Acute renal failure due to obstruction in Burkitt lymphoma

 Acute renal failure in Burkitt lymphoma is commonly the result of tumor lysis syndrome. We present a 15-year-old boy who developed hypertension, seizures, and acute renal failure due to extrinsic compression of the bladder and ureters by a large retrovesical Burkitt lymphoma. The causes of acute renal failure in Burkitt lymphoma and the incidence of acute urinary obstruction in this disease are reviewed. Received: 18 May 1998 / Revised: 30 June 1998 / Accepted: 1 July 1998     

Florid granulomatous reaction in Epstein-Barr virus-positive nonendemic Burkitt lymphomas: report of four cases

Epithelioid cell granulomas have been reported in association with a wide range of neoplasms including malignant lymphomas. In lymphoma, this refers mainly to Hodgkin disease and T-cell-derived non-Hodgkin lymphomas where a granulomatous reaction is probably evoked by aberrant cytokine production in the tumor cells or other cells composing the tumor background. Here we report on four cases of sporadic Burkitt lymphoma with unusual florid granulomatous reaction. In all samples, the tumor cells were admixed with numerous epithelioid cells that formed clusters and granulomatous lesions. No microorganisms could be detected at the tissue level, and there were no clinical or laboratory indications of an underlying immunodeficiency. The lymphomas harbored a specific morphology and immunophenotype of Burkitt lymphoma, and the presence of translocation breakpoint in MYC gene was confirmed by interphase fluorescence in situ hybridization. In all four patients, the lymphoma was associated with Epstein-Barr virus infection, detected by EBER in situ hybridization and the latency I phenotype as defined by lack of expression of LMP1. All four patients were treated with polychemotherapy, achieved a complete remission, and are alive without evidence of disease. We draw attention to this unusual phenomenon as it caused difficulties in interpretation and delayed diagnosis and hypothesize on the possible role of Epstein-Barr virus products.     

Burkitt's lymphoma of medial part of clavicle: Case report of unusual localization

IntroductionBurkitt lymphoma is a form of non-Hodgkin's lymphoma in which cancer starts in immune cells called B-cells. Recognized as a fast-growing human tumor, it is considered as a medical emergency requiring immediate diagnostic and therapeutic intervention. However, intensive chemotherapy can achieve long-term survival in more than half the people with Burkitt lymphoma.Case reportWe report a case of Burkitt's lymphoma of medial part of clavicle was diagnosed in a 60 years year-old man and review the literature concerning the clinical features, radiological appearance, histopathological findings and treatment options.DiscussionClinical course of Burkitt lymphoma is aggressive and rapid, commonly occurs in children and young adults with frequent involvement of Bone Marrow, associated with impaired immunity and is rapidly fatal if left untreated, early diagnosis can be life saving for Burkitt lymphoma. As per the literature available this is the second case of Burkitt's lymphoma presenting primarily in medial part of clavicle.     

精囊原发性Burkitt淋巴瘤1例报告并文献复习

目的:报告精囊原发性Burkitt淋巴瘤(BL)的临床病理特征。方法:对1例精囊原发性BL的临床特征、组织学、免疫组织化学和原位分子杂交进行观察,并结合文献探讨其病理形态及鉴别诊断。结果:本例临床表现以尿频和排尿困难为特征,发病2个月后出现腹股沟淋巴结肿大。影像学显示病灶精囊呈低密度阴影,组织学呈弥漫单一性中等大小肿瘤细胞浸润性生长伴肿瘤性坏死,核分裂易见并有"星空"图象。免疫标记肿瘤细胞表达CD10、CD20、CD79α和Bcl-6,而CD3、CD5、CD23、MPO、Bcl-2、CyclinD1和TdT缺乏表达,肿瘤细胞Ki-67增殖指数>95%,原位杂交检测肿瘤细胞EBER阳性。结论:精囊原发性BL是一种非常罕见的高度恶性的肿瘤,病程进展快,诊断时应除外精囊外BL累及,确诊依赖病理组织学和实验室检查,并需要与精囊或前列腺发生的弥漫性大B细胞性淋巴瘤、淋巴母细胞淋巴瘤和小细胞癌鉴别。     

Burkitt lymphoma arising in organ transplant recipients: a clinicopathologic study of five cases

We report five cases of Burkitt lymphoma arising in organ transplant recipients. There were four men and one woman with a mean age of 35 years. All were solid organ recipients with three renal, one liver, and one double lung transplantation. The time interval between organ transplantation and lymphoma averaged 4.5 years. Patients typically presented with high-stage disease with generalized lymphadenopathy and bone marrow involvement. Histology showed classic Burkitt lymphoma or atypical variant/Burkitt-like morphology. C-MYC rearrangement, including three cases with immunoglobulin heavy chain and two cases with lambda light chain, and Epstein-Barr virus were detected in all the cases. Additional chromosomal abnormalities were present in two of three cases and p53 mutation was found in one of three cases. Aberrant genotype and phenotype were frequently encountered, including minor monoclonal or oligoclonal T-cell populations and undetectable surface immunoglobulin light chain expression. Four patients received antilymphoma regimens, with combination chemotherapy (three patients) and/or Rituximab (three patients), in addition to reduction of immunosuppression. All four patients achieved complete remission. We conclude that posttransplant Burkitt lymphoma represents a characteristic clinicopathologic entity and occurs later after transplantation. Genotypic and phenotypic aberrations are often present. Rituximab may be an effective alternative to conventional combination chemotherapy in the treatment of a posttransplant Burkitt lymphoma.     

Clinical implications and surgical management of intussusception in pediatric patients with Burkitt lymphoma

Background/Purpose

Intussusception as a presenting feature of Burkitt lymphoma may be associated with early stage disease, which is curable with less intensive therapy. We determined the incidence, presentation, stage, and outcome of children with Burkitt lymphoma presenting with intussusception.

Methods

The medical records of patients with Burkitt lymphoma treated at our hospital from 1962 to 2005 were reviewed, and the patients presenting with intussusception were then further analyzed.

Results

Of 189 patients with primary abdominal Burkitt lymphoma, 33 (17.5%) presented with intussusception. Their median age at diagnosis was 10 years (range, 3-19 years). Most patients presented with abdominal pain (88%) and/or nausea/vomiting (42%). Twenty-three (70%) of these 33 patients as compared with 10 of the other 156 patients with abdominal lymphoma could have complete resection of their tumor (P < .0001) and hence had low stage disease (stage II). Only 10 patients with intussusception had stage III (n = 7) or stage IV (n = 3) unresectable disease. Twenty-five of the patients remained alive in continuous complete remission for 3 months to 31 years (median, 14 years).

Conclusion

Pediatric patients with Burkitt lymphoma presenting with intussusception often have completely resectable disease and are older than general pediatric patients with intussusception.     

Successful treatment of a bowel perforation after chemotherapy for Burkitt lymphoma

Chemotherapy-induced intestinal perforation after treatment for Burkitt lymphoma is a known potential complication. However, there are very few reports in the pediatric literature that discuss this complication in any detail, and most incidents are secondary to surgical complications such as anastomotic leaks. Furthermore, there are no reports of children that have survived chemotherapy-induced perforation, and thus, guidelines for the diagnosis and treatment of this complication or for the continuation of chemotherapy are lacking. We present a case of a 5-year-old boy who survived jejunal perforation after treatment for his Burkitt lymphoma, and the critical aspects of his care are discussed.     

伯基特淋巴瘤化疗后合并巨细胞病毒视网膜炎一例

目的提高对伯基特淋巴瘤化疗后合并巨细胞病毒视网膜炎的认知。 方法分析1例58岁伯基特淋巴瘤化疗后合并巨细胞病毒视网膜炎患者的诊治过程,并结合相关文献进行复习。 结果该例伯基特淋巴瘤患者多疗程化疗后病情缓解,缓解后出现长期营养不良,抵抗力低下,免疫重建受损,诱发巨细胞病毒感染并引起巨细胞病毒视网膜炎,经更昔洛韦注射液抗病毒治疗后巨细胞病毒DNA低于检测下限,视力逐渐改善。 结论及时确诊及规范治疗是控制免疫化疗后合并巨细胞病毒视网膜炎病情及改善预后的关键。     

Burkitt lymphoma masquerading as cardiac tamponade

A 61 year old man presented with diffuse large B cell lymphoma of the skin of the back of the shoulder which was excised and treated with chemotherapy (CHOP regime) in 1998. He was in complete remission till he presented in 2002 with extranodal marginal zone lymphoma of the parotid gland for which he underwent superficial parotidectomy and radiotherapy. He continued in remission till 2006 when he presented with recurrent pericardial effusion and tamponade. At median sternotomy, pericardial effusion was drained, an anterior pericardiectomy was done and a left posterior pericardial window made, and an enlarged hard paraaortic lymph node excised. Histology, immunocytochemistry and chromosome analysis revealed Burkitt lymphoma. Patient underwent chemotherapy with CODOX-M regime and continues in remission. This report is unusual on account of the highly atypical presentation of Burkitt lymphoma as cardiac tamponade, only a few cases having been reported previously, the occurrence of three lymphomas of different pathological and genomic profiles in one patient over a period of eight years and the relatively slow rate of growth of an otherwise fulminant tumour with high tumour doubling time. A review of literature with special emphasis on chromosomal diagnosis, transformation of other lymphomas into Burkitt lymphoma and mediastinal and cardiac involvement with Burkitt lymphoma is presented.     

Primary burkitt lymphoma of the brain in an immunocompetent patient. Case report

Primary central nervous system (CNS) lymphoma is rare and is most often seen in immunodeficient patients. The majority of these tumors are the non-Hodgkin type and are high grade. Primary Burkitt lymphoma of the CNS in immunocompetent individuals has rarely been reported. The authors treated a 49-year-old woman who presented with left-sided weakness that had lasted 1.5 months. Magnetic resonance imaging revealed an oval mass in the left parietal region. with central necrosis and peripheral edema, and no attachment to the leptomeninges or ependyma. Pathological examination yielded a diagnosis of typical Burkitt lymphoma. Six months postoperatively, the patient is ambulatory and has improving neurological signs. This is a typical case of primary Burkitt lymphoma of the brain in an immunocompetent patient, which is a very rare event. The imaging pattern of the lesion is not typical of brain lymphomas and can result in an incorrect preoperative diagnosis of other brain tumors, such as glioblastoma multiforme. The patient's treatment and follow-up review are discussed.     

男性泌尿生殖系统淋巴瘤临床病理分析

目的:探讨男性泌尿生殖系统淋巴瘤的临床病理特征和免疫表型。方法:回顾性分析35例男性泌尿生殖系统淋巴瘤的病理形态学和免疫组化标记,结合文献对其临床病理特点进行分析。结果:35例淋巴瘤患者的年龄4～83岁,平均年龄56.5岁,≥50岁者28例(80%)。发生于睾丸者最多,28例(80%),其次是前列腺3例(8.6%),精索、精囊、阴茎和附睾各1例(各2.9%)。组织学类型包括:弥漫性大B细胞淋巴瘤(DLBCL)22例(62.9%)、粘膜相关淋巴组织(MALT)淋巴瘤6例(17.1%)、Burkitt淋巴瘤4例(11.4%)、外周T细胞淋巴瘤2例(5.7%)和浆细胞瘤1例(2.9%)。结论:男性泌尿生殖系统淋巴瘤少见,多发生于中老年人,大多是B细胞淋巴瘤,以DLBCL最常见,其次是MALT淋巴瘤、Burkitt淋巴瘤,T细胞淋巴瘤和浆细胞瘤罕见,男性泌尿生殖系统淋巴瘤的确诊依赖组织病理学,免疫组化标记对明确诊断、分型和鉴别诊断具有肯定价值。     

Comprehensive Assessment and Classification of High-Grade B-cell Lymphomas

High-grade B-cell lymphomas (HGBCLs) are a heterogeneous group of neoplasms that include subsets of diffuse large B-cell lymphoma, Burkitt lymphoma, and lymphomas with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. Morphologically indistinguishable HGBCLs may demonstrate variable clinical courses and responses to therapy. The morphologic evaluation and classification of these neoplasms must be followed by further genetic and immunophenotypic work-up. These additional diagnostic modalities lead to a comprehensive stratification of HGBCL that determines the prognosis and optimal therapy. This article reviews the well-established and emerging biomarkers that are most relevant to the clinical management of HGBCL.     

Long-term survival of renal graft complicated with Burkitt lymphoma

A 10-year-old boy with steroid-resistant nephrotic syndrome developed disseminated Burkitt lymphoma 2 years after renal transplantation. Treatment consisting of reduction of immunosuppression and polychemotherapy was initiated, and induced complete tumor remission. A severe cerebellar syndrome attributed to high-dose cytarabine occurred during treatment. The patient recovered partially from this complication. Immunosuppression had to be resumed 2 years later because of a chronic rejection. Finally, at last follow-up, the patient was alive with a stable creatinine of 180 micromol/l.     

Atypical Burkitt lymphoma arising from follicular lymphoma: demonstration by polymerase chain reaction following laser capture microdissection and by fluorescence in situ hybridization on paraffin-embedded tissue sections

A 77-year-old man with disseminated lymphadenopathy was referred to our hospital. An inguinal lymph node biopsy specimen had the appearance of composite lymphoma consisting of follicular lymphoma and atypical Burkitt lymphoma. The bone marrow was involved, and chromosomal analysis of the marrow showed t(8;14) and t(14;18) in identical cells. The patient underwent systemic chemotherapy but died of lymphoma 4 months later. Polymerase chain reactions performed on cells obtained from follicular and diffuse components by laser capture microdissection showed the two components to be clonally identical. Moreover, fluorescence in situ hybridization performed on paraffin-embedded tissue sections demonstrated an immunoglobulin heavy chain (IGH)/BCL2 translocation signal in both follicular and diffuse components, but an IGH/c-MYC translocation signal in only the diffuse component. Accordingly, the atypical Burkitt lymphoma arose from the follicular lymphoma as a consequence of the additional IGH/c-MYC translocation, then involving the bone marrow.     

Aberrant expression of the dendritic cell marker TNFAIP2 by the malignant cells of Hodgkin lymphoma and primary mediastinal large B-cell lymphoma distinguishes these tumor types from morphologically and phenotypically similar lymphomas

Tumor necrosis factor-α-inducible protein-2 (TNFAIP2) is a protein upregulated in cultured cells treated with tumor necrosis factor α (TNF), but its expression in normal and neoplastic tissues remains largely unknown. Here, we use standard immunohistochemical techniques to demonstrate that TNFAIP2 is normally expressed by follicular dendritic cells, interdigitating dendritic cells, and macrophages but not by lymphoid cells in secondary lymphoid tissues. Consistent with this expression pattern, we found strong TNFAIP2 staining of tumor cells in 4 of 4 cases (100%) of follicular dendritic cell sarcoma and in 3 of 3 cases (100%) of histiocytic sarcoma. Although TNFAIP2 is not expressed by the small and intermediate-sized neoplastic B cells comprising follicular lymphoma, small lymphocytic lymphoma, mantle cell lymphoma, or marginal zone lymphoma, we observed strong TNFAIP2 staining of the large, neoplastic cells in 31 of 31 cases (100%) of classical Hodgkin lymphoma, in 12 of 12 cases (100%) of nodular lymphocyte-predominant Hodgkin lymphoma, and in 27 of 31 cases (87%) of primary mediastinal (thymic) large B-cell lymphoma. In contrast, TNFAIP2 was expressed by malignant cells in only 2 of 45 cases (4%) of diffuse large B-cell lymphoma, not otherwise specified, in 2 of 18 cases (11%) of Burkitt lymphoma, and in 1 of 19 cases (5%) of anaplastic large cell lymphoma. Further analysis indicates that TNFAIP2, as a single diagnostic marker, is more sensitive (sensitivity=87%) and specific (specificity=96%) than TRAF1, nuclear cRel, or CD23 for distinguishing the malignant B cells of primary mediastinal (thymic) large B-cell lymphoma from those of its morphologic and immunophenotypic mimic, diffuse large B-cell lymphoma, not otherwise specified. Thus, TNFAIP2 may serve as a useful new marker of dendritic and histiocytic sarcomas, the aberrant expression of which in the malignant cells of classical Hodgkin lymphoma and primary mediastinal (thymic) large B-cell lymphoma serves to distinguish these tumors from other large cell lymphomas in routine clinical practice.     

Ileocaecal intussusception in a 7-year-old patient due to Burkitt’s lymphoma

Intussusception is one of the most common paediatric emergencies, with a greater incidence during the first two years of life. Most cases are characterized as idiopathic. After this age, the incidence of intussusception steadily declines while, on the other hand, the possibility of an underlying causative disease, such as intestinal lymphoma, becomes increasingly stronger. We present the case of a 7-year-old Caucasian male that presented with non-reducible ileocolic intussusception due to a lead point, which proved to be a stage II Burkitt’s lymphoma. Resection, accompanied by chemotherapy, was successful, without complications, and with good response. Intestinal lymphomas may present with a varying clinical picture, from vague systematic symptoms to real surgical emergencies. General surgeons should be aware of that rare occasion where intussusception may be the result of an intestinal lymphoma, especially in children over the age of three, after which age an underlying lead point is more likely to exist.     

Burkitt lymphoma metastatic to penis

The first known case of metastatic Burkitt lymphoma to the penis is presented. The penis is a rare site of metastatic neoplasm in spite of its rich blood supply. Less than 200 cases have been reported in the last one hundred years. Local control was achieved with radiation therapy.     

Unusual presentation for primary appendiceal lymphoma: A case report

Primary appendiceal neoplasms are uncommon, being found in approximately 0.5%–1.0% of appendectomy specimens at pathologic evaluation. Primary appendiceal Burkitt’s lymphomas are rare occurring in 0.015% of all gastrointestinal lymphomas. Almost all reported cases of appendiceal lymphoma have proved to be non-Hodgkin lymphoma. The majority of appendiceal lymphomas are of B-cell. Patients were almost entirely males. Acute appendicitis is the most common clinical manifestation. This report describes a rare case of primary appendiceal lymphoma in a patient presented with hematuria and dull aching right lower abdominal and back pain.     

Burkitt's lymphoma of the caecum as a rare cause of acute abdomen: a case report

Burkitt's lymphoma is a highly aggressive B-lymphoproliferative disorder. Clinically it most often manifests with extranodal involvement, typically affecting the gastrointestinal tract as a large and rapidly proliferating tumor mass, which may present as acute abdomen. We present a case report of a young man admitted with signs of acute appendicitis. A tumor of the ileocaecal junction was discovered perioperatively and resolved by a right-sided hemicolectomy. Histological examination of the tumor confirmed Burkitt's lymphoma. On the ninth postoperative day, the patient developed signs of ileus and a follow-up CT scan showed tumor masses in the area of the ileotransversoanastomosis. He was transferred to undergo radical adjuvant chemotherapy. Intestinal passage was restored during the next two days. Our aim is to report on the limited role of surgical treatment in this type of malignancy, due to the extremely rapid proliferation of the tumor tissue, when the tumor can grow back to its original size over a period of a few hours or days.