Primary Extranodal non-Hodgkin's Lymphoma Research Articles

Clinical characteristics and prognostic analysis of primary extranodal non-Hodgkin lymphoma of the head and neck.

Primary extranodal non-Hodgkin's lymphoma (PE-NHL) of the head and neck is the second common site of extranodal lymphoma, accounting for approximately one-third of all extranodal non-Hodgkin's lymphoma (E-NHL). However, in recent years, large-scale PE-NHL case studies in China and worldwide are rare and not comprehensive enough. This work analyzed the clinical manifestations, pathological features, immunophenotypes and diagnosis of PE-NHL, as well as the factors affecting the treatment and prognosis. A retrospective study was performed on 74 patients who were diagnosed with head and neck PE-NHL and treated for the first time. The clinical manifestations, pathological features, and immunophenotypes were summarized, and the factors related to the treatment and prognosis were analyzed. The most common site of this disease was the Waldeyer's ring, followed by the nasal cavity. Diffuse large B-cell lymphoma was the most common type, followed by extranodal NK T-cell lymphoma nasal type. The 1-year, 2-year, and 5-year progression-free survival (PFS) rates were 76.4%, 67.9%, and 59.3%. The 1-year, 2-year, and 5-year overall survival (OS) rates were 89.4%, 85.6%, and 63.2%. ECOG score ≥ 2, Ann Arbor stage III or IV and IPI risk stratification identifying patients as the high-risk group were independent risk factors affecting the OS of patients with PE-NHL of the head and neck. The most common site of PE-NHL in these Chinese patients was the Waldeyer's ring, but the incidence in the nasal cavity was higher than that reported in Western countries. Radiotherapy combined with chemotherapy had better efficacy than chemotherapy alone, and the prognosis depended on the ECOG score and clinical stage. IPI had a better prognostic value in patients in the high-risk group of head and neck PE-NHL.

A Retrospective Study of 341 Cases of Primary Extranodal Non-Hodgkin Lymphoma of the Head and Neck

Aims: Our goal was to obtain comprehensive and accurate information about primary extranodal non-Hodgkin lymphomas of the head and neck region to contribute to the advancement of medical knowledge in this field. Materiels and methods: We conducted a retrospective study of 341 patients with primary extra-nodal, non-Hodgkin lymphomas of the head and neck, over a period of 7 years from January 2016 to December 2022, in the departments of ENT and Head and Neck Surgery and Hematology, with the help of the department of anatomical pathology of the university hospital Mohammed VI of Marrakech. Results: 341 patients, with primary extra-nodal, non-Hodgkin lymphomas of the head and neck, was included, with an average age of 57 years, and a sex ratio male/female of 2.21 . in more than half of the cases; Waldeyer's ring was concerned; especially palatine tonsils. Type B NHL was the most frequent and involved 308 patients. Diffuse large B-cell lymphoma was the commonly observed histological type, found in 214 patients; followed by a Follicular Lymphoma and Extra-Nodal NK/T‑Cell Lymphoma, Nasal Type. Following the extension workup; patients were all staged according to the ANN ARBOR classification; with a 77.8% of them were localized stages I and II, and of the therapeutically evaluable cases, complete remission was achieved in 112 patients. Conclusion: Our study focused on all extra-nodal localizations of NHL in the head and neck region. Due to the very heterogeneous nature of these tumors, most of the current studies are limited to a specific site of involvement. Nevertheless, our study provides an overall picture; which those specific studies can be based. The management of NHL has evolved considerably in recent years, insisting on the multidisciplinary character to a better management of these patients.

Primary Extranodal Non-Hodgkin Lymphoma of Cheek

Non-Hodgkin’s lymphomas (NHL) comprise a group of extremely diverse lymphoproliferative neoplamas that have a predisposition for extranodal sites. Amongst the extranodal locations rare involvement of oral cavity has been reported and occurrence of these neoplasms on the cheek are thin on the ground. The most commonly encountered is the Diffuse large B-cell lymphoma. The B-cell neoplasms are clonal tumors of mature and immature B cells and the relatedness of B-cell lymphomas to normal B-cell counterparts forms a key aspect in their nomenclature of classification. Diffuse large B-cell lymphoma, not otherwise specified (DLBCL, NOS), consists of medium-sized to large B cells with a diffuse growth pattern. This lesion is a morphologically and molecularly heterogeneous entity which does not meet the diagnostic criteria of any specific large B-cell lymphoma neoplasms. Amongst the cases of DLBCL in n the head and neck region involvement of cheek/ buccal mucosa is scarce.

A An Unusual Presentation of Primary Extra Nodal Non-Hodgkin Lymphoma

Patients presented with soft tissue lesions without any β symptoms can be a case of Primary extranodal Non- non-Hodgkins lymphoma. According to the World Health Organization classification system, there are two types of Lymphoma with various sub-types of Hodgkins Lymphoma and Non-Hodgkin Lymphoma are classified to assess the disease burden Progression and metastasis Imaging modalities are helpful. Commonest site involvement in Non-Hodgkin Lymphoma in the gastrointestinal tract (44%) followed by Head and Neck and may Involve bone (8%) and Central Nervous System 5%. Diffuse large β-Cell Lymphoma is the most common subtype representing 30%-35% of all Non-Hodgkin Lymphoma Cases. Oral and Para oral sites were involved in 2.5% of the cases. A 27-year-old Male noticed a swelling in the flank which was progressively Increasing in size one year back later it was associated with pain which reduced mobility and pain was increased during walking. Due to the advancement of diagnostic techniques, it is easy to get the radiological picture and correlation with clinical presentation and pathological reports, but these facilities are not available at various centers. Usually, patients are present in very advanced stages because of a lack of awareness about the disease, and how to proceed for diagnosis and treatment resulting in poor outcomes for these patients. This presentation and clinical funding are rare in the case of Lymphoma. This case report allows us to think of another diagnosis rather than usual.

"Exploring Diverse Presentations of extra nodal Non-Hodgkin's Lymphoma: A Comprehensive Study of Clinical Staging, Treatment, and Controversies.

Lymphoma involves abnormal lymphoid cell growth, often originating in lymphatic tissue, but in this case series non-Hodking's lymphoma is visible in the extra-nodal region. This research explores different lymphoma cases, focusing on diverse presentations and utilizing modern imaging for evaluation. Four cases are detailed, involving an 18-year-old woman with tonsillar lymphoma, a 69-year-old man with ocular lymphoma, a 38-year-old lady with abdominal lymphoma, and a 30-year-old man with gastric lymphoma. All cases received adjuvant chemotherapy (RCHOP), leading to significant symptom relief after four cycles. The discussion highlights the debate surrounding the classification of primary extranodal non-Hodgkin's lymphoma (NHL), challenging the existing Ann Arbor classification's applicability. A major challenge in managing primary extranodal lymphomas is the absence of specific treatment guidelines. Evaluation criteria, defining complete response as the disappearance of all evidence of the disease, guided the study. In conclusion, this case series underscores the importance of a multidisciplinary approach in diagnosing, staging, and managing non-Hodgkin’s lymphoma. Despite challenges in defining and treating primary extranodal non-Hodgkin’s lymphoma, the study demonstrates the effectiveness of adjuvant chemotherapy in providing relief, as all the cases revealed recovery after 4 continuous cycles of chemotherapy.

Epidemiological data and diagnosis pitfalls in aggressive extranodal non-Hodgkin’s lymphomas: current issues

Introduction. Non-Hodgkin’s lymphoma is a group of malignant tumors that develop from hematopoietic cells located outside the medullary. They are one of the most common forms of hemoblastosis. Non-Hodgkin lymphoma develops in people of all ages. Morbidity of non-Hodgkin lymphoma increases with age reaching its highest level in people over 50 years of age. Material and methods. A study was carried out through a narrative review of the literature in the form of a synthesis article. The article summarized and systematized various primary studies, dedicated to the epidemiological and diagnostic aspects of aggressive extranodal NHL. Results. Aggressive extranodal NHL remains a major problem, with a fairly large increase in incidence globally. This trend is observed in several countries of the world, thus morbidity increases by 3% annually for women and by 4% for men. Globally, aggressive non-Hodgkin’s lymphoma continues to affect the working-age population. Although patients with primary extranodal NHL tend to report to a medical specialist at a lower stage than those with primary ganglion disease, the number of those addressing in advanced stages continues to be increased. Conclusions. Although diagnosing NHL does not involve great impediments, primary care physicians often detect patients in the late stages of the disease either because of delayed referral to the doctor or because of incorrect diagnosis. Despite the development of new methods of diagnosis and treatment, aggressive extranodal NHL continues to be a current problem of clinical medicine and public health, requiring increased managerial and financial efforts.

Primary Extra-Nodal Non-Hodgkin Lymphoma of the Cheek: A Rare Case Report and Literature Review

Non-Hodgkin’s lymphomas are a group of highly diverse lymphoproliferative malignancies and have great tendency to involve in organs and tissues that typically don’t contain lymphoid cells. Primary extra-nodal NHL of the oral cavity are thin on the ground, and cheek location is one of the rarest intraoral sites involved. The majority of oral NHLs reported are of diffuse large B-cell type. The present case highlights one of the scarce forms of primary extra-nodal non-Hodgkin; cheek location reveled by a swelling in the right cheek. Routine laboratory tests were within normal limits and clinical examination showcases painless, poorly limited mass. CT and MRI were performed revealing an enhanced tissue lesion with muscular infiltration and no bone lysis. An initial biopsy of the lesion was determined insignificant. A resection biopsy was evaluated using histologic techniques and immunohistochemistry, which established the proper diagnosis of extra-nodal diffuse large B-cell lymphoma. In view of their malignant nature and the fact that only histopathological examination can define the diagnosis, we report this case to aid an early recognition, diagnosis, and treatment, that are essential for a patient’s survival.

Analysis of the Clinicopathologic Characteristics and Prognosis of Head and Neck Lymphoma.

Statistical reports on non-Hodgkin's lymphoma (NHL) of the head and neck combining clinical medicine with pathology are rare. To provide a basis for prognosis prediction and individualized treatment, we will investigate the clinicopathologic characteristics and prognosis of lymphoma in the head and neck region. Four hundred sixty-one patients with NHL in the head and neck region diagnosed through histological biopsy were retrospectively analyzed. Fluorescence in situ hybridization (FISH) and immunohistochemistry (IHC) were performed in all cases to evaluate the genetic status and protein expression levels. Patients were followed up by telephone. The prevalence rate of primary extranodal NHL (PENHL) in the head and neck region was 44.62% (166/372). The incidence of extranodal lymphoma accounted for 36.66% (169/461) of all head and neck lymphomas. Among the cases of PENHL of the head and neck, diffuse large B-cell lymphoma (DLBCL) (60/76, 78.95%) and extranodal NK/T-cell lymphoma, nasal type (ENKTCL) (21/24, 87.5%) were the most common subtypes originating from B-cell lymphoma (BCL) and T-cell lymphoma (TCL), respectively. The most common sites of nodal and extranodal onset were neck lymph nodes and the gastrointestinal tract, respectively. The most common and primary locations of BCL and TCL were the tonsils and nasal cavity, respectively. The 3-year survival rates of PENHL, ENKTCL, and DLBCL of the head and neck were 42%, 28.57%, and 41.67%, respectively, and the 5-year survival rates were 24%, 19.05%, and 20%, respectively. Survival analysis showed that male sex was a risk factor (HR = 5.421; 95% CI, 1.164-25.267; p < 0.05) and that comprehensive treatment was a protective factor (HR = 0.117; 95% CI, 0.025-0.545; p < 0.05) against extranodal DLBCL in the head and neck region. Bone marrow involvement was a risk factor for PENHL of the head and neck (HR = 5.072; 95% CI, 1.17-21.991; p < 0.05). The purpose of this review is to show that PENHL of the head and neck with high incidence deserves more attention, and a model of multidisciplinary diagnosis and treatment should be adopted.

Diagnostic Interval in Extranodal Non-Hodgkin Head and Neck Lymphomas.

The aim of this study is to measure the diagnostic interval (DI) of primary extranodal non-Hodgkin lymphomas (PE-NHL) affecting the head and neck and to discover any associated factors. With this aim, we performed a retrospective observational study in northwestern Spain on patients diagnosed between 1 January 2005 and 1 January 2016. A search was made across the electronic health records of the public health system of this region (SERGAS). DI was used as the dependent variable, and different clinicopathological data of the corresponding patients and tumors were analyzed as exposure variables. PE-NHLs were mostly located in Waldeyer’s ring, and they presented a B phenotype and had a median DI of 65 days. Shorter diagnostic intervals were observed in (1) PE-NHL patients who had comorbidities (p = 0.02), (2) PE-NHL that caused symptoms of dysphagia (p = 0.04), (3) tumors with the highest proliferative activity (Ki67 > 80%) (p = 0.04), and (4) tumors diagnosed in the advanced stages of the disease (p = 0.004). Univariate analysis revealed a significant association between dysphagia and a shorter DI. We conclude that raising awareness about these neoplasms and warning about the presenting symptoms can contribute to earlier diagnoses of these tumors and to better outcomes.

Primary Extranodal Lymphomas: A Five Year Retrospective Study from a Tertiary Care Cancer Centre, Kerala, India

Introduction: Primary Extranodal Lymphomas (pENLs) are defined as lymphomas with dominant extranodal involvement with no or minor involvement of lymph nodes after routine staging procedures. The present study highlights the profile of pENLs in Kerala, South India which is not yet reported in literature. Aim: To study the frequency, distribution and histopathology profile of pENLs diagnosed in a tertiary care cancer centre. Materials and Methods: This was a cross-sectional study done in a tertiary care cancer centre in Kerala, Southern India for a period of five years. All the cases of pENLs diagnosed during this five year period which fulfilled the inclusion criteria were included in this study. These 3,357 cases were reviewed and classified according to the 2017 revised 4th edition World Health Organisation (WHO) classification of Tumours of Haematopoietic and Lymphoid tissues. Data was summarised using descriptive statistics. The frequency, distribution and histopathology features of pENLs were studied. Results: A total number of 3,357 lymphomas were diagnosed at the centre during this period. Non Hodgkin lymphomas (NHLs) constituted 2610 (78%). pENLs constituted 477 cases which included two cases of classical hodgkin lymphomas. Primary extranodal NHL (n=475) constituted 18.2% of NHL. Diffuse large B-cell lymphoma, Not Otherwise Specified (NOS), (DLBCL, NOS) was the most common histologic subtype (173 cases, 36%) followed by Mucosa Associated Lymphoid Tissue (MALT) lymphoma (77cases, 16%). Most common site of presentation were Gastrointestinal Tract (GIT) (117 cases, 24%) and Head and Neck region (HN) (117 cases, 24%). Conclusion: DLBCL, NOS was the most common subtype. GIT and HN region was the most common site of pENLs in present study. The frequency of pENLs in present study was comparable to few other Indian studies but was lower than Far Eastern studies. The frequency of Adult T-cell leukaemia/ Lymphoma (ATLL) was high which was not mentioned in similar studies on pENLs from India.

EVALUATION OF PROGRAM DEATH 1 (PD-1) AND PD-1 LIGAND (PD-L1) EXPRESSIONS IN HISTOLOGICAL SUBTYPES OF PRIMARY EXTRANODAL NON HODGKIN LYMPHOMA.

Programmed death 1 (PD-1) protein and its ligands, PD-L1 are two proteins that have been widely studied in many solid tumours. However, only limited observations were made in lymphomas, which were mainly Hodgkin lymphomas. We aimed to evaluate the expressions of PD-1 in the tumour microenvironment and PD-L1 in the tumour cells of primary extranodal non-Hodgkin lymphoma (peNHL). A cross-sectional study using 87 archived formalin-fixed paraffin-embedded tissue blocks from patients diagnosed with peNHL. Immunohistochemistry stainings for PD-1 and PD-L1 were done. The protein expressions were evaluated microscopically. The association between expressions of the PD-1 and PD-L1 with the histological subtypes of peNHL were statistically analysed. 46 (52.9%) of the cases had a negative expression of PD-L1 in the tumour cells and 57 (65.5%) had positive PD-1 expression in the tumour microenvironment. Significant associations were found between PD-1 and PD-L1 expressions with subtypes of peNHL (p&lt;0.05). Positive expressions of both proteins were found in diffuse large B cell lymphoma (DLBCL). Our study explored the expressions of PD-1 and PD-L1 in peNHL that has demonstrated a significant association of PD-1 and PD-L1 expressions with subtypes of peNHL, which may provide additional information to the future study of these proteins.

A CASE OF MULTI REGIONAL PRIMARY MUSCLE LYMPHOMA

Primary extranodal non-Hodgkin's lymphoma (eNHL) usually presents at an early stage, as an extranodal organ involvement along with draining lymph nodes only or the predominant site is extranodal. As an eNHL, primary skeletal muscle lymphoma is very rare. The usual clinical picture is local swelling and pain with or without systemic symptoms. MRI features are distinctive and FDG-PET/CT may help to evaluate the stage and monitor the response to the treatment. A 56-year-old male, presented with a one-month history of swelling and pain on his left ankle. There was no history of trauma or any physical strain. A mass lesion was palpated on the calcaneus bone. MRI showed diffuse muscle involvement. The clinical picture was not consistent with infection or hematoma. The blood cell count and biochemical investigations were within normal limits. Serology for hepatitis B, C and HIV were negative. Biopsy was decided. Histological examination revealed CD19, CD20, bcl-2 and bcl-6 positive B-cell lymphoma with a Ki67 proliferation index of 95%. Myc, bcl-2, and bcl-6 gene rearrangements were not detected. Diffuse large B cell lymphoma was diagnosed. FDG-PET/CT showed lesions in multiple regions only limited to skeletal muscles but no other organ involvement. He had no adverse risk factors but bulky lesion (11cm sized lesion). After 6 courses of R-CHOP protocol, he had complete anatomic and metabolic response. Healthy skeletal muscles do not have lymphatic system. Lymphomatous involvement of muscles occurs by 3 pathways as dissemination via the haematogenous or lymphatic pathway, extension from adjacent organs, such as the bones or lymph nodes, and de novo primary extranodal disease. Most of the histology primary skeletal lymphomas have the aggressive B-cell immunophenoty. In general, treatment is similar to nodal lymphomas. In conclusion, we aimed to contribute in experience with this rare eNHL type.

Tumor characteristics and survival in patients with extranodal non-Hodgkin lymphoma of the female reproductive tract

<h3>Objectives:</h3> Primary extranodal non-Hodgkin lymphoma (NHL) of the female reproductive tract is rare, comprising approximately 1% of all NHL. Studies to date have been limited to case reports without longitudinal follow-up. The objective of this study is to describe tumor characteristics and survival in patients with primary vulvar, vaginal, cervical, uterine and ovarian NHL using the Surveillance, Epidemiology and End Results (SEER) cancer database. <h3>Methods:</h3> Adults with a histologic diagnosis of non-Hodgkin lymphoma (ICD-O-3 9590-9823) and a primary site coded as vulva (ICD-10-CM C51.0-51.9), vagina (C52.9), cervix (C53.0-53.9), uterus (C54.0-C55.9), or ovary (C56.9-C57.4) diagnosed between 1973 and 2015 were included in the analysis. Data was abstracted on variables of interest including age at diagnosis, year of diagnosis, demographics, Ann Arbor stage, histologic subtype, treatment regimens and disease-specific survival (DSS). Descriptive statistics were used to compare characteristics between primary sites. Kaplan-Meier survival curves and multivariable Cox proportional hazards survival analysis were performed using SPSS. <h3>Results:</h3> A total of 793 patients with primary gynecologic NHL were identified, including vulva (n=60), vagina (n=101), cervix (n=187), uterus (n=142) and ovary (n=303). The median age at diagnosis was 55 years old and ranged from 18 to 94 years. Vulvar, vaginal and cervical primary cancers were more likely to be diagnosed at an early stage compared to uterus and ovary (p<0.001) and receive radiation (p<0.001). Ovarian primary NHL was most likely to be diagnosed at an advanced stage (65% stage III or IV) and 88% of patients were treated with surgery. There was no significant difference in chemotherapy administration between the primary sites. The median DSS was not reached. The 5-year disease-specific survival rate was 83.2% for cervical, 81.8% for vaginal, 72.3% for ovarian, 70.9% for vulvar and 69.1% for uterine primary sites (p=0.019). On multivariate analysis, compared to primary ovarian NHL, DSS was significantly improved if the primary site was cervix (hazard ratio 0.61, 95% CI 0.39-0.97) or vagina (HR 0.52, 95% CI 0.29-0.92) with no difference in survival with primary vulvar NHL (HR 0.76, 95% CI 0.43-1.35) or primary uterine NHL (HR 0.90, 95% CI 0.59-1.38). Age at diagnosis (HR 1.04, 95% CI 1.03-1.05), histologic subtype (Burkitt's lymphoma vs other NHL, HR 3.26, 95% CI 1.82-5.84), and advanced stage (Stage II vs I: HR 2.16, 95% CI 1.42-3.28; III vs I: 1.34, 0.60-2.98; IV vs I: 3.50, 2.43-5.06) were associated with worse survival and more recent diagnosis (HR 0.98, 95% CI 0.96-1.00) and treatment with surgery (HR 0.52, 95% CI 0.36-0.73)) were associated with improved survival. <h3>Conclusions:</h3> Overall, extranodal NHL of the female reproductive tract has an excellent prognosis. The clinical characteristics, treatment regimens and survival differ between primary sites.

Uterine non-Hodgkin lymphoma: a case report and review of literatures

&lt;p&gt;&lt;span&gt;Primary extra nodal non-Hodgkin lymphomas of the genital tract are uncommon, and involvement of the uterine corpus is not only extremely rare but lacks pathognomonic signs and symptoms, resulting in delayed diagnosis and treatment. This prompted this case report of a multiparous lady who presented with recurrent bleeding per vaginam and progressive abdominal swelling that was initially suspicious of being uterine sarcoma, but histological analysis of excised sample was diagnostic of non-Hodgkin lymphoma of the uterus. The report also highlights the challenges to diagnosis and management in a resource poor setting where advanced diagnostic aids are not readily accessible. The diagnosis of uterine non-Hodgkin lymphoma, though uncommon, should be considered in any patient with an abdominal/pelvic mass and/or abnormal bleeding per vaginam. &lt;/span&gt;&lt;/p&gt;

Primary Extranodal Non-Hodgkin Lymphomas: A First Tertiary Care Experience from Punjab, North India

Background Primary extranodal lymphomas are less frequently encountered and difficult to diagnose in routine practice. The histopathological and immunohistochemical studies are essential in addition to clinical details to diagnose. The incidence is varied in different parts of India along with variation in histopathological spectrum. Methodology The clinical features and histopathological findings of patients diagnosed with primary extranodal lymphoma over 3-year period were retrieved from archives of pathology department and analyzed. Results During the 3-year study period, a total of 135 cases of non-Hodgkin lymphomas were evaluated, and of these, 41.4% (56/135) of cases presented with primary extranodal involvement. The mean age of primary extranodal lymphoma presentation was 61.3 ± 17.5 with M:F ratio of 1:1.1 Most common extranodal site involved was gastrointestinal tract 32.1%, 18/56 (small intestine [17.8%, 10 cases] and large intestine [8.9%, 5 cases]). Non-Hodgkin lymphomas of B cell type were the predominant subtype (48/56, 85.7%), while 8/56 (14.2%) cases were of T cell lineage. Of the B cell non-Hodgkin lymphoma diffuse, large B cell lymphoma was the predominant subtype constituting ~83.3% (40/48). Conclusion Primary extranodal lymphomas exhibit varied histomorphological and clinical presentation. The present study is first such analysis from Punjab that intends to compare with studies from other parts of India.

Primary extranodal non-Hodgkin lymphoma: Clinical characteristics and outcome.

e20027 Background: According to the National Cancer Registry Program, the Indian Council of Medical Research reports that the estimated Incidence rate of Non-Hodgkins Lymphoma in India is 2-3 per 100,000 people. The incidence rate of extranodal Non-Hodgkins Lymphoma (NHL) in Asia is higher than that of the United States. We illustrate a single center review of the clinical characteristics of all the primary extranodal NHL. Methods: We retrospectively identified patients diagnosed with primary extranodal NHL out of 269 NHL patients diagnosed at the department of medical oncology, Kasturba Medical College hospital, Mangalore, India from August 1999 and September 2015. Results: A total of 43 patients(15.9%) with primary extranodal NHL were identified out of which 25 patients (58%) were male with a median age of 49 years (5-92). 11patients (25%) had tonsil as the extranodal location and 10 patients (23%) had musculoskeletal involvement. Lung, thyroid, ovary, nasopharynx, gastrointestinal, paranasal sinuses and central nervous system were the other sites. 17 patients (39.5%) were presented at stage 4. The most common subtype was diffuse large B cell Lymphoma (DLBCL)-25 patients (58%). 2 patients had HIV infection. Some CD 20+ patients could not afford the cost of Rituximab and hence resorted to the CHOP regimen for 6 cycles. Rest received RCHOP 6-8 cycles depending on the stage and response. Follicular Lymphoma patient received 6 cycles of RCHOP and Rituximab maintenance for 12 cycles once in 2 months. Burkitts lymphoma patient received MCP 842. 3 patients denied treatment. 6 patients had recurrence who received DHAP +/- Rituximab or ICE +/- Rituximab. 1 patient-reported progression treated with supportive care. 6 patients (14%) died and 22 (55%) patients attained complete remission. Conclusions: In our cohort, DLBCL was the most common subtype and tonsil, the most involved site. The prognosis was impacted by the stage at which the patient was presented, HIV infections and the higher cost of Rituximab. Primary extranodal NHL must be considered in the differential diagnosis as the current treatments can achieve complete remission.

Primary Mediastinal Nodal and Extranodal Non-Hodgkin Lymphomas: Current Concepts, Historical Evolution, and Useful Diagnostic Approach: Part 2.

Primary mediastinal non-Hodgkin lymphomas (PM-NHLs) represent ∼5% of all non-Hodgkin lymphomas (NHLs) and comprise lymphomas of B-cell and T-cell origin. PM-NHLs are defined as involvement of mediastinal lymph nodes, thymus, and/or mediastinal organs (heart, lung, pleura, pericardium) by NHL without evidence of systemic disease at presentation. The clinical scenario is variable and depends on the lymphoma subtype. The radiologic presentation is also variable ranging from a mediastinal mass with or without superior vena cava syndrome, a pleural or a cardiac mass associated with effusion, or as an effusion only. The diagnosis of PM-NHLs can only be established by microscopic evaluation, and therefore, general pathologists should be aware of these tumors and familiar with their diagnostic approach. The most common anterior mediastinal NHLs (90% to 95%) are primary mediastinal (PM) large B-cell lymphoma and T-lymphoblastic lymphoma. Thymic marginal zone lymphoma and mediastinal gray zone lymphoma are very rare. The remainder PM-NHLs involving middle or posterior mediastinum include diffuse large B-cell lymphoma (DLBCL) and rare cases of T-cell lymphoma, including anaplastic large cell lymphoma and breast implant-associated anaplastic large cell lymphoma extending to the anterior mediastinum. Primary pleural and cardiac NHLs are mostly DLBCLs. Other rare subtypes of PM-NHLs include DLBCL associated with chronic inflammation/pyothorax-associated lymphoma, fibrin-associated DLBCL (both Epstein-Barr virus positive), and pleural and/or pericardial primary effusion lymphoma (human herpesvirus-8 positive/Epstein-Barr virus positive). We review the historical aspects, epidemiology, clinicoradiologic features, histopathology, immunohistochemistry, differential diagnosis, and relevant cytogenetic and molecular features of the remaining mediastinal B-cell lymphomas, including primary thymic marginal zone lymphoma of the mucosa-associated lymphoid tissue type, other PM small B-cell lymphomas, PM plasmacytoma, and the most relevant PM T-cell lymphomas.

Primary Mediastinal Nodal and Extranodal Non-Hodgkin Lymphomas: Current Concepts, Historical Evolution, and Useful Diagnostic Approach: Part 1

Primary mediastinal non-Hodgkin lymphomas (PM-NHLs) represent ~5% of all NHLs and comprise lymphomas of B-cell and T-cell origin. PM-NHLs are defined as involvement of mediastinal lymph nodes, thymus, and/or mediastinal organs (heart, lung, pleura, pericardium) by NHL without evidence of systemic disease at presentation. The clinical scenario is variable and depends on the lymphoma subtype. The radiologic presentation is also variable ranging from a mediastinal mass with or without superior vena cava syndrome, a pleural or a cardiac mass associated with an effusion, or as an effusion only. The diagnosis of PM-NHLs can only be established by microscopic evaluation, and therefore, general pathologists should be aware of these tumors and familiar with their diagnostic approach. The most common anterior mediastinal NHLs (90% to 95%) are primary mediastinal large B-cell lymphoma and T lymphoblastic lymphoma. Thymic marginal zone lymphoma and mediastinal gray zone lymphoma are very rare. The remainder PM-NHLs involving middle or posterior mediastinum include diffuse large B-cell lymphoma (DLBCL) and rare cases of T-cell lymphoma, including anaplastic large cell lymphoma and breast implant-associated anaplastic large cell lymphoma extending to the anterior mediastinum. Primary pleural and cardiac NHLs are mostly DLBCLs. Other rare subtypes of PM-NHLs include DLBCL associated with chronic inflammation/pyothorax-associated lymphoma, fibrin-associated DLBCL (both EBV), and pleural and/or pericardial primary effusion lymphoma (HHV-8/EBV). We review the historical aspects, epidemiology, clinico-radiologic features, histopathology, immunohistochemistry, differential diagnosis, and relevant cytogenetic and molecular features of PM (thymic) LBCL, PM "nonthymic" DLBCL, BCL, unclassifiable, with features intermediate between DLBCL and classic Hodgkin lymphoma (mediastinal gray zone lymphoma), DLBCL associated with chronic inflammation (pyothorax-associated lymphoma), fibrin-associated DLBCL, and primary effusion lymphoma. This review represents the first part of 2 manuscripts covering PM-NHLs.

PB1801 PRIMARY EXTRA‐NODAL DIFFUSE LARGE B‐CELL LYMPHOMA: A RETROSPECTIVE SINGLE INSTITUTE ANALYSIS ON TREATMENT MODALITIES AND OUTCOME.

Background:Extranodal lymphomas comprise around 30% of Non‐Hodgkin lymphomas (NHL) and the majority are diffuse large B‐cell lymphomas (DLBCL). The exact designation of Primary Extranodal NHL (PE‐NHL) is controversial.Aims:The aim of this study is to assess the clinical characteristics of PE‐DLBCL patients focusing on treatment and survival outcome.Methods:Data from patients with PE‐DLBCL treated in the Federico II University of Naples, were retrospectively analyzed. Patients presenting either systemic disease with extranodal involvement or recurrent lymphoma in an extranodal region following prior treatment were excluded. Kaplan‐Meier survival and Log‐Rank test were used for statistical analysis. All cases were reviewed according to the 2016 WHO criteria from our anatomopathologist (MM). PE‐DLBCL was defined as DLBCL histology in a extranodal site and with a FDG‐PETscan completely negative except for lesion uptake. Patients were treated according to the policy of our centre. Complete surgical resection (SR) of the lesion or organ was performed in case of acute complications and in some cases represented the sole treatment action. Treatment modalities were either radiotherapy (RT) or immune‐chemotherapy (R‐CHOP21), alone or in combination. When a Complete Remission (CR) was documented by a negative FDG‐PET/CT and a negative Gastrointestinal (GI) endoscopy for GI lymphomas, patients were followed every 3 months in the first two years, 6 months in the third and one in the subsequent years.Results:From November 2000 to December 2018 fifty‐four patients with PE‐DLBCL were enrolled. Clinical characteristics were reviwed and divided according to primary organ diagnosis (Fig). No patients had B symptoms but some had minor symptoms related to organ involvement. The most frequent comorbidities were viral infections. FDG‐PET was performed and median glucose uptake was calculated divided by type of extranodal diagnosis, some scans were negative because performed after complete SR. No statistically significance difference was found except for bulky disease, more frequent in the GI tract. Patients were divided in 3 cohorts according to the type of treatment received: the first one (61%) were patients treated with 6 cycles of R‐CHOP‐21 with no dose reductions or delays; in the second group patients (31%) received a combined treatment with R‐CHOP‐21, median of 4 cycles (3‐4), following SR; in the last one, (8%) were treated only with SR or RT (Fig). All patients obtained a CR after treatment. During the follow up (median 54 mo) 5 patients relapsed. Three of them received R‐CHOP 21 alone (2 skin PE‐DLBCL and 1 gastric DLBCL), one had a gonad lymphoma treated with SR and the last one had a splenectomy plus R‐CHOP. Relapses involved both extranodal and nodal sites. Median time to relapse was 24mo from the last treatment received. Total PFS was 85%; PFS for R‐CHOP treated patients was 84%, for locally treated patients 75% and for the combined treatment 90% (p = 0.338); OS was 89%.Summary/Conclusion:PE‐DLBCL is a heterogeneous disease with various clinical manifestations at different anatomical sites. Our retrospective analysis showed that PE‐DLBCLs have a better outcome in term of PFS and OS than reported for nodal DLBCLs. Further prospective data analyses are required so as to better elucidate the biology and course of PE‐DLBCL. These data suggest a successful long lasting response to immune‐chemotherapy that prevent patients from undergoing a surgical approach that can be influenced by many complications or radiotherapy approach possibly characterized by critical site‐specific side effects.image

PB1831 PRELIMINARY RESULTS OF THE COMBINED TREATMENT PRIMARY NON‐HODGKIN ORBITAL LYMPHOMAS

Background:Non‐Hodgkin Lymphomas (NHL) effected organ of vision compose 8–12 % of all primary extranodal NHL, and may be the first and only sign of the disease.Aims:To estimate a therapy of patients with extranodal orbital lesion in our cancer center.Methods:Since 2014 to 2018 in our center treated 17 patients with extranodal damage to the orbit of the eyes. The age of patients ranged from 23 to 92 years, the average age of patients was 57 years. The number of men were 8 people, women ‐ 9. Unilateral orbital damage was registered in 15 patients, bilaterally in 2 patients. In 12 cases process localized in the anterior part of the orbit, in 5 ‐ in the middle and deep parts of the orbit with the spread in the maxillary sinus. Clinical sings of NHL characterized with exophthalmos in varying degrees of severity, difficult reposition, displacement of the eyeball and restriction of its mobility, swelling of the eyelid. By all the observations: to 12 patients performed biopsy of the tumor, 4 patients underwent therapeutic and diagnostic orbitotomy by Killian, to 1 patient ‐ eye orbit exentation. Histological variants: MALT‐ lymphomas – 4 patients, Diffuse B‐cell Lymphoma – 8, Mantle Cell Lymphoma – 2 and Follicular Lymphoma ‐ 2 patients.Results:All patients underwent 6 courses of chemotherapy according to the R‐EPOCH protocol. Complete answer registered in 16 cases, partial answer in 1 of cases, and this patient received a course of radiation therapy on the affected area. Observation median was 3 years (15 month‐5 years).Summary/Conclusion:Therefore, treating orbital NHL requires complex multidisciplinary approach, allowing determining the correct diagnosis and prescribing adequate treatment. Standart first line therapy achieved good results of relapse‐free survival. Chemotherapy showed the same results as the combined treatment (chemotherapy and orbitotomy).