A clinicopathologic study of malignant lymphomas of the nose, paranasal sinuses, and hard palate, including cases of lethal midline granuloma

Malignant lymphomas of the nose, paranasal sinuses, and hard palate show marked clinicopathologic, immunologic, and prognostic diversity. The clinical features and pathologic spectrum of these lesions were studied in 20 cases (11 female and 9 male cases) with a mean age of 51 years at initial presentation. Malignant lymphomas of the large cell type were most frequently encountered (11/20). The next largest category was malignant lymphoma, diffuse, mixed small and large cell type (six of 20). Two thirds, 13 of 20 cases, had morphologic features suggestive of peripheral T-cell lymphomas. Necrosis, an angiocentric growth pattern, and epitheliotropism were found in nine, eight, and three cases, respectively. Of ten cases immunophenotyped on fresh-frozen or fixed, paraffin-embedded tissue sections, eight had a T-cell phenotype and two had a B-cell phenotype. Of 17 patients with sufficient follow-up data, ten are alive (median follow-up 33 months) and seven are dead (median survival 12 months). Patients with clinical Stages IE and IIE did not have a superior 5-year survival to those with more advanced disease. Histologic type also did not correlate with survival but this may be due to the aggressive histologic grade of the majority of cases and the retrospective nature of this study. The authors conclude that, despite the overall high-grade histologic type, the pathologic spectrum of malignant lymphomas involving this anatomic region is broad. Furthermore, some cases do not fit well into the National Cancer Institute (NCI) Working Formulation but more closely resemble the histologic features of peripheral T-cell lymphomas described in Japan.     

Combination chemotherapy with adriamycin, cyclophosphamide, vincristine, prednisolone (VEPA) in non-Hodgkin's lymphoma, with special reference to correlation of surface phenotype with response and survival

Thirty-seven previously untreated patients with advanced non-Hodgkin's lymphoma were treated with VEPA therapy. The complete remission (CR) rate was higher in the patients with diffuse B-cell lymphoma (75%) than in those with follicular B-cell lymphoma (20%) and T-cell lymphoma (42%). Two characteristics, i.e., elevated LDH and bone marrow involvement, were negatively associated with response rate in patients with diffuse lymphoma (B-, T-). The median duration of CR has not yet been reached, and the 2-year relapse-free rate was 64% for cases of diffuse B-cell lymphoma, while for T-cell lymphoma patients, the median duration of CR was 7 months. For diffuse B-cell lymphoma patients, the median survival has not yet been reached, and the 2-year survival rate was 57%. On the other hand, median survival for T-cell lymphoma patients was 12 months. VEPA therapy was less effective for the treatment of T-cell lymphoma, and a more intensive regimen should therefore be designed to overcome the potential aggressiveness of T-cell lymphoma.     

Prognostic significance of the immunophenotype versus the International Prognostic Index in aggressive non-Hodgkin's lymphoma

The International Prognostic Index (IPI) is currently the most widely accepted prognostic factor system for patients with aggressive non-Hodgkin's lymphoma (NHL). However, in constructing the model, the immunophenotype of the disease was not used as an independent variable. The purpose of the present study was to assess and compare the prognostic significance of the immunophenotype (B-cell vs. T-cell) of aggressive NHL with other well-established prognostic determinants, in particular the IPI. Between January 1995 and December 2000, a retrospective analysis was conducted of clinical and pathological data on 181 patients aged = 15 years who had been newly diagnosed with aggressive NHL. All pathology slides were reviewed and defined according to the Revised European-American Lymphoma classification. Forty-one patients (23%) had T-cell lymphoma and 140 patients (77%) had B-cell lymphoma. Diffuse large B-cell lymphoma and unspecified peripheral T-cell lymphoma were the 2 most common entities, comprising 63% and 14% of patients, respectively. Most of the pretreatment characteristics, including IPI risk groups, were not significantly different between B-cell and T-cell lymphomas. The rates of complete remission (71% vs. 54%, P = 0.038) and progressive disease (39% vs. 63%, P = 0.023) significantly favored patients with B-cell lymphoma. With a median follow-up time of 31 months (range, 10-81 months), the 5-year overall survival (49% vs. 27%; P < 0.001) and event-free survival (35% vs. 10%; P < 0.001) were significantly better in B-cell lymphoma. The 5-year disease-free survival was also in favor of the B-cell group (48% vs. 21%; P = 0.086). Patients with T-cell lymphoma yielded inferior survival in all IPI risk groups. Multivariate analysis revealed T-cell lymphoma as the most significant factor associated with short overall survival (relative risk [RR], 3.4; 95% CI, 1.9-5.9) and event-free survival (RR 2.7, 95% CI, 1.7-4.3). When a second multivariate analysis was done using IPI (age, stage, performance status, number of extranodal sites, and serum lactate dehydrogenase) as one independent variable, T-cell phenotype remained the strongest factor affecting the survival of patients (P < 0.001). T-cell lymphoma is an independent prognostic factor, the significance of which is at least comparable to the IPI for patients with aggressive NHL.     

Non-Hodgkin's lymphoma of Waldeyer's ring and nasal cavity. Clinical and immunologic aspects

Twenty-nine cases of non-Hodgkin's lymphoma of Waldeyer's ring (W-NHL) and nasal cavity or paranasal sinus (N-NHL) were studied for tumor-surface marker phenotype and histopathologic correlation with clinical features. Immunostaining procedures on tissue sections by using xenoantisera and monoclonal antibodies to human B- and T-cells enabled the authors to demonstrate precise surface marker phenotypes of tumor cells and, moreover, the histologic localization of normal or neoplastic B- and T-cells in preserving the original structure of lymphoid organs or tumor tissues. In 22 cases of W-NHL, 19 (86%) had B-cell markers and 3 (14%) had T-cell markers, whereas 6 of 7 cases (86%) of N-NHL had T-cell markers. Tumor cells in T-cell lymphomas in W-NHL and N-NHL reacted with antibodies to peripheral T-cells except one case of W-NHL. Rappaport "histiocytic" subtype was heterogeneous with respect to both surface marker characteristics and morphologic features, i.e., seven had B-cell markers and four had T-cell markers, and they were all subdivided into "large cell" or "large cell, immunoblastic" in Working Formulation and "large cell" or "pleomorphic" in Lymphoma Study Group classification. The actuarial survival curve for all T-cell lymphoma patients was characterized by a rapid initial decline and a subsequent plateau, which contained two of the long survivors. In contrast, the B-cell lymphoma group had a more graded decline. The median and actuarial survivals of the T-cell lymphoma group were far inferior to those for the lymphoma group that expressed B-cell markers.     

Non-Hodgkin's lymphoma: correlation of cell surface marker phenotype with clinical features and prognosis

The correlation of surface marker phenotype with prognosis was analysed in 64 patients with non-Hodgkin's lymphoma who had been treated in Shikoku Cancer Center Hospital. B-cell lymphomas (21 cases) had significantly better prognosis than T-cell lymphomas (21 cases). The complete remission (CR) rate was 52%, and the 50% survival time was 13 months for T-cell lymphomas. All T-cell lymphoma patients died within 31 months. In B-cell lymphomas, on the other hand, the CR rate was 100%, 50% survival time was 30 months, and there were no cases of relapse in patients who had been in continuous CR for more than 2 years. About 40% of B-cell lymphomas appeared to have the potential for cure.     

Large cell non-Hodgkin lymphoma of childhood: Analysis of 78 consecutive patients enrolled in 2 consecutive protocols at the Memorial Sloan-Kettering Cancer Center

BACKGROUND: The authors report a study of pediatric patients with advanced diffuse large cell lymphoma (DLCL) who were treated with 2 consecutive regimens, LSA2-L2 and LSA4, over a 25-year-period at the Memorial Sloan-Kettering Cancer Center. They also describe a comparative analysis of two subgroups retrospectively identified as having CD30 positive (+) anaplastic large cell lymphoma (ALCL) and CD30 negative (-) DLCL. To the authors' knowledge, this study represents the longest follow-up on the largest series of uniformly treated pediatric DLCL patients reported to date. METHODS: A total of 78 consecutive patients were treated for Stage III/IV DLCL. Immunophenotypic data were obtained retrospectively for 52 patients using a panel of monoclonal antibodies against CD30, CD15, CD45, CD45Ro, CD43, epithelial membrane antigen, CD5, BCL-2, cyclin-D, and p53. RESULTS: A disease free survival rate of 72% in patients with advanced stage DLCL using the LSA2-L2 and LSA4 regimens. Of the 78 treated patients, 56 are alive and without evidence of disease with a median follow-up of 120 months (range, 24-312 months). The recurrence rate was significantly higher in the CD30+ ALCL subgroup (33%) than in the CD30- DLCL group (0.04%). Of 52 patients for whom immunophenotypic data were available, 28 had disease of B-cell lineage, 24 had disease of T-cell/null phenotype, 19 were CD30+ (36. 5%), 18 had disease of T-cell phenotype, and 1 had disease of B-cell lineage. CONCLUSIONS: The CD30- DLCL cases mostly were of B-cell lineage, had a small risk of treatment failure, and did not develop a recurrence off therapy. A distinct clinical pattern was identified for the CD30+ ALCL group; although these tumors were of T-cell lineage and had a significantly higher rate of late recurrences (median follow-up of 24 months) they all were salvageable. Based on the findings of the current study, the authors propose that T-cell CD30+ ALCL be addressed in the future according to equal dose intensity regimens in induction therapy, as is done for B-cell lymphomas; prolonged periods of maintenance chemotherapy, as is done for T-cell lymphoblastic lymphomas; and no central nervous system prophylaxis beyond the induction period unless other recognized risk factors are present.     

Bronchial-associated lymphoid tissue lymphoma: a clinical study of a rare disease

Bronchial-associated lymphoid tissue (BALT) lymphoma is a distinct subgroup of low-grade B-cell extranodal non-Hodgkin's lymphoma, classified as marginal-zone lymphoma. This study was performed in order to assess the natural history of this rare entity. We evaluated retrospectively the clinical data of 22 patients with biopsy-proven BALT lymphoma at two tertiary-care institutions from 1996 to 2002. Immunophenotyping was done to confirm the abnormal populations of B-lymphoid cells in all cases, and clonality was determined by flow cytometry or molecular studies. There were 11 men and 11 women in the sample, median age 61 years (range 21-80 years); nine were asymptomatic at diagnosis. All 13 symptomatic patients had non-specific pulmonary complaints. On computed tomographic examination of the chest, 11 patients had bilateral disease, 12 had lung nodules, and 10 had a mass or air-space consolidation. In all but one case the disease was localised to the lung at diagnosis and none had peripheral blood or bone marrow involvement. Out of 22 patients, 20 received treatment in various combinations, 12 had chemotherapy and/or rituximab, six had surgery, and two received radiation therapy as primary treatment. A complete response (CR) was achieved in nine patients and a partial response was obtained in 10 patients. Seven of 10 patients who had unilateral disease achieved a CR. The estimated progression-free survival was 53 months. All patients were alive during the median follow-up period of 36 months (range 12-76 months). It appears that BALT lymphoma tends to be localised to lung at the time of diagnosis, responds well to local or systemic therapy, and has a favourable prognosis.     

Clinical significance of immunophenotype in diffuse aggressive non-Hodgkin's lymphoma

We performed a prospective study of the clinical significance of immunophenotype in 110 patients with aggressive non-Hodgkin's lymphoma (NHL) treated by oncologists in the Nebraska Lymphoma Study Group between October 1982 and May 1986. All patients were immunophenotyped from biopsies performed before therapy was administered. The patients were treated with a uniform protocol of radiotherapy for minimal nonbulky, stage I or II disease (seven patients) or a single, six-drug chemotherapy regimen cyclophosphamide, doxorubicin, procarbazine, bleomycin, vincristine, and prednisone (CAP-BOP) in patients with more extensive disease (103 patients). Ninety-one patients (83%) had B-cell lymphoma and 19 patients (17%) had T-cell lymphoma. The histologic diagnosis of diffuse mixed-cell lymphoma was significantly associated with T-cell immunophenotype (45% v 5%; P less than .001), and the diagnosis of diffuse large-cell lymphoma was significantly associated with B-cell immunophenotype (40% v 5%; P = .006). However, no significant difference in frequency of prognostic variables such as age, stage, systemic symptoms, tumor bulk, serum lactic dehydrogenase, or performance status was found between the B-cell and T-cell groups. Patients with B-cell NHL had a slightly higher complete remission rate (74% v 53%; P = NS), similar durability of complete remission (75% v 70% at 3 years; P = NS), and a slightly but not significantly better overall survival (50% v 41% at 3 years; P = NS). The slight advantage in response rate and survival for B-cell patients was related to a very poor outcome for patients with stage IV T-cell NHL. For patients with stage I to III disease, neither the complete remission rate (B-cell, 82% v T-cell, 91%; P = NS) nor overall survival (3-year survival for B cell, 58% v T cell, 73%; P = NS) were significantly different. However, with stage IV disease B-cell patients fared far better than those with T-cell NHL for both complete remission rate (67% v 0%; P = .002) and overall survival (3-year survival, 44% v 0%; P = .002). Immunophenotyping intermediate- and high-grade NHL allowed identification of a subgroup of patients who had a very poor prognosis with this treatment approach and for whom alternate therapy might be considered.     

Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course.

PURPOSE: To describe and identify the clinical and pathologic features of prognostic significance for natural killer (NK) and NK-like T-cell (NK/T-cell) lymphoma presenting in the skin. PATIENTS AND METHODS: This study was a retrospective review of 30 patients with CD56+ lymphomas initially presenting with cutaneous lesions, with analysis of clinical and histopathologic parameters. RESULTS: The median survival for all patients was 15 months. Those with extracutaneous manifestations at presentation (11 patients) had a shorter median survival of 7.6 months as compared with those without extracutaneous involvement (17 patients), who had a more favorable median survival of 44.9 months (P =.0001). Age, gender, extent of cutaneous involvement, and initial response to therapy had no statistically significant effect on survival. Seven patients (24%) had detectable Epstein-Barr virus (EBV) within neoplastic cells. The patients with tumor cells that coexpress CD30 (seven patients) have not yet reached a median survival after 35 months of follow-up as compared with those with CD30- tumor cells (20 patients), who had a median survival of 9.6 months (P <.02). Routine histopathologic characteristics had no prognostic significance nor did the presence of CD3epsilon, EBV, or multidrug resistance. CONCLUSION: NK/T-cell lymphoma is an aggressive neoplasm; however, a subset with a more favorable outcome is identified in this study. The presence of extracutaneous disease at presentation is the most important clinical variable and portends a poor prognosis. The extent of initial skin involvement does not reliably predict outcome. Patients from the United States with NK/T-cell lymphoma presenting in the skin have a low incidence of demonstrable EBV in their tumor cells. Patients with coexpression of CD30 in CD56 lymphomas tend to have a more favorable outcome.     

Ki-1-positive large cell lymphomas, a heterogenous group of neoplasms. Morphologic, immunophenotypic, genotypic, and clinical features of 24 cases.

Clinical and pathologic features of 24 patients with large cell lymphomas that expressed the activation antigen Ki-1 are described. Phenotypic and/or genotypic studies characterized these neoplasms as T-cell (16 cases), B-cell (six cases), or null cell (two cases) type. Males predominantly were affected. Age of patients ranged from 19 to 73 years, with a bimodal distribution, with peaks in the third and seventh decades. Lymphadenopathy was present in nearly all patients. Extranodal involvement, including skin, soft tissue, bone, central nervous system, lung, or small intestine was observed in a total of 54% of the patients, either at presentation or during the course of disease. "Prototypic" features of large cell anaplastic lymphomas were observed for eight T-cell lymphomas, with morphologic heterogeneity noted for the remainder. Eight patients, all with T-cell neoplasms (only one with prototypic morphology), have died of lymphoma (median survival, 5 months). An antecedent history of a lymphoproliferative disorder (mycosis fungoides, B-cell lymphoma, immunoblastic lymphadenopathy) was apparent in seven patients. An 8-year history of Crohn's disease occurred in one patient with a T-cell lymphoma involving small intestine. Phenotypically, loss of one or more markers was typically noted for T-cell neoplasms. Leukocyte common antigen was detected in all cases, although partial loss of immunoreactivity was noticed in some cases. Nearly all cases evaluated for Ia antigen or alpha-1-antichymotrysin were reactive. Eleven of 16 T-cell, two of six B-cell, and two null cell lymphomas expressed epithelial membrane antigen. Ki-1-positive large cell lymphomas are characterized by clinical, morphologic, and immunophenotypic heterogeneity.     

Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: a systematic analysis of 156 patients reported in the literature

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon type of skin lymphoma. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined. METHODS: The authors performed a systematic analysis of all patients with SPTCL reported on in the English-language medical literature, with emphasis on specific clinical features, experiences involving the use of radiotherapy and systemic agents, and prognostic factors predictive of treatment response and clinical outcome. RESULTS: One hundred fifty-six patients with SPTCL were identified in the literature. Hemophagocytic syndrome (HPS) was a presenting feature in 37% of patients, and > 90% of patients required treatment at diagnosis. Prednisone was used frequently as initial therapy in patients who had less aggressive disease at presentation; however, durable complete remissions (CR) were infrequent. Anthracycline-based chemotherapy regimens were the most commonly used and most effective systemic treatment options, producing long-term CR in approximately 30% of patients. Among patients who received high-dose chemotherapy and stem cell transplantation (HDT-SCT) for refractory or recurrent disease, 92% achieved CR, with a median response duration of > or = 14 months. The presence of HPS at diagnosis and expression of the gamma/delta T-cell receptor (TCR) by tumor cells were associated with poor survival, whereas age was not. After a median follow-up of 24 months, 48% of patients died of disease. The median survival duration was 27 months. CONCLUSIONS: SPTCL has an aggressive natural history. Nonetheless, a subgroup of patients with SPTCL can have long-term disease remission following anthracycline-based initial therapy or subsequent HDT-SCT. HPS and the TCR phenotype may be useful prognostic markers for patients with this malignancy.     

Clinical relevance of immunophenotype in a retrospective comparative study of 297 peripheral T-cell lymphomas, unspecified, and 496 diffuse large B-cell lymphomas: experience of the Intergruppo Italiano Linformi

BACKGROUND: To assess the impact of T-cell/B-cell phenotype on clinical outcome, the authors retrospectively compared patients who had peripheral T-cell lymphoma, unspecified (PTCL-U), with patients who had diffuse large B-cell lymphoma (DLBCL). METHODS: Two hundred ninety-seven cases of PTCL-U and 496 cases of DLBCL that had been transferred from the files of the Intergruppo Italiano Linfomi or the Gruppo Italiano Linfomi were integrated into a unique working file and reviewed by the authors. RESULTS: The PTCL-U group and the DLBCL group had significantly different distribution patterns with respect to patient age, gender, disease stage, performance status (PS), the presence or absence of systemic "B" symptoms, the presence or absence of bulky disease, lactic acid dehydrogenase (LDH) levels, and number of extranodal sites (ENS). A significantly greater number of patients in the DLBCL group experienced complete remission (P < 0.0001). Multinomial logistic regression analysis confirmed that immunophenotype, PS, LDH concentration, and number of ENS were independent predictors of response. At a median follow-up duration of 43 months, there was no observable difference in disease-free survival (DFS) between patients with DLBCL and patients with PTCL-U; however, multivariate analysis did reveal that poorer PS and bone marrow involvement were significantly associated with shorter DFS. Furthermore, although the overall survival (OS) curves associated with the T-cell and B-cell immunophenotypes were significantly different from each other at a median follow-up duration of 37 months (P = 0.0012), Cox multivariate analysis excluded immunophenotype from the final OS model. CONCLUSIONS: The findings made in the current study indicate that the natural history of PTCL-U may differ from that of DLBCL. Patients with PTCL-U tended to have less favorable clinical outcomes, although the observed difference in outcome was only partially attributable to immunophenotype, which was independently associated with response, but not with survival. Differences in prognostic factor distributions between patients with PTCL-U and patients with DLBCL may account for some portion of the expected phenotype-associated risk.     

Clinical features associated with transformation of cerebriform T-cell lymphoma to a large cell process

Some patients with cerebriform T-cell lymphoma (CTCL) undergo morphologic transformation to a large cell lymphoma. From a series of 113 patients with CTCL, 22 patients were identified with transformed CTCL. Stages of involvement at diagnosis were: I (seven), II (four), III (four), IV (seven). Nine patients had transformation at the initial diagnosis while the median time from diagnosis to transformation in the other 13 patients was 16 months (range: 3 months-6 years). Thirteen had transformation extracutaneously: lymph nodes (eight), central nervous system (two), and other extranodal sites (three). T cell markers were identified in all cases; of 15 cases with complete phenotypes, there were eight T-helper, three T-suppressor, and four aberrant T phenotypes. Serology for human T-leukemia virus-I (HTLV-I) was negative in eight patients tested. Median survival from diagnosis was 27 months compared to 53 months in 53 patients without transformation (p = 0.003). Despite combination chemotherapy in 12 patients following transformation, median survival after transformation was 12 months and only 7 months with extracutaneous disease. The likelihood of transformation could not be predicted by the initial histology, immunophenotype, or stage of disease.     

Proliferation rate and outcome in children with T-cell rich B-cell lymphoma: a clinicopathologic study from the NHL-BFM-study group

T-cell rich B-cell lymphoma (TCRBCL) is considered a rare variant of aggressive B-cell lymphoma characterized by few neoplastic cells and a large reactive infiltrate with striking similarities to nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL). In childhood, these tumors occur even less frequently. Biopsy specimens at diagnosis from 16 children (13 boys) with a median age of 12 years (range, 7 to 18) were immunophenotyped. The proliferation rate was assessed with monoclonal antibodies against Ki-67 and repp86 antigens and additional clonality analyses were performed. Ten patients had localized disease and only two had B symptoms. All patients showed high Ki-67 expression (median: 80%, range 40% to 90%), but low repp86 expression (median: 25%, range 10% to 50%). PCR-based clonality studies of the hypervariable CDR III region of the immunoglobuline heavy chain and the T-cell receptor γ-chain genes demonstrated predominant clones in nine and five patients, respectively. Three patients had previous or concomitant NLPHL and two of them received initial treatment for Hodgkin's disease. All patients achieved remission after a brief polychemotherapy regimen. Two patients subsequently relapsed and one was rescued by salvage therapy including autologous stem cell transplantation. At a median follow-up of 23 months, 15 patients (94%) are alive. The striking contrast between the proliferation rates determined by Ki-67 and repp86 expression points to a possible arrest in the G₁ cell cycle phase because repp86 expression is restricted to the S, G₂ and M cell cycle phases. This G₁ arrest may explain the paradoxon of high Ki-67 expression in a paucicellular lymphoma with a favorable prognosis in young patients.     

Cutaneous lymphomas other than mycosis fungoides: follow-up study of 52 patients

Cutaneous lymphomas other than mycosis fungoides (MF) represent a rare and heterogeneous group of lymphomas. Their clinical behavior remains largely unknown. In this study, the clinical and immunohistologic characteristics and follow-up data of 52 well-documented cases of cutaneous lymphomas other than MF, presenting with initial cutaneous lesions, were reviewed. Twenty-seven patients presented with skin disease alone (stage IE), and 25 patients had concurrent cutaneous and extracutaneous disease (stage IV). The tumors were grouped into high-grade lymphomas (HGLs; 21%), intermediate-grade lymphomas (IGLs; 58%), and low-grade lymphomas (LGLs; 21%). A B-cell phenotype was most often expressed by cutaneous lymphomas (73%), particularly by stage IE lymphomas (85%). Among 13 cases of T-cell lymphomas, loss of one of the pan-T-cell antigens was detected in all cases but one. The clinical course of cutaneous lymphoma was closely dependent on stage and histologic subtype but not on T-cell or B-cell phenotype. Of 20 patients with stage IV HGL or IGL, 13 were treated by polychemotherapy with curative potential. Their median survival was 37 months. Fourteen patients with stage IE HGL or IGL were treated by radiotherapy alone. Nine patients (69%) relapsed within 2 years posttreatment. Seven of them relapsed in the skin outside the initial site involved, suggesting that radiotherapy alone is not an adequate treatment for these patients. Preliminary results concerning seven other patients with stage IE IGL or HGL treated by an initial third-generation polychemotherapy regimen are presented.     

Breast lymphoma: favorable prognosis after treatment with standard combination chemotherapy

PURPOSE: This paper is to determine the clinicopathological features and outcome of patients with breast lymphoma seen at a single institution. PATIENTS AND METHODS: We have reviewed data on 14 patients with breast lymphoma seen at our institution from 1990 to 2003. RESULTS: All patients were female, with a median age of 47.6 years. Diffuse large B-cell lymphoma (DLBCL) was observed in 9 cases, while follicular, Burkitt's, small lymphocytic, MALT and T-cell lymphoma were observed in 1 case each. 5 patients (35.7%) had stage IE disease, 6 patients (42.9%) had stage IIE disease and 3 patients (21.4%) had stage IV disease. Standard CHOP with or without rituximab was given to all patients with aggressive breast lymphoma (n = 10), while 1 patient with Burkitt's lymphoma received a CHOP-based regimen. The 3-year actuarial survival estimate among all 11 patients with aggressive breast lymphoma was 73%. Among those with localized disease, the estimated 3-year survival was 90%. The actuarial 3-year overall survival (OS) estimate for the entire cohort of 14 patients was 76.9%. CONCLUSION: Our results indicate that breast lymphoma is not associated with an inferior outcome when treated with standard CHOP-based chemotherapy.     

14例原发性鼻窦非霍奇金淋巴瘤的临床分析

背景与目的:原发性鼻窦非霍奇金淋巴瘤(primary paranasal sinus lymphoma,PPSL)临床上较罕见,其生物学行为较独特.本文总结和分析PPSL的临床病理特点和治疗情况,以期增加对此病的认识.方法:回顾性分析1994～2006年中山大学肿瘤防治中心收治的14例PPSL,分析其临床和病理特点、治疗经过和随访结果.结果:14例中原发于上颌窦者11例,筛窦2例,蝶窦1例.根据淋巴瘤Ann Arbor分期,所有患者均为Ⅰ～Ⅱ期.根据美国癌症联合委员会(AJCC)的鼻窦实体瘤TNM分期标准,大部分患者(11/14)属于局部晚期T3～T4.病理类型:B细胞性12例(85.7%),其中弥漫大B细胞性占42.9%(6例);T细胞性1例,非B非T细胞性1例.2例接受上颌窦根治术,12例为肿物切除或活检;14例全部接受化疗,6例化疗后接受局部放疗.中位生存期59.5个月,全组5年总生存率和无事件生存率均为78.6%.结论:PPSL发病部位以上颌窦多见,病理类型以B细胞性为主,其中弥漫大B细胞性最常见,肿瘤局部侵犯广泛为其重要特征;采用全身化疗和局部放疗联合治疗,患者总体预后相对较好.     

Prognostic implications of karyotype and morphology in patients with non-Hodgkin's lymphoma

Clonal chromosome abnormalities were observed in 30 patients with non-Hodgkin's lymphoma; the type of lymphoma was characterized on the basis of the International Working Formulation. The 30 patients were classified into five groups according to the chromosome abnormality. There were 8 patients with t(14;18), 3 with t(8;14), 7 with a translocation to the long arm of chromosome 3 (a 3q+ chromosome), 5 with near-tetraploidy, and 7 with other abnormalities. Among the 8 patients with t(14;18), 5 had follicular small cleaved-cell lymphoma (FSC), I had follicular mixed cell lymphoma (FM), and 2 had diffuse large-cell lymphoma (DL); the diagnosis in these 2 patients was based on extranodal tissue. All 3 patients with t(8;14) had DL and B-cell markers. Except for 1 patient, all those with a 3q+ chromosome had DL; 4 of those who were tested had B-cell or pre-B-cell markers. Four of the 5 patients with near-tetraploidy had follicular mixed-cell lymphoma, and 2 of the 7 patients with other abnormalities had T-cell lymphoma. Thus, patients with a t(8;14), a 14q+ chromosome, or a 3q+ chromosome all tend to have diffuse large-cell lymphoma, usually of the non-cleaved type. On the other hand, our data suggest that patients with FSC generally have a t(14;18) whereas those with follicular and diffuse mixed small cleaved cells and large noncleaved cells have a different pattern with modal chromosome numbers in the tetraploid range. We added 17 previously reported patients to the 30 presented here and correlated the karyotype with survival. The 6 patients with near-tetraploidy had the longest median survival, 69 months, the 15 patients with t(14;18) had the next longest, 48 months. The 4 patients with t(8;14) had the shortest survival, 12 months, and the 9 with other abnormalities had the next shortest, 17 months. Intermediate survivals of 27 and 30 months were observed in patients with a 14q+ or a 3q+ chromosome, respectively. The median survival of these various categories differs and our data, thus, indicate that the karyotypic pattern of the malignant cell may be a significant independent prognostic feature influencing the survival of patients with non-Hodgkin's lymphoma.     

Primary thyroid lymphoma: the 40 year experience of a UK lymphoma treatment centre

We report the 40-year unselected experience of a UK lymphoma treatment centre. Between 1970 and 2010, 3363 cases of non-Hodgkin lymphoma were managed by the Sheffield Lymphoma Team. Seventy cases of primary thyroid lymphoma were identified during this time. This retrospective review of the clinical and pathological features of patients with thyroid lymphoma comprises one of the largest series conducted in the UK. The series included 57 females and 13 males with a median age at diagnosis of 69.5. The pathological subtypes were diffuse large B-cell lymphoma (DLBCL) in 50 patients, MALT lymphoma in 13, indolent B-cell lymphoma not otherwise specified (NOS) in 6 and T cell lymphoma in one patient. Of the 64 patients fully staged, 53 had Stage IE and 11 Stage IIE disease. Management modalities included surgery, chemotherapy, radiotherapy or combination treatment. Five- year survival rates for DLBCL, MALT lymphoma and indolent B-cell lymphoma NOS were 45%, 62% and 75%, respectively, with a median overall survival of all histological subtypes of 68 months (range 0-148) or 5.7 years. The outcomes of this series confirm previous experience. If treatment is needed after surgery radiotherapy alone is sufficient for Stage I and II low grade thyroid lymphoma. Combination chemotherapy or adequate chemotherapy followed by radiotherapy is warranted in high grade thyroid lymphoma.     

Recent thymic emigrants and prognosis in T- and B-cell childhood hematopoietic malignancies

The concentration of T-cell receptor rearrangement excision DNA circles (TRECs) in peripheral blood mononuclear cells (PBMCs) is currently known to be a marker of recent thymic emigrants. We evaluated the hypothesis that TREC values would be lower in childhood T-cell hematopoietic malignancies than in childhood B-cell acute lymphoblastic leukemia (ALL) or healthy controls because the former category may reflect compromised thymic function. From the Greek national childhood leukemia/lymphoma database we obtained all 30 available T-cell leukemia/non-Hodgkin's lymphoma cases, 30 age- and sex-matched childhood B-cell origin cases of ALL and 60 healthy hospital controls. We compared TREC levels in PBMCs using a real-time PCR assay. There was highly significant reduction of TREC values in children with T-cell malignancies (median 3,100 TRECs/10(6) PBMCs), whereas children with B-cell origin ALL had slightly but nonsignificantly lower TREC values compared to healthy children (medians 19,300 and 22,500 TRECs/10(6) PBMCs, respectively). During a median follow-up period of about 19 months, only 4 children died. All of them had a T-cell hematopoietic malignancy and relatively low TREC values. The number of TRECs was higher among healthy girls than among healthy boys, and a similar pattern was evident in T-cell malignancies. It appears that there is a pattern of concordance of high TREC values with better disease prognosis in hematologic childhood malignancies. This applies to specific disease entities with better prognosis (B-cell origin ALL having higher TREC values than T-cell leukemia/lymphoma) and to gender, another important predictor of prognosis conditional on disease entity (girls having higher TREC values than boys); however, it may also be true for the survival of individual patients. These preliminary findings can be used as hypothesis-generating indications that should be confirmed in larger data sets.