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Ureteral obstruction in a patient with Burkitt's lymphoma and aids
URETERAL OBSTRUCTION IN A PATIENT WITH BURKITT’S LYMPHOMA AND AIDS SCOTT COMITER, M.D. JEFFREY GLASSER, M.D. SALAH AL-ASKARI, M.D.
From the Department of Urology, New York Uni\.ersit> School of Medicine, New York, New York ABSTRACT-Burkitt’s lymphoma presenting as an intrinsic ureteral mass is rare. We report on an immunocompromised patient with hydronephrosis .swondary to direct involvement of the ureter by this non-Hodgkin:s lymphonla. of AZDS and the treatment of this This case illustrates one of the manifestations manifestation. The relationship of AIDS and its seyucla to the genitolcr&ar!y system ir discussed. -._ The patient is a forty-two-year-old homosexual white man with documented human immunodeficiency virus (HIV) infection, who presented to Bellevue Hospital with a six-month history of dull, inlyermittent pain on the left side of his abdomen and left flank discomfort. He also complained of intermittent low-grade fevers at home over the past few months as well as a generalizedl feeling of weakness. The patient had no complaints of lower urinary tract voiding symptoms and denied presence of hematuria. Physical examination revealed an indurated, nontender mass involving the upper thigh on the left side. There was a 3 x 6-inch firm, nontender mass involving the medial aspect of the right thigh, just above the knee. Several palpable, nontender, rubbery lymph nodes were evident in the left inguinal region. There was no abdominal mass or tenderness. The prostate was 10 g in size, smooth, and nontender. The patient was afebrile on admission; urinalysis was normal, and serum chemistries and hematologic values were within normal limits. An excretory urogram performed on admission revealed a normal right kidney. The left kidney, however, showed delayed visualization with moderate hydroureteronephrosis (Fig. 1). A computerized tomography (CT) scan of the abdomen confirmed the hydronephrosis of the left kidney with nodular small masses in the renal pelvic wall (Fig. 2). There was thickening
FIGURE 2. CT scan of abdomen demonstrating hydronephrosis of left kidney with intrinsic proximal ureteral lesion and tumor mass in wall of left renal pelvis.
FICUHE 4. Left retrograde urogram showing hydronephrosis and involvement of the upper ureter by a mass lesion.
FIGURE 3. (A) CT scan of abdomen showing mass lesion involving left upper ureter; (B) after contrast injection there is increased attenuation of the mass.
FIGURE 5. Histopathology of thigh mass, demonstrating Burkitt? lymphoma, distinguished by the many large macrophages interspersed among tumor cel1.s creating “starry sky” histologic pattern.
and obstruction of the left ureter by what appeared to be a tumor near the ureteropelvic junction (Fig. 3A and B). A left retrograde pyelogram demonstrated a narrowed proximal left ureter involved by an extrinsic mass and a hydronephrotic collecting system (Fig. 4). A CT scan of the left thigh mass showed a solid infiltrating process involving the muscles of the medial portion of the left thigh. It was
homogeneous in appearance and displayed no areas of necrosis or hemorrhage. Open biopsy specimens of the right thigh mass and a left inguinal lymph node revealed closely packed, basophilic cells, uniform in size, with round-tonucleoli. oval nuclei and prominent Interspersed among the tumor cells were many large macrophages, creating a “starry sky” pattern, typical of Burkitt’s lymphoma (Fig. 5).
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UROLOGY
/ MARCH 1992 / VOLLJ.MEXXXIX,NUMBER 3
FI(:URE 6. CT Scan of abdomen demonstrating normal-appearing kidneys and ureters without hydronephrosis or ureteral lesions following completion o_fcoww of chemotherapy. The diagnosis of Burkitt’s lymphoma with hydronephrosis secondary to ureteropelvic involvement was made. The patient was entered into a phase I trial of methotrexate, bleomycin, Adriamycin, cyclophosphamide, vincristine, dexamethasone, and granulocyte-macrophage colony-stimulating factor. This chemotherapy regimen was given in three-week cycles over a four-month period. A CT scan of the abdomen performed six days after the completion of chemotherapy revealed normal-appearing kidneys and ureters bilaterallly without evidence of hydronephrosis or ureteral lesions (Fig. 6). The right thigh mass was no longer present on CT scan or physical examination. Likewise, the abdominal pain and the flank pain had resolved. The patient had been in complete remission from his Burkitt’s lymphoma six months after chemotherapy was begun. However, the patient was found to have lymphoma of the central nervous system, and succumbed to his disease five month5 later. Comment Lymphomas are characterized by the neoplastic proliferation of cells within the elements of the reticuloendothelial system-lymph nodes, bone marrow, spleen, and liver. The term malignant lymphoma emphasizes the predictable patterns of tumor involvement and spread within the lymphoreticular tissues. Malignant lymphomas are usually divided into two groups, Hodgkin’s disease and non-Hodgkin’s lymphomas. The distinguishing histologic feature of Hodgkin’s disease is the presence of giant
multinucleated cells, called Sternberg-Reed cells, in affected lymph nodes, The nonHodgkin’s lymphomas are a more heterogeneous group, composed of relatively indolent disorders such as the nodular lyrnphocytic lymphomas as well as more aggressive neoplasms such as diffuse histiocystic ly,mphomas and Burkitt’s lymphoma. Burkitt’s lymphoma is composed of neoplastic cells derived from B-cell lymphoid precursors. The Epstein-Barr virus has been identified as the possible cause of this lymphoma. 1.2 Several large series, consisting of individuals with malignant lymphomas, demonstrate involvement of the genitourinary system in 4 to 7 percent of patients. 3m5Symptoms are present in only 29 to 49 percent of cases, with hematuria most commonly seen. 3.s The majority Iof cases of lymphomatous involvement of the genitourinary tract are found on postmortem examinations. 3 Hydronephrosis secondary to direct involvement of the ureter by malignant lvmphoma is an unusual finding, being present in about 0.5 percent to 7 percent of all cases of lymphoma. The patient in this report was found to have a proximal ureteral mass as the cause of the hvdronephrosis. Hydronephrosis, however, -is most commonly secondary to ureteral obstruction by retroperitoneal lymph nodes, and can be seen as compression mainly along the distal ureter with the intravenous pyelogram demonstrating lateral displacement of the tlreter by enlarged lymph nodes.3 r, Neoplasms are seen with increased frequency in patients with immune deficiency, being ten thousand times more common than in the general population.“i Immune deficiency may be congenital, iatrogenic, or acquired, as in human immunodeficiency virus infection. Lymphoproliferative disorders are the most common neoplasms found in immunocompromised populations. B An associatio-n between lymphoproliferative tumors and AIDS patients is apparent. There is a nearly two-hundred-fold increase in malignant lymphoma among AIDS patients as compared with a simil,ar control population. Another feature of non-,Hodgkin’s lymphoma among AIDS patients is the extranoda1 involvement commonly found .’ Patients with AIDS-related lymplhomas are treated with standard chemotherapy regimens, the most recent regimen consisting of methobleomycin, Adriamycin, ,cyclophostrexate, phamide. vincristine, and dexanlethasone.h The
overall response rate of standard chemotherapy protocols is about 65 to 70 percent.7 Complete responders have been found in about 50 percent of patients treated.R.g Granulocyte-macrophage colony-stimulating factors (GMCSF), a hematopoietic growth factor, has been added to chemotherapy regimens in an attempt to reduce the duration of neutropenia. lo In this case, Burkitt’s lymphoma arising in an immunocompromised individual presented as hydronephrosis secondary to direct involvement of the ureter and lower extremity masses. After a course of chemotherapy the hydronephrosis resolved, and the lower extremity masses disappeared. The resolution of the ureteral mass and hydronephrosis after a course of chemotherapy for non-Hodgkin’s lymphoma confirmed the diagnosis of a lymphomatous involvement of the ureter causing obstruction. Cases of AIDS and manifestations of AIDS are frequently seen by the urologist. When presented with tumors involving the genitourinary tract in an immunocompromised individual, rare types of tumors, such as lymphoma, should be considered.
280
New York, New York 10016 (DR. AL-ASKARI) References 1. Fishman MC, ct al: Leukemia and Lymphoma, in Medicine, Philadelphia, J.B. Lippincott Co.. 1981, pp 360-369. 2. Owens AH, and Sensenbrenner LL: The Iymphorcticular proliferative disorders, in IIarvey AM, cf (I/. (Eds): Principles and Practice of Medicine, Connecticut, ApI’leton-Cerltrlr~-Crofts, 1984, pp 565-581. 3. Watson EM, Sauer HH, and Sadugor MC: Manifestations of the lymphoblastomas in the genitourinary tract, J Ural 61: 626 (1949). 4. Rosenberg SA, Diamond IID, ‘Ihslouitz B, and Craver LF: Lymphosarcoma: a review of 1,269 cases, Medicine 40: 31 (1961). 5. Abeloff MD, and Lenhard RE: Clinical management of ureteral obstruction secondary to malignant lymphonra, Johns Hopkins Med J 134: 34 (1974). 6. Ioachim HI,, Cooper MC, and IIeIhnan CC: I,ymphomas in men at high risk for acquired immune deficiency syndrornc (AIDS), Cancer 56: 2831 (1985). 7. Beckhardt RN, ef al: Increased incidence of malignant lynphoma in AIDS: a comparison of risk groups and possible ctiologic factors, Mt Sinai J Med 55: 383 (1988). 8. Gill PS, ct al: AIDS-related malignant lynrphoma: results of prospective treatment trials, J Clinic Oncol 5: 1322 (1987). 9. Ziegler IL. ct al: Non-Hodgkin’s Ivmnhoma in 90 homosexrral men: relation to generalized lymphadenopathy and the acquired immunodcficiency syndrome, N Engl J Mcd 311: 56.5 (1984). 10. Gill PS, and Levine AM: IIIV-related malignant Iynphoma: clinical aspects, treatment and pathogenesis, Cancer Invest 6: 413 (1988). j-l
I/
UROLOGY
/
MAHCII
1992
i
VOI,UME
L
XXXIS,
NUMBKR
3
From the Department of Urology, New York Uni\.ersit> School of Medicine, New York, New York ABSTRACT-Burkitt’s lymphoma presenting as an intrinsic ureteral mass is rare. We report on an immunocompromised patient with hydronephrosis .swondary to direct involvement of the ureter by this non-Hodgkin:s lymphonla. of AZDS and the treatment of this This case illustrates one of the manifestations manifestation. The relationship of AIDS and its seyucla to the genitolcr&ar!y system ir discussed. -._ The patient is a forty-two-year-old homosexual white man with documented human immunodeficiency virus (HIV) infection, who presented to Bellevue Hospital with a six-month history of dull, inlyermittent pain on the left side of his abdomen and left flank discomfort. He also complained of intermittent low-grade fevers at home over the past few months as well as a generalizedl feeling of weakness. The patient had no complaints of lower urinary tract voiding symptoms and denied presence of hematuria. Physical examination revealed an indurated, nontender mass involving the upper thigh on the left side. There was a 3 x 6-inch firm, nontender mass involving the medial aspect of the right thigh, just above the knee. Several palpable, nontender, rubbery lymph nodes were evident in the left inguinal region. There was no abdominal mass or tenderness. The prostate was 10 g in size, smooth, and nontender. The patient was afebrile on admission; urinalysis was normal, and serum chemistries and hematologic values were within normal limits. An excretory urogram performed on admission revealed a normal right kidney. The left kidney, however, showed delayed visualization with moderate hydroureteronephrosis (Fig. 1). A computerized tomography (CT) scan of the abdomen confirmed the hydronephrosis of the left kidney with nodular small masses in the renal pelvic wall (Fig. 2). There was thickening
FIGURE 2. CT scan of abdomen demonstrating hydronephrosis of left kidney with intrinsic proximal ureteral lesion and tumor mass in wall of left renal pelvis.
FICUHE 4. Left retrograde urogram showing hydronephrosis and involvement of the upper ureter by a mass lesion.
FIGURE 3. (A) CT scan of abdomen showing mass lesion involving left upper ureter; (B) after contrast injection there is increased attenuation of the mass.
FIGURE 5. Histopathology of thigh mass, demonstrating Burkitt? lymphoma, distinguished by the many large macrophages interspersed among tumor cel1.s creating “starry sky” histologic pattern.
and obstruction of the left ureter by what appeared to be a tumor near the ureteropelvic junction (Fig. 3A and B). A left retrograde pyelogram demonstrated a narrowed proximal left ureter involved by an extrinsic mass and a hydronephrotic collecting system (Fig. 4). A CT scan of the left thigh mass showed a solid infiltrating process involving the muscles of the medial portion of the left thigh. It was
homogeneous in appearance and displayed no areas of necrosis or hemorrhage. Open biopsy specimens of the right thigh mass and a left inguinal lymph node revealed closely packed, basophilic cells, uniform in size, with round-tonucleoli. oval nuclei and prominent Interspersed among the tumor cells were many large macrophages, creating a “starry sky” pattern, typical of Burkitt’s lymphoma (Fig. 5).
278
UROLOGY
/ MARCH 1992 / VOLLJ.MEXXXIX,NUMBER 3
FI(:URE 6. CT Scan of abdomen demonstrating normal-appearing kidneys and ureters without hydronephrosis or ureteral lesions following completion o_fcoww of chemotherapy. The diagnosis of Burkitt’s lymphoma with hydronephrosis secondary to ureteropelvic involvement was made. The patient was entered into a phase I trial of methotrexate, bleomycin, Adriamycin, cyclophosphamide, vincristine, dexamethasone, and granulocyte-macrophage colony-stimulating factor. This chemotherapy regimen was given in three-week cycles over a four-month period. A CT scan of the abdomen performed six days after the completion of chemotherapy revealed normal-appearing kidneys and ureters bilaterallly without evidence of hydronephrosis or ureteral lesions (Fig. 6). The right thigh mass was no longer present on CT scan or physical examination. Likewise, the abdominal pain and the flank pain had resolved. The patient had been in complete remission from his Burkitt’s lymphoma six months after chemotherapy was begun. However, the patient was found to have lymphoma of the central nervous system, and succumbed to his disease five month5 later. Comment Lymphomas are characterized by the neoplastic proliferation of cells within the elements of the reticuloendothelial system-lymph nodes, bone marrow, spleen, and liver. The term malignant lymphoma emphasizes the predictable patterns of tumor involvement and spread within the lymphoreticular tissues. Malignant lymphomas are usually divided into two groups, Hodgkin’s disease and non-Hodgkin’s lymphomas. The distinguishing histologic feature of Hodgkin’s disease is the presence of giant
multinucleated cells, called Sternberg-Reed cells, in affected lymph nodes, The nonHodgkin’s lymphomas are a more heterogeneous group, composed of relatively indolent disorders such as the nodular lyrnphocytic lymphomas as well as more aggressive neoplasms such as diffuse histiocystic ly,mphomas and Burkitt’s lymphoma. Burkitt’s lymphoma is composed of neoplastic cells derived from B-cell lymphoid precursors. The Epstein-Barr virus has been identified as the possible cause of this lymphoma. 1.2 Several large series, consisting of individuals with malignant lymphomas, demonstrate involvement of the genitourinary system in 4 to 7 percent of patients. 3m5Symptoms are present in only 29 to 49 percent of cases, with hematuria most commonly seen. 3.s The majority Iof cases of lymphomatous involvement of the genitourinary tract are found on postmortem examinations. 3 Hydronephrosis secondary to direct involvement of the ureter by malignant lvmphoma is an unusual finding, being present in about 0.5 percent to 7 percent of all cases of lymphoma. The patient in this report was found to have a proximal ureteral mass as the cause of the hvdronephrosis. Hydronephrosis, however, -is most commonly secondary to ureteral obstruction by retroperitoneal lymph nodes, and can be seen as compression mainly along the distal ureter with the intravenous pyelogram demonstrating lateral displacement of the tlreter by enlarged lymph nodes.3 r, Neoplasms are seen with increased frequency in patients with immune deficiency, being ten thousand times more common than in the general population.“i Immune deficiency may be congenital, iatrogenic, or acquired, as in human immunodeficiency virus infection. Lymphoproliferative disorders are the most common neoplasms found in immunocompromised populations. B An associatio-n between lymphoproliferative tumors and AIDS patients is apparent. There is a nearly two-hundred-fold increase in malignant lymphoma among AIDS patients as compared with a simil,ar control population. Another feature of non-,Hodgkin’s lymphoma among AIDS patients is the extranoda1 involvement commonly found .’ Patients with AIDS-related lymplhomas are treated with standard chemotherapy regimens, the most recent regimen consisting of methobleomycin, Adriamycin, ,cyclophostrexate, phamide. vincristine, and dexanlethasone.h The
overall response rate of standard chemotherapy protocols is about 65 to 70 percent.7 Complete responders have been found in about 50 percent of patients treated.R.g Granulocyte-macrophage colony-stimulating factors (GMCSF), a hematopoietic growth factor, has been added to chemotherapy regimens in an attempt to reduce the duration of neutropenia. lo In this case, Burkitt’s lymphoma arising in an immunocompromised individual presented as hydronephrosis secondary to direct involvement of the ureter and lower extremity masses. After a course of chemotherapy the hydronephrosis resolved, and the lower extremity masses disappeared. The resolution of the ureteral mass and hydronephrosis after a course of chemotherapy for non-Hodgkin’s lymphoma confirmed the diagnosis of a lymphomatous involvement of the ureter causing obstruction. Cases of AIDS and manifestations of AIDS are frequently seen by the urologist. When presented with tumors involving the genitourinary tract in an immunocompromised individual, rare types of tumors, such as lymphoma, should be considered.
280
New York, New York 10016 (DR. AL-ASKARI) References 1. Fishman MC, ct al: Leukemia and Lymphoma, in Medicine, Philadelphia, J.B. Lippincott Co.. 1981, pp 360-369. 2. Owens AH, and Sensenbrenner LL: The Iymphorcticular proliferative disorders, in IIarvey AM, cf (I/. (Eds): Principles and Practice of Medicine, Connecticut, ApI’leton-Cerltrlr~-Crofts, 1984, pp 565-581. 3. Watson EM, Sauer HH, and Sadugor MC: Manifestations of the lymphoblastomas in the genitourinary tract, J Ural 61: 626 (1949). 4. Rosenberg SA, Diamond IID, ‘Ihslouitz B, and Craver LF: Lymphosarcoma: a review of 1,269 cases, Medicine 40: 31 (1961). 5. Abeloff MD, and Lenhard RE: Clinical management of ureteral obstruction secondary to malignant lymphonra, Johns Hopkins Med J 134: 34 (1974). 6. Ioachim HI,, Cooper MC, and IIeIhnan CC: I,ymphomas in men at high risk for acquired immune deficiency syndrornc (AIDS), Cancer 56: 2831 (1985). 7. Beckhardt RN, ef al: Increased incidence of malignant lynphoma in AIDS: a comparison of risk groups and possible ctiologic factors, Mt Sinai J Med 55: 383 (1988). 8. Gill PS, ct al: AIDS-related malignant lynrphoma: results of prospective treatment trials, J Clinic Oncol 5: 1322 (1987). 9. Ziegler IL. ct al: Non-Hodgkin’s Ivmnhoma in 90 homosexrral men: relation to generalized lymphadenopathy and the acquired immunodcficiency syndrome, N Engl J Mcd 311: 56.5 (1984). 10. Gill PS, and Levine AM: IIIV-related malignant Iynphoma: clinical aspects, treatment and pathogenesis, Cancer Invest 6: 413 (1988). j-l
I/
UROLOGY
/
MAHCII
1992
i
VOI,UME
L
XXXIS,
NUMBKR
3