睾丸原发性弥漫性大B细胞淋巴瘤临床病理学分析

目的:探讨睾丸原发性弥漫性大B细胞淋巴瘤(DLBCL)的临床病理特征、免疫表型及治疗方法。方法:回顾性分析23例睾丸DLBCL的病理形态学及免疫组化标记,结合文献对其临床病理学特点进行分析。23例患者年龄48～76岁,平均61.4岁,82.6%患者超过50岁。病变部位左侧睾丸9例,右侧14例,均为单侧发病。临床主要表现为睾丸无痛性进行性肿大。结果:组织学主要表现为肿瘤细胞弥漫浸润于睾丸实质,细胞体积较大,异型性明显,核分裂易见。免疫表型均表达B细胞标志物。5例获得随访资料,4例截止随访时均存活,随访时间2～32个月,1例随访9个月后死亡。结论:睾丸DLBCL少见,多发于老年男性患者,具有侵袭性生物学行为。诊断时极易误诊或漏诊,其确诊依赖组织病理学,免疫组化标记对明确诊断及鉴别诊断有一定价值。     

Diffuse large B-cell lymphoma presenting as a chronic leg ulcer: the importance of repeat tissue biopsy

Ulceration of the leg is often associated with significant consequences for both the individual and society. The diagnosis of chronic leg ulcer is not appropriate. Primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, is a distinct clinicopathological entity. Chemotherapy in the form of R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, Oncovin and prednisolone) is considered to be the first line of treatment for these lymphomas. We report a 69-year-old man who presented with chronic leg ulcer with a first negative biopsy and a diagnosis of PCLBCL, leg type, verified on the subsequent biopsy. This case report emphasises the importance of differential diagnosis of lymphoma in non-healing ulcers and also the value of repeat tissue biopsy in cases with a negative initial result but strong clinical suspicion.     

Diffuse large B-cell lymphoma with extra Bcl-2 gene signals detected by FISH analysis is associated with a "non-germinal center phenotype"

Amplification and translocation of the Bcl-2 gene has been detected in a certain subset of diffuse large B-cell lymphomas (DLBCL). The correlations among Bcl-2 protein expression, gene translocation or amplification, and the molecular signature determined by cDNA array are poorly understood. This study examined 25 cases with de novo nodal DLBCL. Interphase fluorescence in situ hybridization (FISH) analysis was performed to evaluate the Bcl-2 gene using IGH/BCL2 and CEP18 centromere probes (Vysis). When extra Bcl-2 gene signals were observed in each tumor cell and when these signals were in proportion to the extra CEP18 probe signals, we regarded the findings as indicating the presence of an additional chromosome 18; when extra Bcl-2 signals were observed but additional CEP18 signals were not, we regarded the findings as indicating the presence of gene amplification. A panel of 3 antigens (CD10, Bcl-6, and MUM-1) was applied to categorize each case as either a "germinal center B-cell (GCB) phenotype" or a "non-GCB phenotype." Of the 25 cases examined, 8 cases (32%) were classified as "GCB phenotype" and 17 cases (68%) were classified as "non-GCB phenotype." A FISH analysis revealed that t(14;18) was detected in 2 of the 8 cases (25%) with the "GCB phenotype" but in none of the 17 "non-GCB phenotype" cases. Extra Bcl-2 gene signals were detected in 7 of the 25 (28%) cases examined: n = 5 for an additional chromosome 18, n = 1 for gene amplification, and n = 1 for additional chromosome 18 + gene amplification. Extra Bcl-2 gene signals were exclusively detected in DLBCL with the "non-GCB phenotype"; these cases, with the exception of one, stained strongly positive for Bcl-2. The DLBCLs with Bcl-2 protein overexpression were classified into at least two heterogeneous molecular groups, based on the results of the FISH analysis.     

Intravascular large B-cell lymphoma of the breast

The case of an 80-year-old woman with symmetrical breast engorgement and nonspecific systemic symptoms progressively developing over 3 months and confirmed on surgical biopsy to be due to an intravascular large B-cell lymphoma (ILBCL) is presented. To our knowledge, ILBCL has never been reported in the breast before and its mammography and ultrasound appearances are described.     

Primary cardiac diffuse large B-cell lymphoma, non-germinal centre B-cell type in an immunocompetent woman

Primary cardiac lymphomas are extremely rare and always occur in immunodeficient persons. Here, we report a very rare case of a primary cardiac diffuse large B-cell lymphoma in an immunocompetent 41-year old woman. Echocardiography and computed tomography revealed a mass measuring 74 mm 49 mm in the right atrium. No tumour formations were recognized in other organs. Laboratory data did not reveal immunosuppression, and the human immunodeficiency virus was negative. Histological and immunohistochemical studies showed that the cardiac tumour was diffuse large B-cell lymphoma, non-germinal centre B-cell type. Epstein-Barr Virus-encoded small RNA was negative by in situ hybridization. The patient died 6 months after the operation.     

Diffuse large B-cell lymphoma in the liver accompanied by primary biliary cholangitis: A rare and difficult-to-diagnose tumor with portal venous thrombosis

Introduction and importanceThe most common liver malignancies are hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic tumors. Hepatocellular carcinoma and intrahepatic cholangiocarcinoma may invade the portal vein (PV). An association between diffuse large B-cell lymphoma (DLBCL) and primary biliary cholangitis (PBC) remains unclear. We herein report a thought-provoking case of a difficult-to-diagnose liver tumor with PV thrombosis in a PBC patient.Presentation of caseA 66-year-old woman had PBC, systemic sclerosis, diabetes, and osteoporosis. A solitary liver tumor accompanied by macrovascular thrombosis in the PV was detected incidentally. Based on dynamic imaging findings, we considered the tumor to be intrahepatic cholangiocarcinoma, and right lobectomy with lymphadenectomy was performed. Unexpectedly, pathological assessment made a definitive diagnosis of DLBCL that did not invade the vessels and bile duct. In fluorine-18-fluorodeoxyglucose positron emission tomography, abnormal accumulations were clearly observed in the breast tissue and peritracheal, parasternal, mediastinal, and pericardial lymph nodes. The patient achieved complete remission after systemic chemotherapy, and there has been no recurrence 3 years after surgery.Clinical discussionPrimary lymphoma in the liver is rare, and we did not consider our patient's tumor as primary liver lymphoma. Our case actually showed no tumor thrombosis in the PV. Although autoimmune disorders may increase the risk of non-Hodgkin's lymphoma, an association between DLBCL and PBC is still unclear, and we must remember that DLBCL may develop rarely in a PBC patient.ConclusionOur case report provides a timely reminder for clinicians and surgeons in the fields of hepatology and hematology.     

Autologous bone marrow transplant in a patient with sickle cell disease and diffuse large B-cell lymphoma

As the life expectancy of patients with homozygous sickle cell anemia (SCA) improves, SCA care providers are confronted with diseases of the adult SCA population rarely seen before. We report here a 40-year-old woman with SCA who developed diffuse large B-cell non-Hodgkin's lymphoma (NHL) that was treated with eight cycles of chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide (CHOPE), without complete remission. She subsequently underwent high-dose cyclophosphamide and total-body irradiation followed by autologous bone marrow transplantation (BMT). To reduce the risk of sickle cell crisis precipitated by G-CSF, she underwent hypertransfusion to maintain a low % hemoglobin S throughout her treatment course. Although she has required iron chelation therapy and shows no sign of modification of her underlying SCA, she remains in remission from NHL 12 years posttransplant. To our knowledge, this is the first reported case of autologous BMT in a patient with SCA. Our patient illustrates that SCA in itself does not preclude autologous stem cell transplantation for lymphoma in selected patients, and this report should encourage others to consider autologous BMT in adults with SCA where it represents a lifesaving therapy for malignant diseases.     

Primary esophageal diffuse large B-cell lymphoma: Report of a case

Primary esophageal lymphoma is very rare, with fewer than 25 cases documented in the English-language literature. We report a case of primary diffuse large B-cell lymphoma of the esophagus in a 42-year-old woman. Barium esophagogram revealed almost complete esophageal obstruction at the level of the cervical esophagus, and flexible endoscopy showed a circumferential submucosal tumor covered with intact mucosa. Neck magnetic resonance imaging (MRI) showed a wide cervical mass circumferentially encompassing the lumen of the cervical esophagus. Biopsies taken with multiple forceps during flexible and rigid esophagoscopy were nondiagnostic. Finally, external esophageal wall biopsies taken during neck exploration provided information that helped us establish the diagnosis. Pathohistological findings confirmed non-Hodgkin's lymphoma of the diffuse large B-cell type. The patient was treated with combined immunochemotherapy, consisting of rituximab plus cyclophosphamide, vincristine, adriablastin, and prednisone (CHOP), followed by irradiation. A complete response was achieved, and 3 years after diagnosis and treatment the patient was disease-free.     

Diffuse large B-cell lymphomas with plasmablastic differentiation represent a heterogeneous group of disease entities

Plasmablastic lymphoma was initially described as a variant of diffuse large B-cell lymphoma (DLBCL) involving the oral cavity of HIV+ patients and characterized by immunoblastic morphology and a plasma cell phenotype. However, other lymphomas may exhibit similar morphologic and immunophenotypic features. To determine the significance of plasmablastic differentiation in DLBCL and examine the heterogeneity of lymphomas with these characteristics, we examined 50 DLBCLs with low/absent CD20/CD79a and an immunophenotype indicative of terminal B-cell differentiation (MUM1/CD38/CD138/EMA-positive). We were able to define several distinct subgroups. Twenty-three tumors were classified as plasmablastic lymphoma of the oral mucosa type and showed a monomorphic population of immunoblasts with no or minimal plasmacytic differentiation. Most patients were HIV+ and EBV was positive in 74%. Eleven (48%) cases presented in the oral mucosa, but the remaining presented in other extranodal (39%) or nodal (13%) sites. Sixteen cases were classified as plasmablastic lymphoma with plasmacytic differentiation. These were composed predominantly of immunoblasts and plasmablasts, but in addition exhibited more differentiation to mature plasma cells. Only 33% were HIV+, EBV was detected in 62%, and 44% had nodal presentation. Nine cases, morphologically indistinguishable from the previous group, were secondary extramedullary plasmablastic tumors occurring in patients with prior or synchronous plasma cell neoplasms, classified as multiple myeloma in 7 of the 9. Two additional neoplasms were an HHV-8+ extracavitary variant of primary effusion lymphoma and an ALK+ DLBCL. HHV-8 was examined in 39 additional cases, and was negative in all. In conclusion, DLBCLs with plasmablastic differentiation are a heterogeneous group of neoplasms with different clinicopathological characteristics that may correspond to different entities.     

Primary diffuse large B-cell testicular lymphoma: magnetic resonance imaging findings

Testicular lymphoma represents the commonest testicular malignancy in men older than 50 years. MR imaging of the scrotum is an efficient supplemental diagnostic tool in the evaluation of scrotal diseases. We report two cases of primary diffuse large B-cell testicular lymphoma, presented in men over the age of 50 years. MR imaging revealed the presence of a hypointense intratesticular mass lesion on T2-weighted images, strongly and heterogeneously enhancing after gadolinium administration.     

早期原发性胃弥漫大B细胞淋巴瘤的治疗与预后分析

目的探讨早期原发性胃弥漫大B细胞淋巴瘤（DLBCL）的预后影响因素及治疗方式的选择。方法回顾性分析天津医科大学附属肿瘤医院1993年1月至2008年8月间经胃镜活检或手术病理证实的75例早期DLBCL患者的临床病理资料。结果75例患者中接受单纯化疗20例．手术联合化疗55例：两组患者完全缓解率分别为65．0％（13／20）和83．6％（46／55），治疗有效率分别为75．0％（15／20）和92．7％（51／55），5年生存率分别为86．9％和78．7％，差异均无统计学意义（均P〉0．05）。单因素和多因素预后分析显示，国际预后指数（IPI）评分是DLBCL患者的独立预后因素（P〈O．05．HR=11．350，95％CI：1．011-127．371）。结论IPI评分是早期胃DLBCL中的独立预后因素：单纯化疗与手术联合化疗的疗效无显著差异。     

Primary diffuse large B-cell lymphoma of the sigmoid colon

IntroductionExtranodal lymphomas are commonly encountered in the gastrointestinal tract but lymphomas of colon and rectum are rare. Non-Hodgkin lymphoma is the most common type of colonic lymphoma and represents less than 0.5% of colorectal neoplasms. Chemotherapeutical agents are gateway to disease remission and sometimes cure in most patients but surgery may be necessary in emergent situations.Case presentationA 77-year-old male patient presented with abdominal discomfort, constipation, and obstructive defecation symptoms. Radiological imaging revealed a mass in the sigmoid colon extending towards the rectum. Colonoscopy was performed and biopsy of a nearly 10 cm ulcerovegetative lesion was obtained. Histological examination following biopsy revealed it to be a diffuse large B-cell lymphoma of the sigmoid colon. There was no indication for surgery and the patient was referred to medical oncology clinic for chemotherapy treatment.DiscussionNon-Hodgkin lymphoma is a lymphoproliferative disorder with the diffuse large B cell lymphoma (DLBCL) being the most common subtype. The DLBCL subtype is rarely observed in the colon and rectum. Chromosomal abnormalities are involved in the pathophysiology and gene rearrangements lead to adjustments in lymphocyte function and differentiation.ConclusionIn this case report, we present a rare presentation of a Non-Hodgkin lymphoma presenting in the sigmoid colon. The disease can present with nonspecific symptoms and various imaging modalities along with histopathological evaluation is necessary for the correct subtyping of lymphoma. Chemoradiotherapy is key for treatment, and surgery is usually reserved for cases of obstruction, perforation, or bleeding.     

原发性胃弥漫大B细胞淋巴瘤38例临床预后分析

目的探讨原发性胃弥漫大B细胞淋巴瘤(PG-DLBL)的外科手术治疗的意义。方法回顾性分析我院1996年1月至2007年12月经胃镜活检或手术病理证实的38例PG-DLBL患者的临床病理κ料,根据治疗情况分为手术联合化疗组㈦单纯化疗组,比较两组预后情况。结果手术联合化疗17例,单纯化疗21例。两组发病年龄、性别、肿瘤分期、肿瘤大小均无显著性差异,手术联合化疗组的5年生存率为56%,单纯化疗组为33%,差异无统计学意义(P=0.676)。单纯化疗组并发消化道出血1例。手术联合化疗组的完全缓解率为94.1%,单纯化疗组为81.0%(P=0.233)。结论手术不应是PG-DLBL的首选治疗措施。     

Primary cutaneous diffuse large B-cell lymphoma: prognostic significance of clinicopathological subtypes

Classification and subdivision of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) are a matter of ongoing debate. In this study we assessed the morphologic, immunophenotypic, and clinical features of 30 cases of PCDLBCL identified during a review of all primary cutaneous B-cell lymphomas in the Scotland and Newcastle Lymphoma Group database. We also determined the number of cases harboring t(14;18) using a polymerase chain reaction and primers to the major breakpoint cluster region. The effect on prognosis of a variety of clinical and pathologic factors was assessed for the group of 30 PCDLBCL and the 5-year disease-specific survival (DSS) of this cohort compared with that of 195 cases of stage I diffuse large B-cell lymphoma arising primarily in lymph nodes, also identified from within the Scotland and Newcastle Lymphoma Group database. Location on the leg was the only independent prognostic factor for determining outcome in PCDLBCL (67% 5-year DSS compared with 100% for the upper body; P = 0.0047). The presence of multiple lesions, involvement of more than one body site, and expression or not of CD10, bcl-2, bcl-6, and CD10 and bcl-6, had no effect on survival. Compared with cases arising above the waist, those on the leg were more often female, were of an older age, and had a significantly higher incidence of bcl-2 expression (P = 0.002) as well as the aforementioned poorer prognosis. They also showed more frequent co-expression of CD10 and bcl-6, supporting a follicle center cell origin for some, but this difference was not statistically significant. Although there was no significant difference in the 5-year DSS between the group of PCDLBCL and the cases of stage I nodal diffuse large B-cell lymphoma (88% 5-year DSS vs. 78%; P = 0.06), the latter were generally treated with more aggressive therapy. Moreover, a significant difference in 5-year DSS was seen when the nodal DLBCLs were compared with PCDLBCLs arising above the waist (78% vs. 100% respectively; P = 0.0135). These results support the current EORTC approach of subdividing PCLBCL on the basis of site to produce prognostically relevant groupings.     

Mediastinal lymphoblastic lymphoma with an immature B-cell immunophenotype.

A 19-year-old woman presented with a large mediastinal mass, histologically shown to be malignant lymphoma of lymphoblastic type (LBL). Immunophenotypic and gene rearrangement analysis unequivocally demonstrated that the neoplasm was of B-cell lineage. The neoplastic cells expressed terminal deoxynucleotidyl transferase, the pan-B cell antigens CD19, CD20, and CD22, and were negative for immunoglobulins and numerous T-cell antigens tested. Southern blot analysis showed rearrangement of one allele of the immunoglobulin heavy chain gene while the immunoglobulin kappa and T-cell receptor beta chain genes were in the germline configuration. Thus, the immunophenotypic and molecular findings in this case correspond to an early stage of B-cell differentiation, the pre-pre B-cell stage as has been named by others. In contrast with LBL of immature T-cell lineage, precursor B-cell LBLs involving the mediastinum are truly rare. Occasional cases have been reported that have arisen elsewhere and subsequently involved the mediastinum at time of relapse or tumor progression. Well-documented examples of immature B-cell LBL arising in the mediastinum are virtually unreported. The site and cell population giving rise to this neoplasm is unknown. However, origin from precursors of normal thymic medullary B cells is proposed as one possibility.     

Intravascular large B-cell lymphoma presenting with anasarca-type edema and acute renal failure

Abstract

Intravascular lymphoma (IVL) is a rare extra nodal subtype (usually of B-cell origin) presenting with infiltration of large neoplastic lymphocytes into lumina of blood vessels, leading to vascular occlusion. The early diagnosis is very crucial, however it is usually diagnosed postmortem investigation in most of the cases. A 56-year-old female presented with elevated creatinine level, and anasarca-type edema that superimposed with hard, indurated, erythematous plaques extending to inguinal region, abdomen, anterior aspect of chest, and face. B-cell IVL was confirmed with skin biopsy. The patient had some degree of clinical improvement following chemotherapy. B-cell IVL presenting with anasarca edema was not previously reported in the literature. Even if its rarity, IVL should be considered in the differential diagnosis of renal failure with anasarca edema.     

Angiotropic large B-cell lymphoma misdiagnosed as urosepsis with multiple organ dysfunction syndrome

A 53-year-old woman with a history of cervical carcinoma 14 years ago, treated with hysterectomy and radiation therapy, was admitted to the intensive care unit with severe SIRS (systemic inflammatory response syndrome) progressing to shock, multiple organ failure and death within 5 d. Bilateral hydronephrosis diagnosed by sonography and an enlarged left kidney with suspected abscesses verified in a CT-scan suggested the diagnosis of urosepsis. However, multiple microbiological examinations remained sterile. Despite surgical treatment and aggressive intensive care, she died in unresponsive shock. Pathohistologically, an angiotropic large B-cell lymphoma, a rare diffuse intravascular neoplasm of lymphoid origin, was diagnosed. The patient's history of abdominal radiation therapy 14 years earlier as well as multiple negative microbiological specimens in a patient with suspected urosepsis should have initiated the search for a non-infectious cause of the disease.