Primary early-stage intestinal and colonic non-Hodgkin's lymphoma: Clinical features, management, and outcome of 37 patients

AIM： To analyze the clinical features, management, and outcome of treatment of patients with primary intestinal and colonic non-Hodgkin＇s lymphoma （PICL）. METHODS： A retrospective study was performed in 37 patients with early-stage PICL who were treated in our hospital from 1958 to 1998. Their clinical features, management, and outcome were assessed. Prognostic factors for survival were analyzed by univariate analysis using the Kaplan-Meier product-limit method and log-rank test. RESULTS： Twenty-five patients presented with Ann Arbor stage Ⅰ PICL and 12 with Ann Arbor stage Ⅱ PICL. Thirty-five patients underwent surgery （including 31 with complete resection）, 22 received postoperative chemotherapy or radiotherapy or both. Two patients with rectal tumors underwent biopsy and chemotherapy with or without radiotherapy. The 5- and 10-year overall survival （OS） rates were 51.9% and 44.5%. The corresponding diseasefree survival （DIS） rates were 42.4% and 37.7%. In univariate analysis, multiple-modality treatment was associated with a better DFS rate compared to single treatment （P= 0.001）. While age, tumor size, tumor site, stage, histology, or extent of surgery were not associated with OS and DFS, use of adjuvant chemotherapy significantly improved DFS （P = 0.031） for the 31 patients who underwent complete resection. Additional radiotherapy combined with chemotherapy led to a longer survival than chemotherapy alone in six patients with gross residual disease after surgery or biopsy.CONCLUSION： Combined surgery and chemotherapy is recommended for treatment of patients with PICL, Additional radiotherapy is needed to improve the outcome of patients who have gross residual disease after surgery.

Potential model for differential diagnosis between Crohn's disease and primary intestinal lymphoma

AIM To evaluate the usefulness of different parameters to differentiate Crohn's disease(CD) from primary intestinal lymphoma(PIL).METHODS The medical records of 85 patients with CD and 56 patients with PIL were reviewed retrospectively. Demographic, clinical, laboratory, endoscopic, and computed tomographic enterography(CTE) parameters were collected. The univariate value of each parameter was analyzed. A differentiation model was established by pooling all the valuable parameters. Diagnostic efficacy was analyzed, and a receiver operating characteristic(ROC) curve was plotted.RESULTS The demographic and clinical parameters that showed significant values for differentiating CD from PIL included age of onset, symptom duration, presence of diarrhea, abdominal mass, and perianal lesions(P < 0.05). Elevated lactate dehydrogenase and serum β2-microglobulin levels suggested a PIL diagnosis(P < 0.05). The endoscopic parameters that showed significant values for differentiating CD from PIL included multiple-site lesions, longitudinal ulcer, irregular ulcer,and intraluminal proliferative mass(P < 0.05). The CTE parameters that were useful in the identification of the two conditions included involvement of ≤ 3 segments, circular thickening of the bowel wall, wall thickness > 8 mm, aneurysmal dilation, stricture with proximal dilation, "comb sign", mass showing the "sandwich sign", and intussusceptions(P < 0.05). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of the differentiation model were 91.8%, 96.4%, 93.6%, 97.5%, and 88.5%, respectively. The cutoff value was 0.5. The area under the ROC curve was 0.989.CONCLUSION The differentiation model that integrated the various parameters together may yield a high diagnostic efficacy in the differential diagnosis between CD and PIL.

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.

Diagnostic classification of endosonography for differentiating colorectal ulcerative diseases: A new statistical method

AIM To establish a classification method for differential diagnosis of colorectal ulcerative diseases, especially Crohn's disease(CD), primary intestinal lymphoma(PIL) and intestinal tuberculosis(ITB).METHODS We searched the in-patient medical record database for confirmed cases of CD, PIL and ITB from 2008 to 2015 at our center, collected data on endoscopic ultrasound(EUS) from randomly-chosen patients who formed the training set, conducted univariate logistic regression analysis to summarize EUS features of CD, PIL and ITB, and created a diagnostic classification method. All cases found to have colorectal ulcers using EUS were obtained from the endoscopy database and formed the test set. We then removed the cases which were easily diagnosed, and the remaining cases formed the perplexing test set. We re-diagnosed the cases in the three sets using the classification method, determined EUS diagnostic accuracies, and adjusted the classification accordingly. Finally, the re-diagnosing and accuracy-calculating steps were repeated.RESULTS In total, 272 CD, 60 PIL and 39 ITB cases were diagnosed from 2008 to 2015 based on the in-patient database, and 200 CD, 30 PIL and 20 ITB cases were randomly chosen to form the training set. The EUS features were summarized as follows: CD: Thickened submucosa with a slightly high echo level and visible layer; PIL: Absent layer and diffuse hypoechoic mass; and ITB: Thickened mucosa with a high or slightly high echo level and visible layer. The test set consisted of 77 CD, 30 PIL, 23 ITB and 140 cases of other diseases obtained from the endoscopy database. Seventy-four cases were excluded to form the perplexing test set. After adjustment of the classification, EUS diagnostic accuracies for CD, PIL and ITB were 83.6%(209/250), 97.2%(243/250) and 85.6%(214/250) in the training set, were 89.3%(241/270), 97.8%(264/270) and 84.1%(227/270) in the test set, and were 86.7%(170/196), 98.0%(192/196) and 85.2%(167/196) in the perplexing set, respectively.CONCLUSION The EUS features of CD, PIL and ITB are different. The diagnostic classification method is reliable in the differential diagnosis of colorectal ulcerative diseases.

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.

以腹水为特点的肠道原发性非霍奇金淋巴瘤1例报道

肠道原发性非霍奇金淋巴瘤(primary intestinal non-Hodgkin’s lymphoma,PINHL)是一种起源于胃肠道黏膜的恶性肿瘤,以腹水为主要表现者临床较为少见。本文将结合临床1病例对PINHL的诊断、治疗、预后等因素进行阐述。

克罗恩病与原发性肠道淋巴瘤的临床分析和比较

目的比较CD与原发性肠道淋巴瘤(PIL)的临床特点,探寻对鉴别诊断有价值的相关指标。方法纳入2010年至2017年在解放军总医院第七医学中心消化内科确诊为CD和PIL的患者共91例,其中CD 76例,PIL 14例,CD继发淋巴瘤1例。回顾性分析纳入患者的病历资料,统计学方法采用t检验、非参数检验或卡方检验。结果CD患者的确诊年龄为(37.7±16.0)岁,小于PIL患者的(52.6±19.6)岁,差异有统计学意义(t=-3.085,P=0.003)。CD患者的病程中位数为36.0个月,长于PIL患者的3.5个月,差异有统计学意义(Z=-3.616,P<0.01)。腹痛、肠外表现(口腔溃疡、关节损伤、结节性红斑和肛周病变等)在CD患者中更常见,差异均有统计学意义(χ2=9.427、5.173,P均<0.05)。CD患者单凭肠镜下活组织检查未能获得确诊;14例PIL患者中,13例经肠镜下活组织检查和免疫组织化学检查确诊,1例经术后确诊;PIL组病理活组织检查确诊率高于CD组,差异有统计学意义(χ2=82.584,P<0.01)。1例患者初期临床诊断为CD,采用英夫利西单克隆抗体治疗5次后继发淋巴瘤。结论年轻、病程长、腹痛、肠外表现等更支持CD诊断,而PIL在肠镜下活组织检查的确诊率较CD高。