Study of extra-nodal lymphoma with special reference to histomorphology, immunohistochemistry, and topographical distribution

Background: Primary extra-nodal lymphomas (ENLs) are rare neoplasm but can affect many organ systems of body. Proper and timely diagnosis and justified treatment of primary ENL are often affected due to variety of lymphoma types and the relative rarity of many of these tumor types. They are mainly of Non-Hodgkin lymphoma which further consists of many subtypes having variation of epidemiology and clinical presentation along with geographic distribution. Aim and Objectives: Aim and objectives of the study were to find out topographical distribution and histological patterns of ENL along with their immunohistochemical character in a tertiary care hospital of Eastern India. Materials and Methods: All the cases from April 2015 to March 2018 diagnosed as ENL by histopathological examination were included in this descriptive cross-sectional study for further immunohistochemical analysis for CD45 Life-cycle assessment (LCA), CD3 and CD 20 to identify the cell of origin (B cell vs. T cell). Results: Out of total 31 cases, 67.74% were male and the most common age group was 51–60 years. Gastrointestinal tract was the most common organ system (61.29% of cases) affected and stomach (29.03%) followed by Ileum (9.67%) were the most common sites affected by ENL. Histomorphologically diffuse large cell lymphoma was the most common pattern while immunohistochemistry study revealed all the cases were B cell type with LCA and CD 20 positive and CD3 negative. Conclusion: This study revealed the topographical pattern along with predominant histomorphological and immunohistochemical character of ENL in study population. However, further studies with meta- analysis can enlighten these characters more comprehensively.

CT and MRI in the diagnosis of extra-nodal nasal type NK/T cell lymphoma originating in the nasal cavity or sinus / 医学研究生学报

Objective Early diagnosis of extra-nodal nasal type NK /T cell lymphoma (ENKTCL) is difficult and it is often misdiagnosed.This article was to analyze the CT and MRI findings of ENKTCL originating in the nasal cavity or sinus and investigate the values of CT and MRI in the diagnosis of the disease . Methods We retrospectively analyzed the clinical data and CT and MRI findings about 13 cases of ENKTCL originating in the nasal cavity or sinus examined in our hospital from January 2012 to December 2017. Results Non-contrast CT scanning displayed soft tissue density shadow in the nasal cavity or sinus , with homogeneous density in the tumor and soft tissue mass with bony destruction ( mostly infiltrative absorption) in the midline nasal cavity and sinal area , and the area of soft tissue lesion was obviously larger than that of bony de -struction.Ten cases of contrast-enhanced CT of the tumor manifested mild to moderate heterogeneous density ; 10 cases of plain MRI pres-ented isointensity of T1WI and slightly increased intensity of T 2WI, while enhanced MRI showed mild to moderate heterogeneous density . Low-intensity T1WI, high-intensity T2WI and edge-enhancement were observed in the 10 cases accompanied by obstructive sinusitis . Conclusion ENKTCL originating in the nasal cavity or sinus has its own specific CT or /and MRI manifestations.Conventional CT or /and MRI combined with clinical signs and symptoms can accurately determine the site of lesion and scope of invasion and is therefore of important clinical significance for the early diagnosis and prognosis of the disease.

PRDM1 expression and its relationship with PI3K/AKT pathway activation in extranodal NK/T cell lymphoma-nasal type / 中华血液学杂志

Objective: To investigate the expression of PRDM1 and its relationship with PI3K/AKT pathway activation in extranodal NK/T cell lymphoma-nasal type. Methods: Immunocytochemistry and Western blot were used to detect the expression of PRDM1 and p-AKT in 10 EN-NK/T-NT tissue or 3 cell lines (PRDM1-positive YT cell line, PRDM1-negative NKL and NK92 cell lines). Nanostring gene expression profiling technique was used to detect the activation of the PI3K/AKT pathway in normal nasal mucosa, PRDM1-negative and positive EN-NK/T-NT tissue. MTS was used to detect cell proliferation, and flow cytometry was used to detect cell cycle and apoptosis. Results: Nanostring gene expression profiling revealed that genes associated with PI3K/AKT signaling pathway (eg, IL-7, BRCA1, ITGA8, IL2RB, FASLG, CDK2, COL27A1, CSF3R, KITLG and IL-6) were highly expressed in EN-NK/T-NT cases (P<0.05). Also, we found that p-AKT was highly expressed in YT cell line, but lower or not expressed in NK92 and NKL cells. In addition, LY294002, a PI3K/AKT pathway inhibitor, increased PRDM1 and PTEN expression in a dose dependent manner in YT cells. More importantly, YT cell were treated with 20 μmol/L LY294002 48 h, the proliferation rate was significantly decreasing (58.18% vs 100.00%, t=12.770, P=0.006), and the proportion of cells in G(1) phase was significantly increased (30.05% vs 76.93%, t=11.570, P<0.001). However, there was no significant difference in cell proliferation and cell cycle between NKL cells and control group (P>0.05). Conclusion: The activation of PI3K/AKT pathway is positive associated with the expression of PRDM1 in EN-NK/T-NT, and inhibition of PI3K/AKT pathway may have significant therapeutic potential for PRDM1-positive EN-NK/T-NT.

PRDM1 expression and its relationship with PI3K/AKT pathway activation in extranodal NK/T cell lymphoma-nasal type / 中华血液学杂志

Objective@#To investigate the expression of PRDM1 and its relationship with PI3K/AKT pathway activation in extranodal NK/T cell lymphoma-nasal type.@*Methods@#Immunocytochemistry and Western blot were used to detect the expression of PRDM1 and p-AKT in 10 EN-NK/T-NT tissue or 3 cell lines (PRDM1-positive YT cell line, PRDM1-negative NKL and NK92 cell lines). Nanostring gene expression profiling technique was used to detect the activation of the PI3K/AKT pathway in normal nasal mucosa, PRDM1-negative and positive EN-NK/T-NT tissue. MTS was used to detect cell proliferation, and flow cytometry was used to detect cell cycle and apoptosis.@*Results@#Nanostring gene expression profiling revealed that genes associated with PI3K/AKT signaling pathway (eg, IL-7, BRCA1, ITGA8, IL2RB, FASLG, CDK2, COL27A1, CSF3R, KITLG and IL-6) were highly expressed in EN-NK/T-NT cases (P<0.05). Also, we found that p-AKT was highly expressed in YT cell line, but lower or not expressed in NK92 and NKL cells. In addition, LY294002, a PI3K/AKT pathway inhibitor, increased PRDM1 and PTEN expression in a dose dependent manner in YT cells. More importantly, YT cell were treated with 20 μmol/L LY294002 48 h, the proliferation rate was significantly decreasing (58.18% vs 100.00%, t=12.770, P=0.006), and the proportion of cells in G1 phase was significantly increased (30.05% vs 76.93%, t=11.570, P<0.001). However, there was no significant difference in cell proliferation and cell cycle between NKL cells and control group (P>0.05).@*Conclusion@#The activation of PI3K/AKT pathway is positive associated with the expression of PRDM1 in EN-NK/T-NT, and inhibition of PI3K/AKT pathway may have significant therapeutic potential for PRDM1-positive EN-NK/T-NT.

Clinical features and prognosis analysis of 15 cases of extra-nodal nasal-type natural killer/T-cell lymphoma originated from the larynx / 中华放射肿瘤学杂志

Objective To analyze the clinical features and prognosis of extra-nodal nasal NK/T cell lymphoma originated from the larynx. Methods Clinical data of 15 cases of extra-nodal nasal NK/T-cell lymphoma originated from the larynx were retrospectively analyzed. The overall survival ( OS ) and progression-free survival ( PFS) were calculated by Kaplan-Meier survival analysis. The effect of different clinical factors on the clinical prognosis was assessed by univariate analysis. Results Among 15 patients,13 cases were male and 2 female. The median age of onset was 40 years. In 8 cases,the lesions were confined to the larynx,and only 4 cases suffered from cervical lymph node involvement. According to Ann Abor staging, 11 cases were classified as grade I,3 as gradeⅡand 1 as gradeⅢ.The median OS was 28. 0 months and the 5-year OS was 32. 0％.The median PFS was 24. 7 months and the 5-year PFS was 33. 3％.Among 14 patients with stage Ⅰ/Ⅱ,the clinical prognosis of patients receiving combined chemo-radiotherapy was significantly better than those of their counterparts undergoing radiotherapy or chemotherapy alone ( median OS:37. 2 vs. 11. 2 vs.3. 7 months,P=0. 004) . Conclusion Extra-nodal nasal NK/T cell lymphoma originated from the larynx is extremely rare, predominantly in middle-aged male patients. The general condition is relatively favorable. Patients present with multiple lesions in the early stage and relatively poor prognosis. The clinical efficacy of chemotherapy combined with radiotherapy is probably higher compared with that of radiotherapy or chemotherapy alone.

Lymphoma of the thyroid gland: a clinicopathologic study over a period of five years in a tertiary care center.

Background: Primary Thyroid Lymphoma (PTL) is uncommon, accounting for only 5% of thyroid neoplasms and less than 2.5 to 7% of extranodal lymphomas. The study aims to analyze the histopathological findings and to correlate it with clinical features. Methods: This study includes cases of PTL received in the department of pathology in a tertiary care center, Kerala, south India over a period of 5 years. Patient details and follow up data were obtained by communication with treating doctors and reviewing the hospital records. All cases had a minimum follow up of 6 months. Results: 15 cases of PTL were diagnosed over the five year period. In total 72 cases of extranodal lymphomas were diagnosed, thus forming 20.83% of extra nodal lymphomas. Lymphocytic thyroiditis was present in 93.3%. Most of the cases were Extranodal Marginal Zone B Cell Lymphoma (EMZBCL) and Diffuse Large B Cell Lymphoma (DLBCL). Conclusion: It is important to consider the diagnosis of primary thyroid lymphoma in patients presenting with an enlarging neck mass especially with the history of Hashimoto’s thyroiditis. Random microscopic foci of DLBCL or small areas of MZBL could be overlooked examination or missed with limited sampling. The distinction between MZBL and DLBCL in the thyroid is clinically significant.

A Case of Recurred Follicular Lymphoma in Sublingual Gland after Complete Remission / 대한이비인후과학회지

Non-Hodgkin's lymphoma (NHL) is a group of neoplasm affecting the lymphoid system. Follicular lymphoma is typically a slow-growing, indolent form of NHL that accounts for 1/3 of all NHL. It is one of the B-cell lymphomas, generally characterized by multiple relapses after responses to a variety of therapies. When lymphoma relapses, it may occur in the same or a different part of the body from where it occurred originally. Recurrence in the primary site is most common but recurrence in other parts is rarely observed. We experienced a rare case of 70-year-old woman who complained of swelling in the left mouth floor and was diagonosed with a recurrent NHL. It recurred in the sublingual gland after a complete remission of the follicular lymphoma.

Lymphomas of the gastro-intestinal tract - Pathophysiology, pathology, and differential diagnosis.

The gastrointestinal tract (GIT) is the most commonly involved site of extranodal lymphomas. The close association between chronic inflammation and specific GIT lymphomas not only provide interesting insights into the pathobiology of lymphomas but also poses unique diagnostic challenges. A clear understanding of marginal zone and mucosa associated lymphoid tissue (MALT) in health and disease is helpful to place GIT lymphomas in proper context. A wide variety of lymphomas besides MALT lymphomas occur in various parts of the GIT. The characteristic pathological, immunophenotypic, and genetic features of different GIT lymphomas categorized according to World Health Organization (WHO) classification are presented. The epidemiological, clinical, and pathological features of lymphomas occurring in each part of the GIT are summarized and the key points regarding lymphomas at each site are emphasized. A tabular summary of the important differential diagnostic considerations at each site is given and suggestions for a minimal diagnostic work up are provided.

Primary central nervous system lymphoma: A study of clinicopathological features and trend in western India.

Introduction: Primary central nervous system lymphoma (PCNSL) is rare and accounts for 1-2% of all primary intracranial tumors (ICT). There are conflicting reports regarding the increased incidence of PCNSL over the last two decades in both immunocompromised and immunocompetent patients. Aim: This study was designed to study the clinicopathological characteristics of PCNSL and to access the trend of PCNSL at our institute. Materials and Methods: All the histopathologically proven cases of PCNSL were reviewed from January 1997 to December 2009 (13 years). Immunophenotyping was performed on available paraffin-embedded tissue blocks. Immune status was evaluated and human immunodeficiency virus (HIV) serology was performed in all cases. Cerebrospinal fluid (CSF) findings were recorded whenever available. Possibility of secondary involvement by a systemic lymphoma was excluded in every case. Statistical analysis was done using χ2 -test. Results: During the study period (13 years), a total of 4715 cases of ICT were diagnosed, out of which 66 cases were PCNSL, which accounted for 1.4%. The age ranged from 10 to 75 years with a median age of 46 years. All the patients were immunocompetent. Frontal lobe was the most common site of involvement. Diffuse large B-cell lymphoma was the histological pattern in all the cases. CSF involvement was seen in only one case. Conclusions: In this study, no significant increase in the incidence of PCNSL was found at our institute. Association of PCNSL cases with HIV or acquired immunodeficiency syndrome was not found in our study.

Mainly Adrenal Gland Involving NK/T-Cell Nasal Type Lymphoma Diagnosed with Delay due to Mimicking Adrenal Hemorrhage

A 29-yr-old man, presented with abdominal pain and fever, had an initial computed tomography (CT) scan revealing low attenuation of both adrenal glands. The initial concern was for tuberculous adrenalitis or autoimmune adrenalitis combined with adrenal hemorrhage. The patient started empirical anti-tuberculous medication, but there was no improvement. Enlargement of cervical lymph nodes were developed after that and excisional biopsy of cervical lymph nodes was performed. Pathological finding of excised lymph nodes was compatible to NK/T-cell lymphoma. The patient died due to the progression of the disease even after undergoing therapeutic trials including chemotherapy. Lymphoma mainly involving adrenal gland in the early stage of the disease is rare and the vast majority of cases that have been reported were of B-cell origin. From this case it is suggested that extra-nodal NK/T-cell lymphoma should be considered as a cause of bilateral adrenal masses although it is rare.

Tejido linfoide y linfomas gástricos / Lymphoid tissue and gastric lymphomas

En este artículo hemos realizado una amplia revisión de los linfomas primarios gástricos, su clasificación y aspectos clínico-patológicos más importantes, haciendo énfasis especial en los linfomas MALT o asociados a las mucosas y su relación con la infección por Helicobacter pylori.

In this review we describe various aspects of the primary gastric lymphomas, the classification, and the most important clinico-pathological aspects, with emphasis in mucosa associated lymphoma (MALT) and the Helicobacter pylori infection.

Primary non-hodgkin's lymphoma of bone: Experience of a decade.

Objective: To study the prevalence and characterization of primary non Hodgkin's lymphoma of bone in a major referral center of Pakistan. Methodology: All cases of primary non Hodgkin's lymphoma of bone, diagnosed at a referral center in Pakistan from January 1998 to July 2008 have been included. Patients with diagnosis of lymphoma at some other site prior to diagnosis in bone biopsy were excluded from the study. Results: Sixty cases of primary non Hodgkin's lymphoma of bone were received, with a male to female ratio of 7: 3 (42 males and 18 females). Overall, 52/60 (86.6 %) patients were diagnosed with Diffuse large B-cell non-Hodgkin's lymphoma. The largest fraction of patients was found in the pediatric group i.e. 33/60(55%) followed by elderly group i.e.15/60 (15%) and adults i.e. 12/60 (20%). The commonest sites of involvement were femur (28.3 %), hip bone (16.6%) and humerus (10%). Conclusion: Diffuse large B-cell lymphoma is the commonest type of primary non Hodgkin's lymphoma of bone in the Pakistani population. It is common in all ages. The sites most often involved are femur followed by hip bone. Primary non Hodgkin's lymphoma of bone appears to be more common in males. Our results are comparable to international data with various studies also showing that diffuse large B cell non Hodgkin lymphoma is the commonest primary bone lymphoma. However, primary Bone Lymphoma is more common in children and young adults.