The epidemiology of non-Hodgkin's lymphoma: Comparison of nodal and extra-nodal sites

International population-based cancer incidence data, coded according to the International Classification of Diseases for Oncology (WHO, 1990), were used to describe geographical patterns of incidence of extra-nodal non-Hodgkin's lymphomas. Incidence data from the USA were also used to describe age and sex distribution of lymphomas at different extra-nodal sites. The percentage of all non-Hodgkin's lymphomas coded as being of extra-nodal origin is between 25% and 35% in most countries, with the stomach, skin and small intestine being the most common extra-nodal sites. In general, the pattern of incidence rates for extra-nodal lymphomas tends to reflect that of other lymphomas. For example, the age incidence curve of each site-specific extra-nodal lymphoma is similar to that of nodal lymphomas, and in countries where total lymphoma incidence is high the incidence of lymphomas at each extra-nodal site also tends to be relatively high. Although specific factors are known to increase the risk of lymphomas at certain anatomical sites, these data suggest that the aetiology of extra-nodal lymphomas is not entirely independent from that of nodal lymphomas. Int. J. Cancer 72:923–930, 1997. © 1997 Wiley-Liss, Inc.     

Clinico-pathological features of malignant lymphomas in 294 Hong Kong Chinese patients, retrospective study covering an eight-year period

The clinical records and histological material from 294 adult Chinese patients with malignant lymphoma were examined. These patients were first seen at the Queen Mary Hospital, Hong Kong, during the 8-year period 1975-82. There were 27 patients (9.2%) with Hodgkin's disease (HD) and 267 with non-Hodgkin's lymphoma (NHL). The median age at presentation was younger for HD (45 years) and the male: female ratio was higher (2:1) than the corresponding figures for NHL of 51 years and 1.4:1. In 76 patients (28.5% of NHL), the disease was thought to have originated in an extra-nodal site, 48 of these cases being gastrointestinal lymphomas. It was possible to reclassify 234 NHL according to the Rappaport and Kiel classifications, and the Working Formulation (WF) proposed by the US National Cancer Institute Study; for HD, the Rye classification was used in 26 cases where suitable material was available. Nodular/follicular lymphomas made up 17.1% of nodal NHL and 5.3% of extra-nodal NHL. The "histiocytic" (Rappaport) or large-cell (WF) subtype was the commonest amongst diffuse NHL. There were only four cases of Burkitt's lymphoma. For HD, the nodular sclerosing subtype was commonest in females (5 out of 8 cases) and for males, the commonest was mixed cellularity (10 out of 18 cases). Of patients with nodal NHL 64.7%, presented with Stage IV disease. For HD, there were about equal numbers of patients presenting with Stage II and Stage IV disease (10 and 9 respectively). The low incidence of Hodgkin's disease and of follicular lymphomas is comparable to figures from other "oriental" countries such as Japan.     

Non-Hodgkin's lymphomas in the Middle East. A study of 417 patients with emphasis on special features

A total of 417 evaluable patients with non-Hodgkin's lymphomas were diagnosed between January 1974 and December 1983 at the American University of Beirut Medical Center in Beirut, Lebanon. Of these, 179 (43%) patients had nodal lymphomas, and 183 (44%) had extranodal lymphomas. The commonest lymphoma was diffuse large cell (27%), followed by large cell immunoblastic (21%). The histopathologic pattern was follicular in 18% of the nodal lymphomas and in 5.3% of the extranodal forms. The most common site of extranodal lymphoma was the gastrointestinal tract (46.5%), followed by Waldeyer's ring (19%). Small intestinal lymphomas were three times more common than gastric lymphomas. Immunoproliferative small intestinal disease (IPSID) was diagnosed in 20 of 59 patients who had primary small intestinal lymphoma. Of the 34 patients who had Waldeyer's ring lymphoma, 7 had gastrointestinal involvement at some time during the course of the disease. Nodal lymphomas were associated with poor prognostic factors: 82% were diffuse; 77% had advanced disease at presentation; 77% had intermediate- or high-grade malignancy lymphoma; 40% had marrow involvement; and 46% had B symptoms. In children, the most common lymphoma was Burkitt's, and 80% of pediatric lymphomas were high-grade malignancy. In conclusion, this study delineates the special features of non-Hodgkin's lymphomas in the Middle East: The presence of IPSID; the high incidence of extranodal forms, in particular the intestinal ones; and the rarity of follicular lymphomas.     

Lymphoma of the thyroid and head and neck

The most common sites for extra-nodal lymphoma of the head and neck are Waldeyer's ring, most frequently the tonsil, and the salivary glands, usually the parotid. Most are B-cell malignancies and stage IE or IIE at diagnosis. Marginal zone lymphoma of mucosa-associated lymphoid tissue type is particularly associated with inflammatory conditions in the thyroid and salivary glands. The management of extra-nodal lymphoma in the head and neck is similar to nodal B-cell lymphoma with R-CHOP chemotherapy followed by radiotherapy, recommended for early-stage high-grade disease, and radiotherapy alone for localised low-grade lymphoma. The notable exception is NK/T-cell lymphoma of nasal type where radiotherapy is critically important and recommended to a higher dose, partly because of poor response to anthracycline-based chemotherapy regimens like CHOP. Given the higher doses required and the proximity of critical normal structures, intensity-modulated radiotherapy should be considered for these tumours.     

CD52 expression in non-mycotic T- and NK/T-cell lymphomas

CD52 antigen (Campath-1) is expressed in high density by lymphocytes and monocytes. Campath-1H or alemtuzumab, a human anti-CD52, has been shown to be effective in T-cell malignancies; however, there is very limited information on CD52 expression in T-cell lymphoma (TCL). This study retrospectively investigated 97 TCL cases by immunohistochemistry using paraffin sections to elucidate the CD52 expression rates in various TCL sub-types. Fourteen cases of angioimmunoblastic T-cell lymphoma (AITL) were excluded as there were no reliable criteria to differentiate whether the CD52-positive cells were neoplastic T-cells, which are usually small-sized, or the usually abundant, small-to-large residual/reactive B-cells in this lymphoma sub-type. In the remaining 83 tumors, CD52 was expressed in 29 (35%) tumors including 8/17 (47%) NK/T-cell lymphomas, 14/35 (40%) unspecified peripheral TCLs and 4/18 (22%) anaplastic large cell lymphomas. There was no statistical significance in CD52 expression in terms of patient age, gender, nodal vs extra-nodal presentation or tumor sub-types. The authors recommend performing CD52 immunostaining for future clinical trials of alemtuzumab on TCL patients and to correlate the staining results with treatment outcome.     

Marginal zone B-cell lymphoma: A clinical comparison of nodal and mucosa-associated lymphoid tissue types. Non-Hodgkin's Lymphoma Classification Project.

PURPOSE: In the International Lymphoma Study Group classification of lymphoma, extranodal marginal zone B-cell lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT) type is listed as a distinctive entity. However, nodal MZL is listed as a provisional entity because of questions as to whether it is truly a disease or just an advanced stage of MALT-type MZL. To resolve the issue of whether primary nodal MZL without involvement of mucosal sites exists and whether it is clinically different from extranodal MALT-type lymphoma, we compared the clinical features of these two lymphomas. PATIENTS AND METHODS: Five expert hematopathologists reached a consensus diagnosis of MZL in 93 patients. Seventy-three were classified as having MALT-type MZL because of involvement of a mucosal site at the time of diagnosis, and 20 were classified as having nodal MZL because of involvement of lymph nodes without involvement of a mucosal site. RESULTS: A comparison of the clinical features of nodal MZL and MALT-type MZL showed that more patients with nodal MZL presented with advanced-stage disease (71% v 34%; P =. 02), peripheral lymphadenopathy (100% v 8%; P <.001), and para-aortic lymphadenopathy (56% v 14%; P <.001) than those with MALT-type MZL. However, fewer patients with nodal MZL had a large mass (> or = 5 cm) than those with MALT-type MZL (31% v 68%; P =.03). The 5-year overall survival of patients with nodal MZL was lower than that for patients with MALT-type MZL (56% v 81%; P =.09), with a similar result for failure-free survival (28% v 65%; P =.01). Comparisons of patients with International Prognostic Index scores of 0 to 3 showed that those with nodal MZL had lower 5-year overall survival (52% v 88%; P =.025) and failure-free survival (30% v 75%; P =.007) rates than those with MALT-type MZL. CONCLUSION: Nodal MZL seems to be a distinctive disease entity rather than an advanced stage of MALT-type MZL because the clinical presentations and survival outcomes are different in these two types of MZL. Clinically, nodal MZL is similar to other low-grade, node-based B-cell lymphomas, such as follicular and small lymphocytic lymphomas.     

T-cell derived cytokines co-stimulate proliferation of CD40-activated germinal centre as well as follicular lymphoma cells

Follicular lymphomas, the malignant counterparts of normal germinal centre (GC) B-cells, grow in vivo in close association with polyclonal T-cells, predominantly from the T-helper cell type. T-cell-derived growth factors are involved in the development of GC B-cells. However, their role in the pathogenesis of follicular lymphomas has not been clearly defined. We investigated the co-stimulatory activity of 14 cytokines (interleukin-1 to -8, IL-10, IL-13, IFN-α, TNF-α, GM-CSF and SCF) on the proliferation of CD40-activated follicular lymphoma cells in comparison to tonsillar GC B-cells. Tonsillar GC B-cells (n=4), malignant cells from diagnostic lymph node biopsies of patients with follicular (n=4) or transformed (n=4) lymphomas were grown on irradiated CD40-ligand transfectants, with and without cytokines. [³H]-thymidine uptake was measured at day 7. IL-10 and IL-4 proved to be the most potent co-stimulators of proliferation of tonsillar GC B-cells, whereas proliferation of follicular lymphoma cells was co-stimulated by IL-4. The fact that IL-4 is a T-cell derived cytokine, suggests that lymphoma infiltrating T-cells play a role in the growth of these malignancies. Moreover, proliferation of both non-neoplastic tonsillar GC B-cells and follicular lymphomas is co-stimulated by T-cell derived cytokines, indicating that responsiveness to paracrine factors may not be a characteristic of the malignant phenotype. © 1997 John Wiley & Sons, Ltd.     

Peripheral/post-thymic T-cell lymphomas: a spectrum of disease. Clinical, pathologic, and immunologic features of 78 cases

The clinical, pathologic, and immunologic features of 78 cases of peripheral/post-thymic T-cell lymphomas are described. These neoplasms were extremely heterogeneous and were classified as small lymphocytic, mixed small and large cell, large cell, lymphoepithelioid cell, angiocentric, and adult T-cell leukemia/lymphoma type. Some cases revealed angioimmunoblastic or Hodgkin's-like features. These neoplasms mainly affected older adults (mean age, 57 years; median age, 60 years). Lymphadenopathy represented the most frequent clinical presentation, although most patients demonstrated both nodal (87%) and extranodal involvement (77%) during the course of disease. Sites of extranodal disease included skin/soft tissue, spleen, lung, liver, bone, gastrointestinal tract, central nervous system, peripheral blood, nasopharynx, and retrovaginal tissue. Splenomegaly at presentation was most frequently observed in lymphoepithelioid cell lymphomas. Angiocentric lymphomas involved lung. A mediastinal presentation was typically observed in young adults and associated with a poor prognosis. Patients with gastrointestinal lymphomas presented with bleeding and/or malabsorption. B symptoms were present in most cases (65%). Hypercalcemia occurred in four patients. Phenotypic studies of T-cell antigens demonstrated the loss of one or more pan-T-cell markers in eight of 47 cases evaluated. Assessment of T-cell subsets revealed a helper/inducer phenotype for nearly all immunoreactive cases. For the overall series, 32 patients died of disease (median survival time, 11.5 mo). There was a statistical difference between the combined groups of small lymphocytic and lymphoepithelioid cell types as compared with mixed and large cell types, with a poorer survival for the latter group. Angiocentric and adult T-cell leukemia/lymphoma were associated with poor survival. This series of T-cell lymphomas further documents the marked heterogeneity of this group of neoplasms as well as the poor prognosis observed for certain histologic types.     

Primary malignant lymphoma of the breast. Lymphoma of the mucosa-associated lymphoid tissue

Eight cases of primary non-Hodgkin's lymphoma of the breast found in the pathology files of the Institute of Oncology, Ljubljana, Yugoslavia, for a period of 25 years (from 1961 to 1985) were analyzed. During the same period 5711 cases of breast carcinoma were seen. Seven cases were diffuse lymphomas, and one case was nodular. Five cases were high-grade large cell lymphomas and three cases were of low-grade type. One case of the latter group was associated with elevated IgA in the serum and showed monoclonal reaction of plasmacytic lymphoma cells for kappa light chain and IgA. In this case amyloid deposits were seen in the breast tumor. In six cases, focal infiltration of ductal/lobular epithelium by lymphoma cells was found. This so-called lymphoepithelial lesion appears to be an important characteristic of various mucosa-associated lymphoid tissue lymphomas which have been described in different extranodal sites (e.g. gastrointestinal tract, and respiratory tract). They may show different behavior from nodal counterparts, especially in terms of their spread to other mucosal sites which may appear before or without nodal dissemination. The breast appears to be yet another location for these lymphomas.     

Malignant Lymphomas in Pakistan According to the WHO Classification of Lymphoid Neoplasms

Objective: To determine the spectrum of malignant lymphomas in our set up, according to the WHO classification. Methods: All the cases diagnosed as malignant lymphoma, during the year 2005, were retrieved from the institution based tumour registry record and classified according to WHO criteria depending on the immunohistochemical results of a panel of lymphoma markers. Results: The male to female ratio was 2.5:1 for almost all types of malignant lymphomas. The age range was 3 to 80 years. The frequency of Hodgkin's lymphoma, Burkitt's lymphoma and lymphoblastic lymphoma were higher amongst the children, whereas follicular lymphomas, mantle cell lymphoma and CLL/SLL were more frequently reported in 5th, 6th and 7th decades. Of the total cases 62% were nodal and 38% extranodal (majority in the GI tract). Non Hodgkin's lymphoma was more (73%) frequent than Hodgkin's disease. Mixed cellularity and nodular sclerosis were the main histological variants of Hodgkin's disease. Conclusions: Immunohistochemistry is not very frequently used in our set up and also at very few other centres. Therefore, its application should be encouraged to raise the quality of data on lymphoid neoplasms and contribute to their control.     

Malignant lymphoma in Hawaii-Japanese: a retrospective morphologic survey

A retrospective morphologic survey (1973-1983) of 146 cases of malignant lymphoma among the Hawaii-Japanese (migrant Japanese and their offspring) was conducted to determine whether differences in the incidence and cytologic types of malignant lymphoma exist when compared to those of native Japanese (lifetime residents of Japan). The age-adjusted incidence rates for malignant lymphoma among the Hawaii-Japanese were similar to rates for U.S. whites. However, higher rates for follicular centre cell (FCC) lymphoma with a follicular pattern were observed in the Hawaii-Japanese population when compared with rates for native Japanese. On the basis of the cytologic types of the Lukes-Collins classification, non-Hodgkin's lymphomas among the Hawaii-Japanese resembled those of Western countries, rather than those of Japan. B-cell lymphomas predominated (72 per cent), while T-cell types comprised 23 per cent of cases. Follicular centre cell types were encountered most often (59 per cent), and the small cleaved FCC subtype was the most common (30 per cent). The high degree of follicularity (29 per cent) was at variance with the consistently low rates reported in Japan. This may be explained, in part, by higher rates of nodal lymphomas among the Hawaii-Japanese. Of the T-cell lymphomas, diffuse large cell types (T-cell immunoblastic sarcoma, T-IBS), often with cytologic pleomorphism, were relatively frequently encountered (16 per cent), and comprised 15 per cent of non-Hodgkin's lymphomas; this observation necessitates special clinical and epidemiologic consideration in view of the large Japanese migration to Hawaii from HTLV-I endemic regions of southern Japan. No registered cases of non-Hodgkin's lymphoma or of Hodgkin's disease were documented in Hawaii-Japanese subjects under the age of 15 years. The age-adjusted incidence rates for Hodgkin's disease among the Hawaii-Japanese were similar with those of native Japanese. Nodular sclerosis was the most frequent histologic subtype. The difficulty in distinguishing between Hodgkin's disease and non-Hodgkin's lymphoma, particularly when immunologic cell surface markers are not available, is addressed. Low rates for chronic lymphocytic leukemia among the Hawaii-Japanese were confirmed. Not one well-documented case was identified in the 11-year period surveyed.     

Radiotherapy studies and extra-nodal non-Hodgkin lymphomas, progress and challenges

Extra-nodal lymphomas may arise in any organ, and different histological subtypes occur in distinct patterns. Prognosis and treatment depend not only on the histological subtype and disease extent, but also on the particular involved extra-nodal organ. The clinical course and response to treatment for the more common extra-nodal organs, e.g. stomach, Waldeyer's ring, skin and brain, are fairly well known and show significant variation. A few randomised trials have been carried out testing the role of radiotherapy in these lymphomas. However, for most extra-nodal lymphomas, randomised trials have not been carried out, and treatment decisions are made on small patient series and extrapolations from nodal lymphomas. Hopefully, wide international collaboration will make controlled clinical trials possible in the less common extra-nodal lymphomas. Modern highly conformal radiotherapy allows better coverage of extra-nodal lymphomatous involvement with better sparing of normal tissues. The necessary radiation doses and volumes need to be defined for the different extra-nodal lymphoma entities. The challenge is to optimise the use of radiotherapy in the modern multimodality treatment of extra-nodal lymphomas.     

Primary Extra Nodal Non Hodgkin Lymphoma: A 5 Year Retrospective Analysis

Background and Aim: The incidence of extra nodal non Hodgkin lymphoma (ENL) is rising throughoutthe world. However, data regarding ENL as a group is limited. The aim was to study the epidemiological andhistomorphological trends of primary ENL (pENL) in India. Material and Methods: The biopsy materials fromsixty eight patients with pENL (45 male, 23 female, M:F= 1.9:1), diagnosed over a five year period (2005-2009),were analysed and pathologically reclassified according to the World Health Organization (WHO) classification,2008 criteria. Results: Primary extra nodal non Hodgkin lymphomas constituted 22.0% (68/308) of all nonHodgkin lymphomas (NHL). The mean age at presentation for pENL and primary nodal NHL was 43 years and58 years, respectively with a male predilection (M: F=2:1). Central nervous system (CNS) constituted the mostcommon extranodal site (20/68, 29.5%) followed by gastrointestinal tract (17/68, 25%), and nose/nasopharynx(8/68, 11.8%). Diffuse large B-cell lymphoma (DLBCL, not otherwise specified), extranodal marginal lymphomaof mucosa associated lymphoid tissue (MALT) type, and B cell NHL unclassified (U) were the three most commonhistological types observed. T-cell phenotype was rarely noted (4%). Follicular lymphomas and anaplastic largecell lymphoma, seen among nodal NHL, were absent at extra nodal sites. Majority (41/68, 60%) of the patientswith pENL were immunocompetent and 55% were in stage I-II with favorable prognosis. Conclusion: Centralnervous system was the most common site of ENL, followed by gastrointestinal tract. Majority of pENL occurredin immunocompetent hosts with a favorable prognosis.     

HTLV-I Induced Extranodal Lymphomas

HTLV-I induced not only nodal but also primary extranodal lymphomas. In this report we describe 12 patients with HTLV-I induced extranodal T-cell lymphoma collected from the literature and our institute experience. There were 5 males and 7 female patients of middle age positive for HTLV-1 antibody. The sites of primary tumor were gastrointestinal, Waldeyer's ring, skin, facial sinuses, and the pleura. All of these were histologically diffuse lymphomas and most of them were found to be a helper/inducer T-cell phenotype, showing integration of HTLV-I proviral DNA. Late leukemic changes and skin infiltration often occurred, but hypercalcemia was rare. Survival time varied from 4 to 35 months, and late organ infiltrations were common. These HTLV-I induced extranodal lymphomas were compared with HTLV-I unrelated extranodal lymphomas or HTLV-I induced nodal lymphomas (lymphoma type ATL). Between 1981 and 1990, we had 110 ATL patients and of these, 5 (4.6%) were HTLV-I induced primary extranodal lymphomas. The frequency of HTLV-I induced extranodal lymphoma might be much higher than expected because until now attention has not been paid to this entity. From the present review, it is suggested that HTLV-I could cause primary extranodal lymphoma which may have some different characteristics from other types of lymphoma. Therefore, patients with T-cell extranodal lymphomas should be investigated further for the presence of HTLV-I antibody and the tumor cells should be examined for the integration of HTLV-I proviral DNA using Southern blot analysis. However, this is sometimes difficult to detect with small specimens and in these cases the PCR method for detection of HTLV-I proviral DNA is worthwhile doing. It should be stressed that characterization of a monoclonal T-cell tumoral expansion with the integration of HTLV-I proviral DNA can be done by surface marker studies and gene analysis at the primary sites in patients with T-cell lymphoma and HTLV-I antibody. Further collection of cases with HTLV-I induced primary extranodal lymphoma is necessary in order to elucidate the clinical characteristics of this rare variant of ATL more precisely.     

F-18-fluoro-deoxy-glucose positron emission tomography in the assessment of peripheral T-cell lymphomas

F-18-fluoro-deoxy-glucose positron emission tomography (PET) is highly sensitive and specific in the imaging of B-cell lymphomas. In contrast, its utility in the diagnostic evaluation of T-cell lymphomas is less defined. In this article, we present our finding utilizing PET in peripheral T-cell lymphomas (PTCL). A retrospective review of patients who underwent PET examinations at our institution produced 24 PET examinations among patients with PTCL. A lesion-based analysis was undertaken to evaluate the diagnostic accuracy of PET in PTCL. PET findings were compared with a standard of reference and sensitivity, specificity, positive and negative predictive values were calculated. PET had an overall sensitivity of 86% and specificity of 100%. PET had high sensitivity (95%) at nodal and non-cutaneous extra-nodal sites and poor sensitivity (13%) at cutaneous sites. The mean SUV of abnormal foci in anaplastic large cell lymphoma was 11 mg/ml (range: 3 - 40), and PTCL-unclassified was 8 mg/ml (range: 1 - 23).     

Primary duodenal NK/T-cell lymphoma with massive bleeding: A case report

Primary natural killer/T-cell (NK/T-cell) lymphoma of the gastrointestinal tract is a very rare disease with a poor prognosis, and the duodenum is quite extraordinary as a primary lesion site. Here, we describe a unique case of a primary duodenal NK/T-cell lymphoma in a 26-year-old man who presented with abdominal pain and weight loss. Abdominal computed tomography scan demonstrated a hypodense tumor in the duodenum. Because of massive upper gastrointestinal tract bleeding during hospitalization, the patient was examined by emergency upper gastrointestinal endoscopy. Under endoscopy, an irregular ulcer with mucosal edema, destruction, necrosis, a hyperplastic nodule and active bleeding was observed on the duodenal posterior wall. Following endoscopic hemostasis, a biopsy was obtained for pathological evaluation. The lesion was subsequently confirmed to be a duodenal NK/T-cell lymphoma. The presenting symptoms of primary duodenal NK-/T-cell lymphoma in this patient were abdominal pain and gastrointestinal bleeding, and endoscopy was important for diagnosis. Despite aggressive treatments, the prognosis was very poor.     

Translocation t(14;18)/IGH‐BCL2 in gastrointestinal follicular lymphoma

BACKGROUND:

Chromosomal translocation t(14;18)(q32;q21) involving the immunoglobulin heavy chain gene (IGH) and the BCL2 gene (t[14;18][q32;q21]/IGH‐BCL2) is present in 60% to 90% of nodal follicular lymphomas. To the authors' knowledge, the prevalence and clinical significance of this translocation have not been examined previously in gastrointestinal follicular lymphomas.

METHODS:

Clinicopathologic and molecular features were investigated in 48 patients who had gastrointestinal follicular lymphoma. The site of involvement was the duodenum in 54% of patients, the jejunum in 52%, the ileum in 52%, the stomach in 29%, and the colorectum in 15%. The presence of the t(14;18)/IGH‐BCL2 translocation was detected by interphase fluorescence in situ hybridization.

RESULTS:

Treatment modalities included surgical resection (n = 16), rituximab plus chemotherapy (n = 13), rituximab alone (n = 6), antibiotics (n = 5), and watchful waiting (n = 8). Complete remission (CR) of lymphoma was achieved in 31 patients (65%). The overall survival and event‐free survival rates after 5 years were 93% and 68%, respectively. The t(14;18)/IGH‐BCL2 was detected in 39 patients (81%). The involvement of multiple sites (69% vs 0%), manifestation of the lymphomatous polyposis type (72% vs 22%), and histologic grade 1 or 2 tumors (92% vs 56%) were more frequent in the t(14;18)‐positive group than in the negative group. In addition, the CR rate was lower in the t(14;18)‐positive group than in the negative group (56% vs 100%; P = .0179), and a trend was observed toward poorer event‐free survival in the positive group (P = .089).

CONCLUSIONS:

The t(14;18)/IGH‐BCL2 chromosomal translocation occurred frequently in gastrointestinal follicular lymphomas. The current results indicated that this translocation may be a predictor of an adverse clinical course. Cancer 2011. © 2010 American Cancer Society.     

Immunologic, Clinicopathologic and Prognostic Features of Lymphoma of the Gastrointestinal Tract

The lymphoma cells of 32 patients with histopathologically provenmalignant lymphomas with gastrointestinal (G-I) tract involvementwere examined for their surface marker characteristics. Twelvecases were diagnosed as primary lymphoma of the G-I tract and20 cases as secondary lymphoma of the G-I tract according toclinical and autopsy findings. Only one of the primary G-I tractlymphomas was identified as T-cell type, seven as B-cell typeand four as surface immunoglobulin (S-Ig)-negative non-T type(defective B-cell type). Ten of the 11 cases of non-T cell typewere histopathologically diagnosed as diffuse large lymphoidlymphoma, and one as diffuse medium-sized or poorly differentiatedlymphocytic lymphoma. This suggests that most primary G-I tractlymphoma would be large lymphoid lymphoma, even if it couldbe found at an early stage. The histopathological diagnosisof one case of T-cell type was controversial lymphocyte depletionof Hodgkin's disease or pleomorphic lymphoma is most probable. Three of the 20 secondary G-I tract lymphomas were identifiedas T-cell type, eight as B-cell type, five as defective B-celltype and four as S-Ig-undetermined non-T cell type. In contrastwith the primary G-I tract lymphomas, all histologic types wereincluded in the secondary G-I tract lymphomas. The period fromonset to G-I tract involvement ranged from four to 88 mo withan average of 32.6 mo. The tumor mass in the G-I tract in B-celllymphoma was usually large enough to be able to be detectedclinically, while a large proportion of the G-I tract lesionsin defective B-cell lymphoma and T-cell lymphoma were so smallthat they were detected only at autopsy. These results suggestthat B-cell lymphoma has a higher incidence of G-I tract involvementand a more pronounced capacity to proliferate in the G-I tractthan defective B-cell lymphoma and T-cell lymphoma. The prognosis for the patients with G-I tract lymphoma variedaccording to the stage at the time of the first examination.All three patients with stage I primary lymphoma of the G-Itract are surviving, while only three of nine patients withstage II or higher are still alive. In the case of secondaryG-I tract lymphoma, although the median survival time of stageI patients was fairly long (66 mo). more than 80% of the patientsdied of the disease. Half of the patients with stage I diseasedied within 6 mo and about 40% of the patients died within 1mo after G-I tract involvement. This indicates that secondaryG-I tract involvement of lynaphoma is a poorer risk factor forprognosis than is primary G-I tract lymphoma.     

Follicular T-cell lymphoma mimicking lymphocyte-rich classic Hodgkin lymphoma: a case report of a diagnostic pitfall

Follicular T-cell lymphoma (FTCL), one of the nodal T-cell lymphomas with T follicular helper (T_^FH) phenotype, is an uncommon disease. The diagnosis of FTCL is challenging on the distinction from the morphological mimics mostly exemplified by follicular lymphoma. Here, we described a case of FTCL that mimicked lymphocyte-rich classic Hodgkin lymphoma (LRCHL). A 47-year-old male presented with cervical lymphadenopathy. The biopsy specimen demonstrated nodular lymphoid proliferation, which included scattered CD30+ CD15- CD20- PAX5 weakly+ Hodgkin and Reed-Sternberg (HRS)-like cells and a rich distribution of CD3+ CD4+ PD1+ T-cells. Epstein Barr virus was not detected in HRS-like cells, but it was detected in a small proportion of the scattered lymphocytes. The large cells were also negative for programmed cell death ligand 1, which appeared to be coincidental as described in our previous report of LRCHL. However, flow cytometry showed a CD3- CD4+ T-cell population that constituted 37.4% of all gated lymphocytes. A PCR analysis showed a clonal T-cell receptor-gamma gene rearrangement, but not a clonal immunoglobulin heavy chain gene rearrangement, and showed RHOA G17V mutation. The constellation of these findings led us to revise the diagnosis to FTCL. This result indicated that our case belonged to a relatively indolent subgroup of nodal peripheral T-cell lymphoma of T_^FH phenotype, which affects patients ≤60 years old, recently proposed by our group. This case report expands our understanding of the morphologic spectrum of FTCL and its clinicopathologic significance.