Abstract:Renal involvement by Non-Hodgkin’s lymphoma (NHL) is very rare, and involvement of the kidney as the primary site of NHL (PRNHL) is much more uncommon. Gold standard for the diagnosis of PRNHL is histology and imaging modalities although helpful are not specific. Nephrectomy has been mostly recommended for low grade lymphomas, and for high grade PRNHLs, chemotherapy without nephrectomy has been recommended as the treatment of choice. This tumor is aggressive with poor prognosis. This poor prognosis is partly b… Show more
“…A systematic review of 187 primary adrenal lymphomas reported in the English literature till 2013 demonstrated that is a typically symptomatic and aggressive, high grade disease, which primarily affects elderly males and is bilateral in 70% of patients. 17 In the past two decades, a total of 83 patients of primary renal NHL lymphoma, including 17 patients with simultaneous involvement of other sites, were reported in the English literature, 18 the majority being DLBCL (n = 47), two of which were incidental findings. [19][20][21] A retrospective review of the cancer registry in Gundersen Health System from 1995 through 2014 yielded 36 patients with lymphoid neoplasms involving prostate, kidney, bladder, and penis.…”
Fibrin-associated large B-cell lymphoma (FA-LBCL) is a rare subtype of Epstein-Barr virus (EBV)-associated lymphoma, recognized as an independent entity per the 5th edition of the WHO classification of hematolymphoid neoplasms. It is usually associated with longstanding chronic inflammation and arises within fibrinous material in confined anatomic spaces. We report the clinicopathologic manifestations of two patients of FA-LBCL involving the adrenal gland and kidney. Both tumors were diagnosed after presenting as cystic masses on imaging studies. These lymphomas were non-invasive, with microscopic aggregates of large B-lymphoma cells along/within cystic wall and admixed with fibrinous material and without prominent inflammation. By immunohistochemistry and in-situ hybridization, lymphoma cells were positive for CD45, PAX5, CD79a, MUM1, BCL2, PD-L1, and EBV/EBER (Epstein-Barr virus encoded small RNA) with a high proliferation index. Both patients remain in remission after management with complete surgical resection and additional chemo-immunotherapy in one patient. Considering its rarity, scant tumor cells, and varied clinical presentations, FA-LBCL may pose diagnostic challenges, especially when presenting as extensively necrotic cystic lesions, needing multidisciplinary collaboration in formulating management.
“…A systematic review of 187 primary adrenal lymphomas reported in the English literature till 2013 demonstrated that is a typically symptomatic and aggressive, high grade disease, which primarily affects elderly males and is bilateral in 70% of patients. 17 In the past two decades, a total of 83 patients of primary renal NHL lymphoma, including 17 patients with simultaneous involvement of other sites, were reported in the English literature, 18 the majority being DLBCL (n = 47), two of which were incidental findings. [19][20][21] A retrospective review of the cancer registry in Gundersen Health System from 1995 through 2014 yielded 36 patients with lymphoid neoplasms involving prostate, kidney, bladder, and penis.…”
Fibrin-associated large B-cell lymphoma (FA-LBCL) is a rare subtype of Epstein-Barr virus (EBV)-associated lymphoma, recognized as an independent entity per the 5th edition of the WHO classification of hematolymphoid neoplasms. It is usually associated with longstanding chronic inflammation and arises within fibrinous material in confined anatomic spaces. We report the clinicopathologic manifestations of two patients of FA-LBCL involving the adrenal gland and kidney. Both tumors were diagnosed after presenting as cystic masses on imaging studies. These lymphomas were non-invasive, with microscopic aggregates of large B-lymphoma cells along/within cystic wall and admixed with fibrinous material and without prominent inflammation. By immunohistochemistry and in-situ hybridization, lymphoma cells were positive for CD45, PAX5, CD79a, MUM1, BCL2, PD-L1, and EBV/EBER (Epstein-Barr virus encoded small RNA) with a high proliferation index. Both patients remain in remission after management with complete surgical resection and additional chemo-immunotherapy in one patient. Considering its rarity, scant tumor cells, and varied clinical presentations, FA-LBCL may pose diagnostic challenges, especially when presenting as extensively necrotic cystic lesions, needing multidisciplinary collaboration in formulating management.
“…Some authors consider nephrectomy as part of the management plan in addition to chemotherapy in unilateral PRL. 1 …”
Section: Discussionmentioning
confidence: 99%
“…However, PRL is extremely rare and comprises less than 1% of all renal masses and less than 1% of extranodal lymphomas. [1][2][3][4][5][6] Primary renal lymphoma is a distinct clinicopathological entity with highly aggressive behavior, and 50% of the cases also exhibit extrarenal involvement. 6,7 Utilizing the Surveillance, Epidemiology, and End Result )SEER( database, Taneja et al 2 and Chen et al 4 showed that diffuse large B-cell lymphoma )DLBCL( is the most common histopathological subtype.…”
mentioning
confidence: 99%
“…Some authors consider nephrectomy as part of the management plan in addition to chemotherapy in unilateral PRL. 1 Primary renal lymphoma must be differentiated from renal cell carcinoma. Standard treatment of renal masses is nephrectomy, but PRL differs in that it is usually managed with chemotherapy followed by nephrectomy.…”
Histopathological and immunohistochemical slides were reviewed, and additional immunohistochemistry stains were carried out in selected cases. Follow-up data were also collected.
Original ArticleResults: There were 17 identified cases of PRL. The age of the patients ranged from 4-76 years )median: 50.5 years and mean: 46.8 years(, 11 )64.7%( were males, and 6 )35.3%( were females. There were 12 cases of diffuse large B-cell lymphoma, 3 cases of Burkitt's lymphoma, and 2 cases of post-transplant lymphoproliferative disorder. The median follow-up duration was 22 months. The one-year overall survival rate was 71% and the 2-year overall survival rate was 43% during follow-up.
Conclusion:Primary renal lymphomas in Saudi patients are more common in males and seen in a relatively younger age group compared to the available worldwide data. The diagnosis of PRL is important to avoid tumor dissemination and unnecessary nephrectomy. Diffuse large B-cell lymphoma is the most common pathological type and non-germinal center B-cell is the most common subtype.
“…Primary renal lymphoma (PRL), one type of NHL, restricting to kidney without other organ infiltration, is extremely rare in children. [ 2 ] Given the rarity and greatly overlapped presentations with other malignant renal lesions, pediatric PRL may be easily misdiagnosed. [ 3 ] As per literature review, most of the time they were diagnosed post-surgical resection for other clinically diagnosed malignancies.…”
Rationale: Only 20 cases of pediatric primary renal non-Hodgkin's lymphoma have been reported since 1995, rare cases and a variety of imaging manifestations have led to difficulties in its diagnosis and treatment.Patient concerns: Herein, we share in detail a case of primary renal lymphoma (PRL) in a child and summarize the common clinical manifestations, imaging features, and prognostic factors of pediatric PRL by retrospectively analyzing cases reported in the literature. A 2-year-old boy presented to the clinic with a large mass on the right side of his abdomen along with loss of appetite.Diagnoses: Imaging revealed a large right renal mass, nearly replacing the entire renal tissue, along with numerous small nodules in the left kidney. Given no local adenopathy and metastases, the diagnosis was unclear. A percutaneous renal puncture was performed, which proved the diagnosis of Burkitt's lymphoma. Since no bone marrow involvement, this child was diagnosed with pediatric PRL.Interventions: This PRL boy was treated with the NHL-BFM95 protocol and supportive care.Outcomes: Unfortunately, this boy died of multiple organ failure in the fifth month of treatment.Lessons: As per literature review, the presentation of pediatric PRL is fatigue, loss of appetite, weight loss, abdominal swelling, or other nonspecific symptoms. Although in 81% of cases it often infiltrates the bilateral kidneys, urine abnormalities caused by pediatric PRL are uncommon. 76.2% of pediatric PRL were boys and 2/3 of all cases presented as diffuse renal enlargement. Those PRL presented as masses could easily be misdiagnosed as WT or other malignancies. Absent of local enlarged lymph node, no necrosis or calcification suggest atypical presentation of renal masses and a percutaneous biopsy is needed in timely establishing the accurate diagnosis for appropriate treatment. Based on our experience, percutaneous renal puncture core biopsy is a safe procedure.
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