Diffuse large B-cell lymphoma in a renal allograft associated with Epstein-Barr virus in the recipient: a case report and a review of lymphomas presenting in a transplanted kidney

Abstract:  The purpose of this review was to evaluate the case reports with lymphoproliferative disorders exclusively localized in the allograft transplanted kidney. We also report a rare case of diffuse large B-cell lymphoma originated from the lymphocytes of the recipient with exclusive localization in the kidney allograft. We searched the medical literature for case reports of this condition. We found only 16 cases of lymphoma presenting in the kidney allograft without systemic affectation. The most frequent clinical manifestations were graft dysfunction and fever. In the majority of patients included in this review, the diagnosis was established from the tissue of explanted allograft. Seven patients were diagnosed with B-cell lymphoma, and the relationship with Epstein-Barr virus could only be demonstrated in four patients. However, the outcome was satisfactory in all cases, except in one case in which death was not related with the lymphoma etiology. Health care providers should be aware of this clinical entity and heightened index of suspicion should be used so as not to delay diagnosis and not to lose the allograft.     

Primary hepatic lymphoma of MALT-type: a tumor that can simulate a liver metastasis

Primary hepatic lymphomas are rare tumors. We report a case of a 72 year-old woman with a past history of colonic adenocarcinoma who presented primary hepatic lymphoma of MALT-type. The patient had been operated on 3 years before for colonic adenocarcinoma, pT3N0, revealed by a bowel obstructive syndrome. She had been treated by chemotherapy for 6 months. During the follow-up, the computed tomography-scan (CT-scan) revealed the presence of a not well-demarcated mass in segment III of the liver, measuring 4 cm in diameter. The tumor was hypodense and was not enhanced on dynamic study. The mass was already present on the initial CT-scan. Left lobectomy was performed with the diagnosis of liver metastasis of the colonic adenocarcinoma. Surgical specimen showed a tumor composed of a dense infiltrate of small lymphocytes positive for B-cell markers on immunohistochemistry. The tumor contained reactive lymphoid follicles and there were numerous lympho-epithelial biliary lesions. The patient is alive and free of disease 2 years after the diagnosis. Primary hepatic lymphoma of MALT-type is a low-grade B cell lymphoma. Twenty-five cases had been reported in the literature so far. The patients were 16 females and 9 males, mean age 63.5 years. The pathogenesis is still unclear but half of the patients had a past history of chronic inflammatory liver disease (hepatitis B or C virus infection, ascaris infection, primary biliary cirrhosis) or malignant neoplasm. This tumor has a good prognosis; it is usually limited to the liver and surgical resection cures the patient in most cases.     

原发性肾脏恶性淋巴瘤（附3例报告）

目的 探讨肾脏原发性恶性淋巴瘤的临床特点。方法 总结3例肾脏原发性恶性淋巴瘤患者临床资料，结合文献复习讨论其发病特点、影像学特征、治疗及预后。结果 3例患者中手术活检加化疗1例，手术切除加化疗1例，术前化疗加手术切除加术后化疗1例。3例均诊断为非何杰金淋巴瘤，1例死于肾衰，另2例分别存活38个月和8个月，仍在随访中。结论 肾脏原发性恶性淋巴瘤影像学征象与肾细胞癌相似，肾脏包膜或包膜下弥漫浸润被认为是原发性肾恶性淋巴瘤的特征性表现，通过经皮穿刺活检可明确诊断，治疗应根据组织学分型、分期及肿瘤大小，采取手术联合化疗及放疗。     

MALT-type primary lymphoma of the urinary bladder: clinicopathological study of 2 cases and review of the literature

Primary lymphoma of the bladder is a rare non-epithelial bladder tumour. It is usually non-Hodgkin's lymphoma with mucosa-associated lymphoid tissue (MALT) lymphoma being its predominant subtype. Most of the literature has been limited to individual sporadic case reports. We present 2 cases of the MALT-type primary lymphoma of the urinary bladder which were treated in our unit. Both patients were females with a history of recurrent urinary tract infections. They were treated successfully with chemotherapy alone. We also present a review of the literature emphasising the clinical presentation, the morphological and immunophenotypic features and the various treatment modalities of this rarely seen bladder pathology.     

肾脏原发性淋巴瘤一例报告并文献复习

目的 提高肾脏原发性淋巴瘤的诊治水平.方法 回顾性分析1例肾脏原发性淋巴瘤患者的临床病理资料,结合文献复习讨论.患者,女,61岁.因左侧腰痛2周入院.查体:左上腹压痛,左肾区叩击痛.B超检查见左肾正常结构未显示,集合系统分离,最宽处约1.0 cm,左肾区可探及一巨大低回声肿物,大小9.7cm×5.3cm,边界模糊.CT检查见左肾中下极肿物,大小9.8 cm×8.9 cm ×8.8 cm,边界不清,平扫CT值为39 HU,增强后为61 HU,强化不明显.术前诊断:左肾恶性肿瘤.结果 患者行根治性左肾切除术.病理报告:左肾大部分被肿瘤组织占据,中下极见10.0cm×9.5 cm ×8.5 cm类圆形肿物,质硬,肿物切面呈灰白色,质地细腻.镜下瘤细胞呈弥漫性浸润,卵圆形或多边形,略大于正常淋巴细胞,核大深染呈不规则形.病理诊断:肾弥漫性大B细胞性淋巴瘤.骨髓穿刺检查未见异常.行环磷酰胺+吡喃阿霉素+长春新碱+泼尼松+利妥昔单抗方案化疗6周期.术后每3个月复查胸部X线片、腹部B超及CT等.随访20个月,未见肿瘤复发.结论 肾脏原发性淋巴瘤临床罕见,症状与肾癌相似,影像学检查无特征性改变,确诊需依靠病理学检查.本病预后不良,手术切除患肾联合规范的全身化疗,可延长患者的生存期.     

Marginal zone lymphoma of both spleen and kidney displaying transformation into large B-cell lymphoma

We report a case of simultaneous involvement of the spleen and the left kidney in a marginal zone lymphoma with a monotypic lymphoplasmacytic cell component, which transformed into a diffuse large B-cell lymphoma of the immunoblastic type. PCR showed that the small and large B-cell populations carried the same type of immunoglobulin heavy chain gene rearrangement. This type of rearrangement was detected in the spleen, the latero-aortic lymphadenopathy and the kidney demonstrating that it is the same lymphoma that affected both organs and the lymph nodes. Primary renal lymphoma is very rare and only a few cases of renal marginal zone lymphoma, MALT type, have been reported. Involvement of simultaneous multiple sites has been described in MALT type lymphoma, but splenic involvement secondary to renal MALT lymphoma seems to have never been observed. Nevertheless, in our case the huge size of the spleen associated with splenic hilar node involvement is consistent with primary splenic marginal zone lymphoma. The extension into latero-aortic lymph nodes of this lymphoma can explain secondary kidney involvement. The nodal Kaposi’s sarcoma observed in this patient of Mediterranean origin was probably coincidental.     

Primary renal lymphoma of mucosa-associated lymphoid tissue

Mucosa-associated lymphoid tissue-type lymphomas have recently been recognized as a distinctive form of B-cell malignant lymphoma. In contrast to other types of low-grade lymphomas, these tumors have a tendency to be localized at diagnosis and to be curable with local therapy. We report an unusual case of primary localized low-grade lymphoma of mucosa-associated lymphoid tissue arising in the kidney. The patient underwent radical nephrectomy and was free of disease at 28 months of follow-up without additional treatment. Once properly staged and classified, lymphoma of mucosa-associated lymphoid tissue involving the kidney can be managed by radical nephrectomy and follow-up.     

Renal cell carcinomas arising from the allograft and bilateral native kidneys

Abstract:   We present the case of a young lady who developed renal cell carcinomas (RCC) in the allograft and bilateral native kidneys metachronously within one year. She received a living donor kidney transplantation from her father. A solid tumor of 4 cm in diameter was uncovered first in the allograft kidney 103 months after transplantation, and was treated with graftectomy. Six months after graftectomy, a right renal tumor measuring 3.5 cm and left renal tumors emerged in the native kidneys. She underwent laparoscopic right and left radical nephrectomy in separate sessions. The pathological diagnosis in the allograft and right renal tumors was clear cell RCC with eosinophilic cytoplasm and that in the left kidney was clear cell carcinoma. Fluorescence in situ hybridization and human leukocyte antigen typing showed that each tumor was most probably primary disease. She was free of disease 18 months postoperatively. This is the first report on RCC arising both in the allograft and bilateral native kidneys.     

Secondary prostatic lymphoma in a kidney transplant patient

Lymphoma involving the prostate is rare, both as a primary and as a secondary presenting. Usually the prognosis remains poor. The clinical presentation is similar to that of other lower urinary tract obstructions, in fact prostatic lymphoma must be considered in patients with these symptoms, particularly in patients with prior history of systemic lymphoma. We report a case of a kidney transplantation in a male patient, diagnosis of lymphoma non Hodgkin, with later recurrence in prostate.     

Right hepatic lobectomy for primary lymphoma: A case report and literature review

Primary lymphoma of the liver is an extremely rare entity; only eight cases have been reported in the literature. We treated a 34-year-old man with primary hepatic lymphoma by right hepatic lobectomy, including right hemicolectomy and nephrectomy. The histological diagnosis was a small cell diffuse histiocytic lymphoma. Details of this case plus that of others in the literature are described in this report.     

Right hepatic lobectomy for primary lymphoma: a case report and literature review

Primary lymphoma of the liver is an extremely rare entity; only eight cases have been reported in the literature. We treated a 34-year-old man with primary hepatic lymphoma by right hepatic lobectomy, including right hemicolectomy the nephrectomy. The histological diagnosis was a small cell diffuse histiocytic lymphoma. Details of this case plus that of others in the literature are described in this report.     

A case of renal malignant lymphoma

Malignant lymphoma affects any organ of the body, but is rarely found in a urological organ. We found a case of renal malignant lymphoma. A 45-year-old male, who had been operated on for primary hepatic malignant lymphoma 9 months previously, was admitted to our clinic, complaining of high fever. X-ray and ultrasonographic examinations suggested a metastatic tumor in the right kidney. Right nephrectomy was performed, and pathohistological examination revealed primary hepatic malignant lymphoma in the right kidney.     

Insuffisance rénale aiguë révélatrice d’un lymphome rénal. À propos d’un cas

Acute renal failure, as the initial manifestation of lymphoma, has been reported only in a few cases. In this work, we report the case of a 28-year-old women admitted for acute renal failure. Her physical examination detected bilateral kidney enlargement. Laboratory evaluation revealed a serum creatinine value 218 μmol/l. A 24-hour urine collection analysis allowed the detection of 1 g of protein. No red cells were found after urinanalysis. Renal ultrasound showed massively enlarged kidneys. Renal biopsy of the kidney and pathologic examination showed diffuse infiltration of the interstitium with lymphocytes and atypical cells positive for CD20 markers. A diagnosis of diffuse large B-cell type non-Hodgkin lymphoma was made. However, investigations revealed the presence of two others sites of lymphoma: gastric and ophthalmic. The patient's renal function and kidney size as well as the other lymphoma locations were normalized after the initiation of chemotherapy.     

Early renal involvement in acute lymphoblastic leukemia and nonHodgkin's lymphoma in children

Clinical manifestations of kidney disease, particularly renal failure, caused by malignant infiltration in patients with acute lymphoblastic leukemia or nonHodgkin's lymphoma have been described rarely. We report 1 case of acute lymphoblastic leukemia and 3 cases of nonHodgkin's lymphoma in which renal disease was the only or one of the presenting manifestations of malignancy. Of these patients 2 had rapidly progressive renal failure with nephromegaly, 1 presented with bilateral abdominal masses caused by severe nephromegaly and with microscopic hematuria, and 1 had microscopic hematuria without nephromegaly. In all 4 patients kidney biopsy revealed malignant infiltration. In the 2 patients who presented with renal failure kidney function promptly returned to normal after chemotherapy and irradiation of the kidneys. Prompt and correct diagnosis of nephropathy, when it is the only or one of the presenting signs of acute lymphoblastic leukemia or nonHodgkin's lymphoma, is necessary to expedite initiation of specific antitumor therapy.     

Primary central nervous system lymphoma presenting as an acute massive intracerebral hemorrhage: case report with immunohistochemical study

BACKGROUND: Hemorrhage at presentation in primary CNS lymphoma is extremely rare and only 2 studies have reported it. We experienced a case of hemorrhage in primary CNS lymphoma, but the feature was different from those in the 2 studies mentioned. We report a case of primary CNS lymphoma presenting as an acute massive ICH causing catastrophic mental deterioration, along with immunohistochemical profiles. CASE DESCRIPTION: A 49-year-old woman was admitted with sudden deterioration of mental status. Immediate CT scan showed a massive ICH in the left frontal lobe with perilesional edema and midline shifting. Emergency operation was performed and the hematoma was removed gross totally, including the abnormal lesions distinguished from adjacent normal brain. The histopathologic diagnosis was non-Hodgkin lymphoma, diffuse, large, B cell type with acute hemorrhage, and immunohistochemistry results showed high VEGF immunoreactivity and intermediate CD34 immunoreactivity. CONCLUSION: Primary CNS lymphoma can present as an acute massive ICH, and immunohistochemisty in the present study strengthened the suggestion that VEGF activity is related to the hemorrhage in primary CNS lymphoma.     

Spontaneous rupture of the ureter as the primary symptom of malignant lymphoma

We report a rare case in which upper ureteral rupture was the primary symptom of malignant lymphoma. A 74-year-old female visited our hospital with left flank pain. Computed tomography showed urinoma around the left kidney and retrograde pyelography showed a diffuse filling defect in the left ureter and a rupture of the upper portion of that ureter. A urine cytology specimen from the left ureter was class V, suggesting undifferentiated carcinoma or malignant lymphoma. An open laparotomy revealed a nodule on the omentum and diffuse fibrosis around both ureters, and the histopathological diagnosis was diffuse large B-cell lymphoma. The patient' s ureteral stenosis disappeared after she received six cycles of R-CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone and rituximab) chemotherapy. We should be aware that malignant lymphoma can be the cause of a spontaneous ureteral rupture.     

Mucosa-associated lymphoid tissue-type lymphomas occurring in post-transplantation patients

Post-transplantation lymphoproliferative disorders (PTLDs) are usually Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorders that vary in their morphologic spectrum. Extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue-type (MALT-type) have not been considered to be part of this spectrum. The authors encountered five such cases recently. The clinical, histopathologic, and immunophenotypic features are reported. There were three men and two women with a mean age of 51.2 years (range, 48-63 years). Two patients were cardiac transplant recipients, two patients were liver transplant recipients, and the remaining patient was a renal transplant patient. Sites of lymphoma were the stomach in three patients and the parotid gland in two patients. Mean time to the lymphoma was 84 months after transplantation. All patients had morphologic features of low-grade extranodal marginal zone lymphomas of the MALT-type, and Helicobacter pylori was present in all three gastric cases. All patients exhibited the B-cell immunophenotype and were negative for EBV by in situ hybridization. These lymphomas were treated with a variety of modalities, including reduction of immunosuppression, antibiotics, surgical resection, radiation therapy, and chemotherapy. At last follow-up, one patient had developed signet ring adenocarcinoma at 27 months but had no evidence of PTLD, one patient relapsed at 17 months but is alive with stable disease at 24 months, and the remaining patients were alive without disease at 11, 12, and 14 months. Extranodal low-grade MALT-type lymphomas can occur in the post-transplantation setting and generally develop years after transplant. As seen in immunocompetent patients, EBV appears to play no role in the pathogenesis of these lymphomas. These lymphomas appear to have more in common with MALT-type lymphomas in nonimmunocompromised patients than conventional PTLDs, although they occur in "at-risk" patients due to their immunosuppressive therapy. These lymphomas do not appear to be clinically aggressive. Recognition of MALT-type lymphomas in the post-transplantation setting as an indolent disease avoids unnecessary treatment.     

Perirenal Pseudolymphoma: A Case Report

A right nephrectomy was performed on a 51 year-old female with a tumor surrounding the right kidney since the possibility of malignant lymphoma could not be excluded based on radiography or a needle biopsy. An immunohistological examination of the surgically-resected specimen confirmed a diagnosis of pseudolymphoma. A pseudolymphoma involving the urinary tract is rare, and this is only the second reported case of pseudolymphoma in the perirenal space.     

Primary intestinal posttransplant T-cell lymphoma

There have been only five reported cases of primary posttransplant T-cell lymphoma. We report the first case associated with the use of sirolimus (Rapamycin, Wyeth-Ayerst, Philadelphia, PA). The patient, receiving prednisone, cyclosporine, and sirolimus treatment, developed ascites, diarrhea, and weight loss 7 months after his second renal transplant. Tissue obtained at laparotomy established the diagnosis of primary T-cell lymphoma. Latent membrane protein-1 for Epstein-Barr virus was negative, but in-site hybridization test for Epstein-Barr-encoded RNA was positive. Despite aggressive chemotherapy, the patient died 8 months posttransplant. This is the sixth reported case of primary intestinal posttransplant T-cell lymphoma, but it is the first case associated with the use of sirolimus. The incidence of posttransplant lymphoproliferative disease in patients receiving sirolimus should be studied.     

A case of primary mucosa-associated lymphoid tissue lymphoma of the bladder regressed after rituximab in combination with CHOP chemotherapy

Primary mucosa-associated lymphoid tissue (MALT) lymphoma of the bladder is a rare disease, and the most effective therapeutic procedure remains unknown. We report a case of primary MALT lymphoma of the bladder regressed after rituximab in combination with CHOP chemotherapy (R-CHOP). The patient, a 84-year-old woman, presented with general fatigue and loss of weight. Computed tomography (CT) showed a solitary mass at the bladder. Histological diagnosis of the tissue obtained by transurethral biopsy was extranodal marginal zone B cell lymphoma of MALT. The patient was in complete remission after only one cycle of R-CHOP. This is the fourteenth case of MALT lymphoma of the bladder in Japan.