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Primary cardiac lymphoma is a rare entity. We report on the clinicopathological features of 2 patients with primary cardiac lymphomas: one involving the right atrium resulting in intractable right heart failure, and the other involving the pericardium with massive pericardial effusion. In the first patient, sternotomy and surgical biopsy of the tumor were performed to arrive at the diagnosis. In the second patient, CT thorax and transesophageal echocardiography helped to diagnose the pericardial tumor, and cytological examination of the pericardial fluid established the pathological diagnosis of lymphoma. Combination chemotherapy (COPP) was started in both patients. The first patient died on the first day of chemotherapy due to intractable heart failure, while the second attained a partial response to chemotherapy but died of progressive disease 8 weeks later. This is followed by a literature review of 21 patients with primary cardiac lymphoma. In conclusion, the prognosis of primary cardiac tumor remains poor.

Epstein–Barr virus (EBV) infection is proved to be associated with clinicopathology of lymphoma. However, little is known about the relationship between EBV‐DNA status after treatment and prognosis. In this study, real‐time polymerase chain reaction (PCR) was used for quantitative detection of EBV‐DNA load in peripheral blood of all 26,527 patients with lymphoma, and the clinical characteristics and prognosis of 202 patients were retrospectively analysed, including 100 patients with positive EBV‐DNA and 102 randomly selected patients with negative EBV‐DNA. We found that the average rate of EBV‐DNA positivity in lymphomas was 0.376%, and EBV‐DNA‐positive patients presented higher risk with elevated lactate dehydrogenase (LDH) and β2‐MG level, B symptoms, secondary hemophagocytic syndrome and lower objective response rate compared to EBV‐DNA‐negative patients. Multivariate analysis revealed EBV‐DNA‐positive patients had inferior progression‐free survival (PFS) and overall survival (OS) and EBV‐DNA level before treatment was related to PFS but not OS of T/NK cell lymphoma. In T/NK cell lymphoma, EBV‐DNA converting negative after treatment was correlated with better PFS but not OS, and second‐line therapy could induce more EBV‐DNA‐negative conversion compared to CHOP‐based therapy. In all, EBV‐DNA positivity before treatment can be a biomarker representing the tumour burden and an independent prognostic factor. EBV‐DNA‐negative conversion after treatment is a good prognostic factor for T/NK cell lymphomas. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Floaters or visual disturbance in a patient with ENKL should prompt evaluation for possible vitreoretinal involvement. Lymphoma with ocular involvement should be treated aggressively and in most cases heralds CNS involvement. [ABSTRACT FROM AUTHOR]

Intestinal T‐cell lymphomas are common in dogs, but histopathological diagnosis remains challenging because of accompanying enteritis with lymphocyte involvement. Invasively taken full‐layer biopsies are still required for reliable differentiation. The detection of specific microRNA expression patterns in canine intestinal T‐cell lymphoma could provide new possibilities to differ intestinal lymphoma from benign inflammation and could lead to further understanding of lymphomagenesis. The objective of this study was to characterize microRNA expression in distinct groups of formalin‐fixed and paraffin‐embedded samples from canine intestinal T‐cell lymphomas, lymphoplasmacellular enteritis and healthy intestinal tissue. In a preliminary test with two samples per group, total RNA was extracted (RNEasy FFPE Kit, Qiagen), reverse transcribed (miScript II RT Kit, Qiagen) and pre‐amplified (miScript PreAmp PCR Kit, Qiagen). We performed comparative quantitative PCR on microRNA PCR Array plates (Qiagen) with pre‐fabricated reactions for 183 different mature canine microRNAs. Subsequently, 12 microRNAs with conspicuous expression changes in the lymphoma group were selected and microRNA expression of all samples (n = 8) per group was analysed with individual microRNA assays (miScript Primer Assays, Qiagen) on the reverse transcribed RNA without pre‐amplification. Our results revealed lymphoma‐specific expression patterns, with down‐regulation of the tumour‐suppressing microRNAs miR‐194, miR‐192, miR‐141 and miR‐203, and up‐regulation of oncogenic microRNAs, including microRNAs from the miR‐106a~363 cluster. In addition, we detected only slight expression alterations between healthy intestinal tissue and lymphoplasmacellular enteritis cases. We conclude that microRNA expression patterns can be used to separate T‐cell lymphomas from healthy tissue and benign inflammatory disorders. [ABSTRACT FROM AUTHOR]

The 2016 World Health Organisation revised classification of lymphoma has sub‐classified well‐defined entities and added a number of provisional entities on the basis of new knowledge on genetic, epigenetics and phenotypical data; prognostic and predictive features are also part of this classification. New knowledge on well‐defined entities further enlightens the mechanisms of lymphomagenesis, which are more complex and multifactorial than once believed. Therapies are also more complex because traditional clinical trials have been integrated with new drugs and compounds with unique mechanisms of actions against distinct molecular targets. As lymphoma acquires additional genetic and phenotypic features over the time, pathological assessment is also necessary. Histological evaluation and tissue collection by surgical biopsies are necessary for phenotypical and molecular purposes; however, these are demanding procedures for both the patient and the health care system. At the same time, the choice of the best treatment for a specific entity, in different phases and different patients requires information that may not be available when the biopsy is performed. Fine needle aspiration cytology (FNAC) is successfully used in lymph nodes (LNs) in combination with different ancillary techniques and might be used to assess the phenotypic and genetic profile of specific targets and to get key information for therapy, in different phases and stages of the disease, with the option to re‐check the same target over time, without surgical excision. This brief review describes LN‐FNAC diagnostic criteria, current therapies for lymphomas and the potential role of LN‐FNAC in selecting non‐Hodgkin lymphomas patients for specific targeted treatments. Traditional therapies for lymphoma have been integrated with new drugs and compounds with unique mechanisms of actions against distinct molecular targets. Lymph node fine‐needle aspiration cytology might be used to evaluate the phenotypic and genetic profiles, identify specific targets and get useful information for therapeutic planning in different phases of the disease with the option to re‐check the same target over time. [ABSTRACT FROM AUTHOR]

Objective The purpose of this study was to analyze computed tomography (CT) and magnetic resonance (MR) images and to evaluate the maximum standardized uptake value (SUV max) of positron emission tomography (PET)/CT parameters between sinonasal non-Hodgkin's lymphoma (NHL) and squamous cell carcinoma (SCC), knowing the imaging features that distinguish sinonasal NHL from SCC. Study Design Case series with chart review. Setting University tertiary care facility. Subjects and Methods We analyzed the features on CT, MR imaging, and PET/CT of 78 patients diagnosed with sinonasal NHL or SCC histopathologically. The CT (n = 34), MRI (n = 25), and PET/CT (n = 33) images of 39 patients with sinonasal NHL and the CT (n = 38), MR (n = 28), and PET/CT (n = 31) images of 39 patients with SCC were evaluated. The sinonasal NHL was diagnosed as natural killer/T-cell lymphoma (n = 28) and diffuse large B-cell lymphoma (n = 11). Results Patients with sinonasal NHL had a larger tumor volume and higher tumor homogeneity than patients with SCC on T2-weighted and postcontrast MR images. Most of the sinonasal NHL and SCC showed a high degree of enhancement. The apparent diffusion coefficient (ADC) values and adjacent bone destruction were significantly lower in sinonasal NHL than in SCC. However, cervical lymphadenopathy, Waldeyer's ring involvement, and PET/CT SUV max showed no significant differences between sinonasal NHL and SCC. Conclusion CT and MR images of sinonasal masses showing a bulky lesion, marked homogeneity, and low ADC values without adjacent bone destruction are more suggestive of sinonasal NHL than SCC. [ABSTRACT FROM AUTHOR]

Primary gastrointestinal (GI) T- and NK-cell lymphomas are usually aggressive neoplasms associated with high morbidity and mortality. Over the past two decades, however, cases of primary GI lymphoproliferative disorders (LPDs) or lymphomas of T- or NK-cell derivation with indolent behavior have been reported. These LPDs are rare and they can be challenging to diagnose as they share clinical and pathological features with both, inflammatory disorders and aggressive T- and NK-cell lymphomas. Primary, indolent clonal T-cell proliferations of the GI tract, which can be CD4+, CD8+ or CD4- CD8-, have been included as a provisional entity in the newly revised World Health Organization (WHO) classification of lymphoid neoplasms and designated 'indolent T-cell LPD of the GI tract'. It is currently unclear whether the indolent NK-cell LPDs represent reactive or neoplastic proliferations. In this review, we describe the clinical, morphologic, immunophenotypic and genetic features of indolent GI T- and NK-cell LPDs and provide guidance in differentiating them from other inflammatory and neoplastic diseases. We believe that greater awareness of these LPDs amongst physicians and the research community will lead to timely and accurate diagnoses, stimulate investigations into the pathogenetic mechanisms underlying different entities thereby enhancing our understanding of disease biology and enable the development of effective therapeutic regimens. Copyright © 2016 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

Cryptococcosis is an opportunistic invasive fungal infection that is well described and easily recognised when it occurs as meningitis in HIV-infected persons. Malignancy and its treatment may also confer a higher risk of infection with Cryptococcus neoformans, but this association has not been as well described. A case of cryptococcosis in a cancer patient is presented, and all cases of coincident C. neoformans infection and malignancy in adults published in the literature in English between 1970 and 2014 are reviewed. Data from these cases were aggregated in order to describe the demographics, type of malignancy, site of infection, clinical manifestations, treatment and outcomes of cryptococcosis in patients with cancer. Haematologic malignancies accounted for 82% of cases, with lymphomas over-represented compared to US population data (66% vs. 53% respectively). Cryptococcosis was reported rarely in patients with solid tumours. Haematologic malignancy patients were more likely to have central nervous system ( P < 0.001) or disseminated disease ( P < 0.001), receive Amphotericin B as part of initial therapy ( P = 0.023), and had higher reported mortality rates than those with solid tumours ( P = 0.222). Providers should have heightened awareness of the possibility of cryptococcosis in patients with haematologic malignancy presenting with infection. [ABSTRACT FROM AUTHOR]

Background Few studies have evaluated the histopathologic features of cutaneous extranodal natural killer ( NK)/T-cell lymphoma ( ENKTL), and the histopathologic spectrum of this disease according to its clinical morphology remains unclear. Objective This study investigated the differences in pathologic findings of cutaneous ENKTL depending on clinical morphology. Methods A total of 41 cases of cutaneous ENKTL were included. Skin lesions were classified according to clinical morphology as: (i) nodular lesions, (ii) cellulitis or abscess-like swellings and (iii) erythematous to purpuric patches. Histopathologic variables were compared between groups. Results Perivascular infiltration of tumor cells and vasculopathy in the dermis and subcutaneous layer were common microscopic findings irrespective of clinical morphology. Erythematous to purpuric patches were mainly composed of small-sized tumor cells, whereas medium- to large-tumor cells were predominant in lesions of other clinical morphologies. The density of tumor cell infiltration was significantly higher in cellulitis or abscess-like lesions or nodular lesions compared with erythematous to purpuric patches. A panniculitis-like pattern and angiocentricity were less common in patch lesions than in cellulitis-like swelling and nodular lesions. Conclusion There is a histopathologic spectrum of cutaneous ENKTL that is dependent on the clinical morphology. [ABSTRACT FROM AUTHOR]

Primary extranodal natural killer/ T- cell lymphoma, nasal type ( NK/TCL) in the lung is extremely rare and associated with Epstein- Barr virus ( EBV) infection. An 80-year-old male presented with hemoptysis, which had lasted three days. Physical examination revealed inspiratory crackles at the left lung base and massive splenomegaly. Chest radiograph shows a mass-like lesion in the left lower lung but no active lesion six months earlier. Computed tomography demonstrated a soft tissue mass (size: 6.6 × 5.1 cm) with increased ground-glass opacities in the left lower lobe, several pulmonary nodules, and mediastinal lymphadenopathy. Transthoracic needle biopsy of the left-lower-lobe lung mass was performed. The pathology revealed atypical lymphoid cell infiltration, which is immunoreactive for cytoplasmic CD3, CD30 and CD56, but not reactive for CK and CD20. EBV-encoded RNA ( EBER) was also detected in these atypical lymphoid cells. The serum EBV DNA level was 7.03 × 106 copies/mL and subtype 1 EBV was identified. No evidence of lymphoma involvement was found in the extrathoracic site. Primary pulmonary lymphoma showing nasal-type NK/T-cell subtype was diagnosed. Chemotherapy with cyclophosphamide and prednisolone was initiated immediately but the patient deteriorated and died three weeks later. In conclusion, patients presenting with rapidly growing lung mass and massive splenomegaly raise the possibility of aggressive pulmonary lymphoma. Extranodal NK/T-cell lymphoma with high baseline plasma EBV DNA levels signifies poor prognosis. Identifying young high-risk patients may have benefits for early aggressive and successful treatment. [ABSTRACT FROM AUTHOR]

Non- Hodgkin lymphoma only rarely occurs as a primary lung mass. We report a very rare case of primary pulmonary anaplastic large cell lymphoma presenting with acute atelectasis in a 55-year-old man. Chest computed tomography revealed a consolidated central mass in the left lung with obstructive pneumonia that had developed into total atelectasis. After a bronchoscopic examination failed to yield a definite diagnosis, supraclavicular lymph node biopsy was performed, revealing an anaplastic large cell lymphoma. This case illustrates the need for rapidly locating a possible biopsy site, other than the primary lung mass itself, and the value of empirical steroid treatment for avoiding devastating exacerbation when aggressive pulmonary lymphoma is suspected. [ABSTRACT FROM AUTHOR]

Primary infection with Epstein-Barr virus ( EBV) is usually asymptomatic and, in a normal host, EBV remains latent in B cells after primary infection for the remainder of life. Uncommonly, EBV can infect T or natural killer ( NK) cells in a person with a defect in innate immunity, and EBV infection can cause unique systemic lymphoproliferative diseases ( LPD) of childhood. Primary infection in young children can be complicated by hemophagocytic lymphohistiocytosis or fulminant systemic T-cell LPD of childhood. Uncommonly, patients can develop chronic active EBV ( CAEBV) disease-type T/ NK LPD, which includes CAEBV infection of the systemic form, hydroa vacciniforme-like T-cell LPD, and mosquito-bite hypersensitivity. The clinical course of CAEBV disease-type T/ NK LPD can be smoldering, persistent or progressive, depending on the balance between viral factors and host immunity. Aggressive NK-cell leukemia, hydroa vacciniforme-like T-cell lymphoma, or uncommonly extranodal NK/ T-cell lymphoma can develop in children and young adults with CAEBV disease-type T/ NK-cell LPD. Extranodal T/ NK-cell lymphoma is a disease of adults, and its incidence begins to increase in the third decade and comprises the major subtype of T/ NK LPD throughout life. Aggressive NK-cell leukemia and nodal T/ NK-cell lymphoma of the elderly are fulminant diseases, and immune senescence may be an important pathogenetic factor. This review describes the current progress in identifying different types of EBV-associated T/ NK-cell LPD and includes a brief presentation of data from Korea. [ABSTRACT FROM AUTHOR]

The aim of the study was to report the outcome of treatment of 97 dogs with lymphoma that received a multi-agent chemotherapy protocol containing epirubicin as the primary anthracycline. Seventy-five dogs received a 25-week protocol with no maintenance phase whilst 22 dogs received a maintenance phase. Complete response rate was 96% and time to first relapse (TTR) and overall survival ( OS) time for all dogs were 216 and 342 days, respectively. Dogs with T-cell lymphoma and those classified as WHO substage b had significantly poorer OS times and TTR. The protocol was well tolerated with toxicity similar to doxorubicin-containing protocols. Epirubicin as part of a multi-agent protocol is safe and effective in the treatment of canine multicentric lymphoma. There is a high initial response rate and an overall median survival time that is similar to other published doxorubicin-containing protocols. [ABSTRACT FROM AUTHOR]

We investigated immunodeficiency-related non-Hodgkin lymphoma for the presence of molecular alterations affecting negative regulators of the Janus family protein tyrosine kinase/signal transducer and activator of transcription pathway. Protein tyrosine phosphatase, non-receptor type 6/Src homology 2-containing tyrosine phosphatase-1 epigenetic silencing was recurrent in primary effusion lymphoma (100%), and diffuse large B-cell lymphoma (63%), with a higher prevalence in the non-germinal centre subtype, and was associated with the activation of the Janus family protein tyrosine kinase/signal transducer and activator of transcription 3 pathway. Suppressor of cytokine signalling ( SOCS)1 and SOCS3 epigenetic silencing were occasionally detected, whereas SOCS1 was frequently mutated in diffuse large B-cell lymphoma and polymorphic post-transplant lymphoproliferative disorders, possibly as a cause of aberrant somatic hypermutation. However, the mutation profile of the coding region of the gene was different from that expected from the aberrant somatic hypermutation process, suggesting that, at least in some cases, SOCS1 mutations may have been selected for their functional activity. Copyright © 2012 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

BACKGROUND. Prognostic factors and outcomes in patients with marginal zone lymphoma (MZL) have been studied in small cohort studies, which may not reflect the population at large. METHODS. Clinical characteristics and survival outcomes of adult patients with MZL who were diagnosed between 1995 and 2009 were evaluated using the Surveillance, Epidemiology, and End Results (SEER) database. The authors generated clinical prognostic models for subtypes of MZL and compared survival during the periods of 1995 through 2000, 2001 through 2004, and 2005 through 2009. RESULTS. The prognosis was significantly better for patients with mucosa-associated lymphoid tissue (MALT) lymphoma (5-year relative survival rate of 88.7%; P < .0001) compared with those with the splenic MZL (SMLZ)or nodal MZL (NMZL) subtypes (5-year relative survival rates of 79.7% and 76.5%, respectively). There was evidence of improved outcomes in patients with NMZL and MALT lymphomas between 1995 and 2009 ( P < .0001), with no difference noted in patients with SMZL ( P = .56). Advancing age and the presence of B symptoms had prognostic significance in all MZL subtypes. Male sex and stage of disease were significant only for the NMZL and MALT categories. Survival in patients with MALT lymphomas varied depending on the site of origin, with a worse prognosis noted in those with gastrointestinal and pulmonary locations of origin (5-year incidence rate of lymphoma-related death, 9.5%-14.3%) compared with ocular, cutaneous, and endocrine sites (4.5%-7.8%; P < .0001). CONCLUSIONS. The survival for patients with SMZL is similar to that for those with NMZL, and unlike the NMZL and MALT subtypes, it has not improved over the past decade. The prognosis of patients with MALT lymphoma varies according to the anatomical site of origin. Cancer 2013. © 2012 American Cancer Society. [ABSTRACT FROM AUTHOR]

Masses within the thymus and mediastinum are frequently biopsied to establish a diagnosis. The specimens sent for histopathological analysis are typically small (needle) biopsy specimens on which the pathologist is challenged to provide a classifying diagnosis. Despite the fact that the pathology of the anterior mediastinum is predominantly centred on the thymus and it thus might be expected that the range of possible diagnoses is limited, the list of disease entities is in fact extensive, encompassing hyperplastic conditions and both benign and malignant neoplasms. In addition to primary thymic epithelial tumours, a number of less common diseases typical for this location may be encountered, including extragonadal germ cell tumours, lymphomas, soft tissue tumours and a number of conditions which may simulate a tumour, such as cysts and inflammatory conditions. In this review, the disease entities that may be encountered in biopsy material from anterior mediastinal/thymic processes are discussed and an approach to tackle the differential diagnosis is provided. [ABSTRACT FROM AUTHOR]

Mucosa-associated lymphoid tissue (MALT) lymphoma of the gallbladder is rare with six cases reported in the English published reports. Most patients present with symptoms consistent with cholecystitis. We describe a case of extranodal marginal zone B-cell lymphoma of MALT of the gallbladder in a 60-year-old woman who presented with abnormal liver function tests and subsequent finding of gallbladder thickening on ultrasonography. Cholecystectomy revealed a chronically inflamed gallbladder with multiple gallstones. Histology revealed abnormal sheets of small B lymphoid cells which were CD 20 positive, consistent with a diagnosis of MALT lymphoma. The pathogenesis of MALT lymphoma in the gallbladder remains controversial. As the gallbladder does not normally contain lymphocytes, persistent lymphoid proliferation due to chronic Gram-negative infection may represent a critical step in the development and progression of MALT lymphoma. Cholecystectomy without adjuvant chemotherapy is the appropriate first line treatment if there is no other symptomatic disease. [ABSTRACT FROM AUTHOR]

The present study reports five CD8+, CD56+ (natural killer (NK)-like) T-cell lymphomas involving the small intestine without evidence of enteropathy, from Japan. Three were intestinal T-cell lymphoma. The site of origin of the other two was not definitive. Four of five patients underwent emergency operation because of intestinal perforation. The small intestines of these patients had multiple ulcerative lesions with or without demarcated tumors. Histologically, the lymphoma cells were monomorphic or slightly pleomorphic and displayed epitheliotropism of varying degrees. Lymphoma cells of all patients shared the common phenotype: CD3+, CD4−, CD5−, CD8+, CD56+, CD57−, T-cell intracellular antigen-1+, granzyme B+. In contrast to nasal/nasal type NK-cell lymphomas, they had clonal rearrangement of T-cell receptor( TCR) genes and were negative for EBV-encoded RNA. Immunohistochemistry and genetics suggested that three cases were of αβT-cell origin and two cases were of γδT-cell origin. There was no evidence of enteropathy in any patient. The cases followed a clinically aggressive course with a frequent involvement of lung. According to the classification based on the recent genetic studies of European enteropathy-type intestinal T-cell lymphoma (ETL), the present cases could be classified as type 2 ETL. [ABSTRACT FROM AUTHOR]

Tumor suppressor gene (TSG) inactivation by chromosomal deletions and/or mutations is a well-characterized genetic alteration in lymphomas. Array-based technologies have greatly increased the detection and characterization of chromosomal imbalances and regions with loss of heterozygosity (LOH), leading to the identification of a number of novel candidate TSG loci. In addition, microarray platforms for studying DNA methylation and histone modifications enable identifying epigenetic changes affecting gene expression of TSG. Combining these microarray technologies with gene expression profiling is a promising strategy to discover novel TSG in regions targeted by genetic or epigenetic alterations. In this review we present an outline of methodological aspects of the various microarray technologies, and discuss their potentials and restrictions. Furthermore, we survey research findings derived from these high-throughput techniques, which are allowing a deeper insight into the mechanisms of lymphomagenesis. [ABSTRACT FROM AUTHOR]

Background. This study reviews the outcome of patients with nasal natural killer (NK)/T-cell lymphoma treated at the Therapeutic Radiology Department, National Cancer Centre, Singapore, from 1997 to 2003. Methods. Twenty-one consecutive patients treated with radiotherapy, with or without chemotherapy, were retrospectively reviewed. Results. The median age was 44 years (range, 27–86 years). Thirteen patients had stage I disease, five had stage II disease, and three had stage IV disease. Immunophenotyping was CD 56+ in 18 patients. Median follow-up for patients still alive was 23.4 months (range, 8.9–78.5 months). A median dose of 50 Gy (range, 35–56 Gy) was delivered. Sixteen patients also received chemotherapy. Two-year overall survival was 52.8%. Five patients had rapidly progressive disease, with a median survival of 89 days from diagnosis. The other 16 patients had complete remission, after which four relapsed. There were two local relapses. Conclusions. This disease often carries a poor prognosis, despite multimodality treatment. Radiotherapy may contribute to local control in some patients. © 2005 Wiley Periodicals, Inc. Head Neck27: XXX–XXX, 2005 [ABSTRACT FROM AUTHOR]

Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We report the case of an immunocompetent 29-year-old male who presented with syncope and arrythmias secondary to a ventricular cardiac mass. Transcutaneous cardiac biopsy was non-diagnostic, therefore an open cardiac biopsy was performed from which a provisional diagnosis of a cardiac inflammatory pseudotumour was made. Six months after presentation, he developed several subcutaneous lesions with systemic symptoms. Histological and immunophenotypic review of the initial cardiac biopsy revealed features consistent with a diagnosis of CD30, ALK1 positive anaplastic large cell lymphoma (ALCL). Despite intensive treatment with combination chemotherapy, there was significant progression of disease, and he died 11 months after diagnosis. The overall prognosis of cardiac lymphoma remains poor, which may be due to the often late presentation of the tumour. To our knowledge, this is the first reported case of a cardiac ALK positive ALCL. Although rare, cardiac presentation of ALCL should be added to the list of differential diagnoses of cardiac lymphomas. [ABSTRACT FROM AUTHOR]

To analyse the clinicopathological findings of extranasal CD56+ cytotoxic T- or NK-cell lymphomas in different organs and to compare Epstein—Barr virus (EBV)+ and EBV— lymphoma of non-blastoid cytomorphology. Extranodal CD56+ EBV— lymphoma at extranasal sites is a clinically less aggressive malignancy and displays less necrosis than CD56+ EBV+ lymphoma. Because CD56+ EBV+ TCR+ lymphomas show similar pathological and clinical findings to CD56+ EBV+ TCR— lymphomas, nasal-type NK / T-cell lymphomas at extra-nasal sites should be diagnosed as such on the basis of EBV+, cytotoxic T or NK phenotype irrespective of the genotype determined by molecular study. [ABSTRACT FROM AUTHOR]

Aims: Rosette-forming malignant lymphoma is very rare. We report a blastic NK-cell lymphoma expressing terminal deoxynucleotidyl transferase (TdT) with formation of Homer–Wright type pseudorosettes. Methods and resultsAn 18-year-old boy presented with an enlarged inguinal lymph node. Histologically, the nodal architecture of the lymph node was diffusely effaced by small to medium sized monomorphic blastoid lymphoid cells which frequently formed Homer–Wright type pseudorosettes. Immunophenotyping of the tumour using immunohistochemistry and flow cytometry revealed LCA+, CD4+, CD56+, CD43+, TdT+, CD2-, cCD3-, CD8-, CD7-, CD34- and TIA-1-. DNA analysis revealed no gene rearrangement of TCR β and γ genes. In situ hybridization for EBER 1 & 2 was negative. No azurophilic granules were found in the Wright stain. Complete remission was achieved with six cycles of chemotherapy with the CHOP regimen. The disease recurred in the paranasal sinuses and bone marrow 2 years later. ConclusionsImmunophenotypic and genotypic similarities of the present case to those of TdT-negative blastic NK-cell lymphoma suggest that these diseases might be categorized as one entity irrespective of expression of TdT. [ABSTRACT FROM AUTHOR]