫组化标记有助于明确诊断。     

An unusual extranodal natural killer/T-cell lymphoma presenting as chronic laryngitis: A case report

Rationale:Nasal-type, extranodal natural killer (NK)/T-cell lymphoma is a rare lymphoma. The tumor usually shows ulcerative and necrotic lesions in the nasal cavities and sinuses. Tissue involvement outside the nasal cavity is uncommon.Patient concern:We describe a 30-year-old man with a 2-month history of hoarseness, weight loss, and dyspnea.Diagnosis:Magnetic resonance image (MRI) showed edema of the larynx with obliteration of the airway. Laryngoscopic examination described necrotic tissue in the glottis and larynx. The biopsy showed chronic, necrotizing laryngitis, with no granulomas, vasculitis, or atypical cells. The immunologic and microbiologic study was negative. Later, after immunosuppressive therapy, the patient presented erythema and diffuse enlargement of the right arm. MRI showed myositis of the biceps and brachial muscles. Infection was rule out, and direct microscopy showed an extensive muscle infiltration by mononuclear cells and abundant mitosis. Immunohistochemistry was positive for CD3, CD8, Ki 67 (90%), and CD56 compatible with extranodal NK/T cell lymphoma.Interventions:The patient initially received immunosuppression treatments (corticoids, cyclofosfamide, and Rituximab) with relapsing episodes. When lymphoma was diagnosed, chemotherapy was started.Outcomes:The patient died during chemotherapy.Lessons:Nasal-type, extranodal NK/T-cell lymphoma should be suspected even when there are no classical findings of neoplasms on histology. Immunohistochemistry is mandatory to rule it out.     

Chronic inflammatory disease, lymphoid tissue neogenesis and extranodal marginal zone B-cell lymphomas

Chronic autoimmune or pathogen-induced immune reactions resulting in lymphoid neogenesis are associated with development of malignant lymphomas, mostly extranodal marginal zone B-cell lymphomas (MZBCLs). In this review we address (i) chemokines and adhesion molecules involved in lymphoid neogenesis; (ii) the autoimmune diseases and pathogens which are associated with development of B-cell lymphomas; (iii) the molecular mechanisms involved in the initiation and progression of MZBCL; and (iv) ‘potential’ mouse models for MZBCL.