Primary gastric non-Hodgkin lymphomas:Recent advances regarding disease pathogenesis and treatment

Primary gastric lymphomas(PGLs)are distinct lymphoproliferative neoplasms described as heterogeneous entities clinically and molecularly.Their main histological types are diffuse large B-cell lymphoma(DLBCL)or mucosaassociated lymphoma tissue.PGL has been one of the main fields of clinical research of our group in recent years.Although gastric DLBCLs are frequent,sufficient data to guide optimal care are scarce.Until today,a multidisciplinary approach has been applied,including chemotherapy,surgery,radiotherapy or a combination of these treatments.In this minireview article,we provide an overview of the clinical manifestations,diagnosis and staging of these diseases,along with their molecular pathogenesis and the most important related clinical published series.We then discuss the scientific gaps,perils and pitfalls that exist regarding the aforementioned studies,in parallel with the unmet need for future research and comment on the proper methodology for such retrospective studies.Aiming to fill this gap,we retrospectively evaluated the trends in clinical presentation,management and outcome among 165 patients with DLBCL PGL who were seen in our institutions in 1980-2014.The study cohort was divided into two subgroups,comparing the main 2 therapeutic options[cyclophosphamide doxorubicin vincristine prednisone(CHOP)vs rituximab-CHOP(R-CHOP)].A better outcome with immunochemotherapy(R-CHOP)was observed.In the next 2 mo,we will present the update of our study with the same basic conclusion.

Ultrasound features of primary non-Hodgkin’s lymphoma of the palatine tonsil:A case report

BACKGROUND Lymphomas are the second most common malignancy of the head and neck.In this region,the vast majority of extranodal lymphomas are located in the palatine tonsil,accounting for about 51%.Tonsillar lymphomas are aggressive tumors with intermediate-or high-grade histology.We here report a case of primary non-Hodgkin’s lymphoma of the palatine tonsil and analyze its ultrasound features.CASE SUMMARY A 40-year-old man presented with right palatine tonsil swelling for 2 mo after a cold,accompanied by dysphagia,snoring,and suffocation.He had no sore throat,fever,or history of upper respiratory tract infection or tuberculosis.The patient was generally in good health and denied other diseases.He was diagnosed with acute tonsillitis initially and treated with antibiotics for 7 d.However,there was no improvement with the treatment.Tonsil biopsy and ultrasound-guided biopsy of the biggest lymph node of the right neck showed the typical pathology of non-Hodgkin lymphoma.CONCLUSION Primary lymphoma of the tonsils is rare,and its diagnosis is challenging.Ul-trasound is a useful modality in diagnosing oropharyngeal diseases,and can clearly show the features of this tumor,but the final diagnosis should be estab-lished by histology.

Primary Ovarian Non-Hodgkin’s Lymphoma: Retrospective Study of 16 Patients

Background: Primary non-Hodgkin’s lymphoma (PONHL) of the ovary is rare disease. Ovarian involvement by NHL is usually secondary, as a part of systemic disease. It is frequently diagnosed as ovarian carcinoma which causes a significant delay in diagnosis and management. Objective: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Material & methods: This was a retrospective single institutional work that included 16 cases of primary ovarian non-Hodgkin lymphoma (PONHL) treated in National Cancer Institute-Cairo University from January 2010 till January 2015. All available medical data including the clinical and pathological characteristics, treatment, and outcomes of patients with PONHL are analyzed. Results: Data from 16 patients are obtained. The patient’s age ranges from 14 to 55 years (mean 28 years). Ascites is the most common manifestation (75%). Tumor size ranges from 5 to 24 cm (mean 13.1). LDH is elevated in all cases (mean 644 U/L) and CA-125 is elevated in only 4 cases (25%) especially when there is an extensive peritoneal irritation. Ten cases (62.5%) are bilateral with stage IV-E according to the Ann Arbor staging system. The remaining six cases (37.5%) are unilateral with Ann Arbor stage I-E. There are no stage II-E or III-E in the current study. Tumors are classified according to the World Health Organization as follows: diffuse large B-cell lymphoma (10 cases) (62.5%), Burkitt’s lymphoma (5 cases) (31%) and only one case of B-lymphoblastic lymphoma/leukemia. All the tumors are of B-cell lineage and are all CD20 positive. All Burkitt’s lymphoma cases show higher Ki67 index (4 cases are 100% and one is 88%). The case of B-lymphoblastic lymphoma/leukemia is positive for TDT & CD 10. Surgery is the main treatment modality for primary diagnosis and for staging, although chemotherapy should have been the primary treatment because it is one of the most chemosensitive tumors. Follow-up period ranges from 3 months to 5 years (mean 33 months). Ten patients are alive without disease. Two cases experienced relapse and one case died during chemotherapy treatment. The remaining three cases died from other causes than disease. The median overall survival time was not reached yet;however, the mean overall survival was 46.8 monthes;median progression free survival was 36 months. Conclusion: Most patients with PONHL present with symptoms attributable to an ovarian mass which necessitates extensive surgical staging that is not mandatory for lymphomas. More studies will be needed to better define and treat this rare entity.

Primary non-Hodgkin' s lymphoma of the pancreas:report of six cases

Objective To evaluate the diagnosis and treatment of primary non-hodgkin’ s lymphoma (PNHL) of the pancreas. Methods The therapeutic result of 6 cases admitted from January 1980 to March 2000 was reviewed retrospectively. Results The lesion was located in the head of the pancreas in 2 cases and the body and/or tail in 4, respectively. Clinical manifestations included epigastric discomfort, loss of weight, abdominal mass and jaundice. Two cases underwent Whipple’ s procedure and distal pancreatectomy respectively, the other 4 underwent laparotomy and biopsy. B cell non-Hodgkin’ s lymphoma of 5 cases and T cell non-Hodgkin’s lymphoma of one were verified by pathology. One patient lost follow-up, the other 5 patients survived 58, 49, 22, 13 and 4 months respectively. Conclusion The clinical manifestation and radiological features of PNHL of the pancreas are not specific. Comprehensive therapy including surgery is beneficial to the long-term survival of patients with PNHL of the pancreas. 4 refs.

Primary cardiac lymphoma:a case report and review of the literature

Primary cardiac lymphoma (PCL) is an extremely rare neoplasm and usually defined as a non-Hodgkin's lymphoma being exclusively located in the heart and/or the pericardium. It is particularly difficult to diagnose this rare disease due to its nonspecific clinical manifestations. The prognosis of this disease is poor. In this diffuse large B-cell lymphoma, the patient finally died of heart failure after 2 courses of chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone).

Primary hepatic peripheral T-cell lymphoma associated with Epstein-Barr viral infection

Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were significant for elevated total bilirubin, alkaline phosphatase, and liver aminotransferases. Following a liver biopsy, histopathology revealed several large dense clusters of atypical T-lymphocytes which were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+ by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. The above histological profile was consistent with peripheral T-cell lymphoma of the liver. Epstein-Barr viral serology indicated a remote infection, a likely risk factor for PTCL. Bone marrow biopsy was negative for malignancy, further supporting hepatic origin.

Primary spinal epidural non-Hodgkin’s lymphoma presentedwith spinal cord compression syndrome

The spinal epidural space is an uncommon presenting site in primary non-Hodgkin’s lymphomas,especially for children.A boy suffered spinal cord compression syndrome caused by primary spinal epidural non-Hodgkin’s lymphoma.Thoracolumbar magnetic reso-nance imaging(MRI)demonstrated an intraspinal mass.An operation was performed with gross total tumor removal.Histological examination revealed a non-Hodgkin’s B-cell lymphoma.Bone marrow aspiration was negative for lymphoma involvement.No other therapies(chemotherapy and/or radiotherapy)were per-formed according to the parents’opinion.The patient died approximately one year after the operation due to brain metastases.The clinical course and imaging features were discussed with a review of literatures.

原发女性生殖系统淋巴瘤临床分析

目的探讨原发性女性生殖系统淋巴瘤(primary female genital system lymphoma,PFGSL)的临床和病理特征,分析诊断治疗方法及影响预后的因素。方法回顾性收集2010年1月至2020年12月首都医科大学附属北京妇产医院初诊8例PFGSL患者的临床资料并随访。结果患者平均发病年龄(54.3±12.6)岁,其中原发于卵巢4例,宫体2例,宫颈1例,外阴1例。主要临床表现为绝经后阴道出血、腹胀、腹水及盆腔肿物。病理类型均为B细胞来源的非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL),免疫组织化学中CD20均呈阳性,Ki67较高。II EA期患者4例,IV A期患者3例,IV B期患者1例。6例患者依据手术后病理诊断,2例患者行肿物活检诊断;5例患者接受了以CHOP方案为主的化学药物治疗(以下简称化疗),其中1例进行了自体干细胞移植。1例失访,2例死亡,5例生存(其中4例无瘤生存)。结论PFGSL发病率低,临床表现无特异性,与原发妇科恶性肿瘤容易混淆。当肿物呈乳白色细腻样或反复抽取腹水无法找到癌细胞时需警惕PFGSL。活检或手术切除肿物可获得病理明确诊断,CHOP为主的化疗方案是其主要治疗手段。

女性原发性生殖系统非霍奇金淋巴瘤的临床特征分析

目的探讨女性原发性生殖系统非霍奇金淋巴瘤的临床特征。方法回顾性分析解放军总医院2010年1月-2016年1月确诊并住院治疗的19例女性原发性生殖系统非霍奇金淋巴瘤患者的临床病例。结果19例患者中以年龄<60岁人群为主,初诊时多未见贫血发生,月经状态无特殊,超过70%的患者无典型症状且无大型肿物,Ann Arbor分期主要为ⅣE期,检出12例;原发或受侵部位以卵巢为主,检出7例;弥漫大B细胞淋巴瘤是其主要病理类型,检出13例;11例患者国际预后指数(International Prognstic Index,IPI)评分多为中高危。诊断方法中,13例患者为手术确诊,6例通过超声引导下穿刺活检确诊。采用的主要治疗方案包括R-CHOP或CHOP、EPOCH、MINE、ESHAP等,其中7例同时给予阿糖胞苷+甲氨喋呤+地塞米松鞘内注射预防治疗;全部患者均接受3~20个疗程化疗,其中9例完全缓解,7例部分缓解,3例病情进展。5例患者化疗后接受放射治疗,1例接受内照射治疗。随访时间为7~60个月(5年),中位随访时间为20.5个月,19例患者1年、3年和5年总生存率分别为89.47%、70.59%和58.33%。19例患者在治疗期间2例死亡,死亡原因为感染、中枢神经系统进展;5年总死亡12例。结论女性原发性生殖系统非霍奇金淋巴瘤较罕见,发病后无典型症状,确诊时多为晚期,手术、化疗、放疗是其主要治疗手段,5年内生存率相对较低,临床应重视该疾病的早期诊断与治疗,以改善患者预后、延长生存时间、提高生存率。