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Renal synchronous carcinoma of clear cells with non-hodgkin lymphoma of phenotype b of type MALT
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actas urol esp. 2010;34(9):815–826
Renal synchronous carcinoma of clear cells with non-hodgkin lymphoma of phenotype b of type MALT Carcinoma renal de células claras sincrónico con linfoma no hodgkiniano de fenotipo B de tipo MALT
Dear Editor, We report the case of a 71-year-old male with a history of smoking, COPD, and diabetes mellitus on current treatment. The patient attended the emergency room of our center complaining of gross hematuria and was admitted for work-up. Supplemental tests performed included: iC omplete blood count and blood chemistry: no significant changes. i Abdominal ultrasound: a hyperechogenic solid mass, 3.6 x 4.2 cm in size, was found in the lower pole of left kidney. i CT scan of chest: no significant changes. i CT scan of abdomen: a mass with a soft tissue density, with lobulated and well defined margins, slightly heterogeneous, and approximately 4 cm in size, consistent with a neoplasm, was seen in the lower pole of left kidney. Laparoscopic left radical nephrectomy was performed on 16-02-2007. No complications occurred after surgery. In the pathological study, two nodular formations, one in each pole, were grossly seen. Microscopically, the nodular formation in the lower pole was characterized as a Fuhrman grade 2 clear cell carcinoma (fig. 1). The mass in the upper pole was characterized by an atypical dense lymphoid proliferation of small and medium-sized elements, consisting of cells with a centrocytic appearance and plasmacytoid and plasma cells, with occasional presence of lymphoepithelial lesions and reactive lymphoid follicles (fig. 2). Immunohistochemistry showed this lymphocyte population to be positive for CD20 and CD79a and negative for CD5, CD23, CD43, CD10, bcl-2, bcl-6, and cyclin D1. Pathological diagnosis was clear cell renal carcinoma in the lower pole synchronous with a low-grade B-cell nonHodgkin MALT lymphoma in the upper pole. Tumor stages were Ib and IE respectively. Primary renal lymphoma is a very uncommon (1%) neoplasm, and was first reported by Knoepp.1 B-cell MALT lymphoma of the kidney is extremely rare2 and was first reported by Pelstring et al.2 Little is known about the histogenesis of MALT lymphoma in the kidney but, as in other organs, it may be related to chronic or autoimmune inflammatory processes which would act as triggering factors. This is however controversial, and not all authors agree with such concept as regards the kidney.3 In the reported case, no chronic inflammatory processes were found, and there was no clinical or laboratory evidence of any immune disorder.
In addition, in order to diagnose primary renal lymphoma, lymphoproliferative conditions in other organs should be ruled out first,4 as was done in the extension study done to the patient. Diagnosis is based on pathological findings and is usually done after nephrectomy because lymphoma cannot be differentiated from other renal tumors based on either the clinical signs or imaging tests. Primary B-cell MALT lymphoma of the kidney generally has a favorable prognosis after surgery because it is usually diagnosed at an early stage (I-II). Stage is known to be the most significant predictor for disease-free and overall survival. Thus, in the Papaxoinis et al study,5 5-year disease-
Figure 1 – Proliferation of atypical epithelial cells of a tubular and cystic pattern with wide cells of clear cytoplasm and mid-sized nucleus with presence of nucleoli. Clear cell renal carcinoma (Fuhrman grade II).
actas urol esp. 2010;34(9):815–826
819
regimens were highly active in patients with relapsed MALT lymphoma. In the Wöhrer et al report,8 authors concluded that the combination of mitoxantrone, chlorambucil, and prednisone was effective and well tolerated in patients with stage III-IV MALT lymphoma (extragastric in 10) regardless of their location. In the reported patient, in whom both tumors were stage I, nephrectomy and regular follow-up were decided. No local or systemic adjuvant treatment was used.
R efere n ces
Figure 2 – Extensive and dense atypical lymphoid population with small and medium-sized elements alternating with frequent reactive lymphoid follicles and plasma cells. B-cell non-Hodgkin MALT lymphoma.
free survival rates were 67% in patients in stages I-II and 13% in those in stages III-IV. The corresponding 5-year overall survival rates were 91% and 51%. Our patient had stage IE disease. Therefore, prognosis will mainly depend on the coexisting clear cell epithelial tumor. As regards treatment of localized renal carcinoma and primary renal MALT lymphoma, consensus appears to exist in that nephrectomy is the procedure of choice. All five patients reported by Kato et al6 underwent nephrectomy, which was followed by complementary chemotherapy in only one patient. The most active chemotherapy regimen and the time of its administration are not defined. In the Raderer et al study7 reporting 26 patients with relapsed MALT lymphoma in various locations, 15 patients received rituximab plus cyclophosphamide, adriamycin, vincristine, and prednisone, and the other 11 patients were given the same regimen but replacing adriamycin by mitoxantrone because they were over 65 years of age or had cardiac comorbidities. Complete and partial responses were reported in 77% and 23% of patients respectively, and the authors concluded that both
1. Knoepp LF. Lymphosarcoma of the kidney. Surgery. 1956;39:510-4. 2. Pelsting RJ, Essell JH, Kurtin PJ, Cohen AR, Banks PM. Diversity of organ site involvement among malignant lymphoma of mucosa-associated lymphoid tissues. Am J Clin Pathol. 1991;96:738-45. 3. Qiu L, Unger PD. Low brade mucosa associated lymphoid tissue lymphoma involving the kidney. Report of 3 cases and review of the literature. Arch Pathol Lab Med. 2006;130:86-9. 4. Dobkin SF, Brem AS, Caldemone AA. Primary renal lymphoma. J Urol. 1991;146:1588-990. 5. Papaxoinis G, Fountzilas G, Rontogianni D, Dimopoulos MA, Pavlidis N, Tsatalas C, et al. Low-grade mucosa-associated lymphoid tissue lymphoma: A retrospective analysis of 97 patients by the Hellenic Cooperative Oncology Group (HeCOG). Ann Oncol. 2008;19:780-6. 6. Kato Y, Hasegawa M, Numasato S, Monma N, Fujioka T. Primary mucosa-associated lymphoid tissue-type lymphoma arising in the kidney. Int J Urol. 2008;15:90-2. 7. Raderer M, Wohrer S, Streubel B, Drach J, Jager U, Turetschek K, et al. Activity of rituximab plus cyclophosphamide, doxorubicin/mitoxantrone, vincristine and prednisone in patients with relapsed MALT lymphoma. Oncology. 2006;70:411-7. 8. Wöhrer S, Drach J, Hejna M, Schethauer W, Dirisamer A, Püspök A, et al. Treatment of extranodal marginal zona B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with mitoxantrone, chlorambucil and prednisone (MCP). Ann Oncol. 2003;14:1758-61.
J.A. Contreras-Ibáñeza,*, L. Díaz-Gómezb, and P. Muriel-Cuetoc aServicio
de Oncología Médica, Hospital Universitario Puerta del Mar, Cádiz, Spain bServicio de Oncología Radioterápica, Hospital Universitario Puerta del Mar, Cádiz, Spain cServicio de Anatomía Patológica, Hospital Universitario Puerta del Mar, Cádiz, Spain *Corresponding author. E-mail: [email protected] (J.A. Contreras-Ibáñez).
actas urol esp. 2010;34(9):815–826
Renal synchronous carcinoma of clear cells with non-hodgkin lymphoma of phenotype b of type MALT Carcinoma renal de células claras sincrónico con linfoma no hodgkiniano de fenotipo B de tipo MALT
Dear Editor, We report the case of a 71-year-old male with a history of smoking, COPD, and diabetes mellitus on current treatment. The patient attended the emergency room of our center complaining of gross hematuria and was admitted for work-up. Supplemental tests performed included: iC omplete blood count and blood chemistry: no significant changes. i Abdominal ultrasound: a hyperechogenic solid mass, 3.6 x 4.2 cm in size, was found in the lower pole of left kidney. i CT scan of chest: no significant changes. i CT scan of abdomen: a mass with a soft tissue density, with lobulated and well defined margins, slightly heterogeneous, and approximately 4 cm in size, consistent with a neoplasm, was seen in the lower pole of left kidney. Laparoscopic left radical nephrectomy was performed on 16-02-2007. No complications occurred after surgery. In the pathological study, two nodular formations, one in each pole, were grossly seen. Microscopically, the nodular formation in the lower pole was characterized as a Fuhrman grade 2 clear cell carcinoma (fig. 1). The mass in the upper pole was characterized by an atypical dense lymphoid proliferation of small and medium-sized elements, consisting of cells with a centrocytic appearance and plasmacytoid and plasma cells, with occasional presence of lymphoepithelial lesions and reactive lymphoid follicles (fig. 2). Immunohistochemistry showed this lymphocyte population to be positive for CD20 and CD79a and negative for CD5, CD23, CD43, CD10, bcl-2, bcl-6, and cyclin D1. Pathological diagnosis was clear cell renal carcinoma in the lower pole synchronous with a low-grade B-cell nonHodgkin MALT lymphoma in the upper pole. Tumor stages were Ib and IE respectively. Primary renal lymphoma is a very uncommon (1%) neoplasm, and was first reported by Knoepp.1 B-cell MALT lymphoma of the kidney is extremely rare2 and was first reported by Pelstring et al.2 Little is known about the histogenesis of MALT lymphoma in the kidney but, as in other organs, it may be related to chronic or autoimmune inflammatory processes which would act as triggering factors. This is however controversial, and not all authors agree with such concept as regards the kidney.3 In the reported case, no chronic inflammatory processes were found, and there was no clinical or laboratory evidence of any immune disorder.
In addition, in order to diagnose primary renal lymphoma, lymphoproliferative conditions in other organs should be ruled out first,4 as was done in the extension study done to the patient. Diagnosis is based on pathological findings and is usually done after nephrectomy because lymphoma cannot be differentiated from other renal tumors based on either the clinical signs or imaging tests. Primary B-cell MALT lymphoma of the kidney generally has a favorable prognosis after surgery because it is usually diagnosed at an early stage (I-II). Stage is known to be the most significant predictor for disease-free and overall survival. Thus, in the Papaxoinis et al study,5 5-year disease-
Figure 1 – Proliferation of atypical epithelial cells of a tubular and cystic pattern with wide cells of clear cytoplasm and mid-sized nucleus with presence of nucleoli. Clear cell renal carcinoma (Fuhrman grade II).
actas urol esp. 2010;34(9):815–826
819
regimens were highly active in patients with relapsed MALT lymphoma. In the Wöhrer et al report,8 authors concluded that the combination of mitoxantrone, chlorambucil, and prednisone was effective and well tolerated in patients with stage III-IV MALT lymphoma (extragastric in 10) regardless of their location. In the reported patient, in whom both tumors were stage I, nephrectomy and regular follow-up were decided. No local or systemic adjuvant treatment was used.
R efere n ces
Figure 2 – Extensive and dense atypical lymphoid population with small and medium-sized elements alternating with frequent reactive lymphoid follicles and plasma cells. B-cell non-Hodgkin MALT lymphoma.
free survival rates were 67% in patients in stages I-II and 13% in those in stages III-IV. The corresponding 5-year overall survival rates were 91% and 51%. Our patient had stage IE disease. Therefore, prognosis will mainly depend on the coexisting clear cell epithelial tumor. As regards treatment of localized renal carcinoma and primary renal MALT lymphoma, consensus appears to exist in that nephrectomy is the procedure of choice. All five patients reported by Kato et al6 underwent nephrectomy, which was followed by complementary chemotherapy in only one patient. The most active chemotherapy regimen and the time of its administration are not defined. In the Raderer et al study7 reporting 26 patients with relapsed MALT lymphoma in various locations, 15 patients received rituximab plus cyclophosphamide, adriamycin, vincristine, and prednisone, and the other 11 patients were given the same regimen but replacing adriamycin by mitoxantrone because they were over 65 years of age or had cardiac comorbidities. Complete and partial responses were reported in 77% and 23% of patients respectively, and the authors concluded that both
1. Knoepp LF. Lymphosarcoma of the kidney. Surgery. 1956;39:510-4. 2. Pelsting RJ, Essell JH, Kurtin PJ, Cohen AR, Banks PM. Diversity of organ site involvement among malignant lymphoma of mucosa-associated lymphoid tissues. Am J Clin Pathol. 1991;96:738-45. 3. Qiu L, Unger PD. Low brade mucosa associated lymphoid tissue lymphoma involving the kidney. Report of 3 cases and review of the literature. Arch Pathol Lab Med. 2006;130:86-9. 4. Dobkin SF, Brem AS, Caldemone AA. Primary renal lymphoma. J Urol. 1991;146:1588-990. 5. Papaxoinis G, Fountzilas G, Rontogianni D, Dimopoulos MA, Pavlidis N, Tsatalas C, et al. Low-grade mucosa-associated lymphoid tissue lymphoma: A retrospective analysis of 97 patients by the Hellenic Cooperative Oncology Group (HeCOG). Ann Oncol. 2008;19:780-6. 6. Kato Y, Hasegawa M, Numasato S, Monma N, Fujioka T. Primary mucosa-associated lymphoid tissue-type lymphoma arising in the kidney. Int J Urol. 2008;15:90-2. 7. Raderer M, Wohrer S, Streubel B, Drach J, Jager U, Turetschek K, et al. Activity of rituximab plus cyclophosphamide, doxorubicin/mitoxantrone, vincristine and prednisone in patients with relapsed MALT lymphoma. Oncology. 2006;70:411-7. 8. Wöhrer S, Drach J, Hejna M, Schethauer W, Dirisamer A, Püspök A, et al. Treatment of extranodal marginal zona B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with mitoxantrone, chlorambucil and prednisone (MCP). Ann Oncol. 2003;14:1758-61.
J.A. Contreras-Ibáñeza,*, L. Díaz-Gómezb, and P. Muriel-Cuetoc aServicio
de Oncología Médica, Hospital Universitario Puerta del Mar, Cádiz, Spain bServicio de Oncología Radioterápica, Hospital Universitario Puerta del Mar, Cádiz, Spain cServicio de Anatomía Patológica, Hospital Universitario Puerta del Mar, Cádiz, Spain *Corresponding author. E-mail: [email protected] (J.A. Contreras-Ibáñez).