- Email: [email protected]
NonHodgkin's Lymphoma Arising in the Urethra of a Man
0022-5347/96/1561-0175$03.00/0 THEJOURNAL OF UROLOGY Copyright 0 1996 by AMERICAN UROLOCICAL SOC CIA TI ON, INC.
Vol. 156, 175-176, July 1996 Printed in U S A .
NONHODGKIN’S LYMPHOMA ARISING IN THE URETHRA OF A MAN HIROSHI KITAMURA, TSUGIO UMEHARA, MASAFUMI MIYAKE, TOSHIAKI SHIMIZU, KYUHEI KOHDA AND MASAKATSU AND0 From the Departments of Urology, Hematology and Pathology, Asahikawa Red Cross Hospital, Asahikawa, Japan
KEY WORDS:urethra; prostate; lymphoma, non-Hodgkin’s
Primary malignant lymphoma is uncommon in the lower urinary tract and especially rare in the urethra. Primary extranodal lymphoma of the urethra has been previously reported in only 12 women and 1 man. We report on the second man to have malignant lymphoma of the urethra. CASE REPORT
A 44-year-old man presented with asymptomatic intermittent gross hematuria but no complaints of fever, general malaise or weight loss. Medical history was noncontributory and there were no abnormal physical signs. Subcutaneous lymph nodes were not enlarged. Cystourethroscopy demonstrated a congested, swollen prostatic urethra. The lesion extended nearly to the external sphincter and 2 small mucosal changes were seen on the bladder trigone. Urine cytology revealed atypical lymphoid cells and malignant lymphoma was suspected. Serum and free prostate specific antigen was normal. Digital rectal examination demonstrated a normal sized, elastic, soft prostate with no palpable induration. Transurethral biopsy showed many small cell lymphocytes, immunoblasts and atypical plasma cells with large nuclei (fig. 1).The tumor was classified as follicular, predominantly small cleaved cell nonsodgkin’s malignant lymphoma with diffuse areas. The specimens involved no prostatic tissues but the 2 areas of bladder mucosal change showed similar findings. Immunohistochemical markers revealed a strong positive reaction for L26, and immunohistochemical staining for immunoglobulin light chain demonstrated strong cytoplasmic positivity for lambda but no staining for kappa. In addition, the Southern blot method revealed the presence of the gene rearrangement of immunoglobulin heavy chain J H but not of T cell antigen receptorC p l (fig. 2). These results indicated the lymphoma to be of B cell origin. Chest x-ray, computerized tomography from lung to pelvis, magnetic resonance imaging of the abdomen and pelvis, g d lium scintigraphy and bone marrow aspiration from the sternum showed no evidence of any masses, enlarged lymph nodes or other abnormal signs. A total of50 Gy. radiation therapy was administered to the tumor bed. &r radiation therapy repeat transurethral biopsy (resection) of the prostatic urethra con-
Accepted for publication February 9, 1996.
firmed complete remission. Followup 9 months later showed no local or other recurrence. DISCUSSION
NonHodgkin’s lymphoma may involve the genitourinary tract primarily or secondarily. However, more than 90% of these tumors arise at nodal sites initially and affect the urogenital system secondarily.’ Although lymphomatous involvement of the urogenital tract at autopsy is relatively common, the clinical manifestations produced by these changes are comparatively infrequent. The primary site of lymphoma origin may appear to be within the genitourinary tract with the testis as the most common genitourinary site. Testicular lymphoma comprises 1to 7% of all testicular tumors. However, primary lymphoma arising in the lower urinary tract is uncommon. Involvement of the urethra by malignant lymphoma is rare with only 13 cases of primary urethral lymphoma reported previously. Only 1 of the 13 patients was a man, who presented in acute urinary retention with a mass protruding from the urethral meatus.2 It is important to differentiate lymphoma in our case from primary lymphoma of the prostate or bladder. The prostate was not the primary site of lymphoma origin since the resected specimen did not involve any prostate tissues. The bladder was not the primary site since no solid or submucosal bladder tumor was noted. This finding is supported by Guthman et al, who observed a solid tumor or submucosal mass on cystoscopy in their cases of bladder lymphoma, possibly causing bladder wall displacement.3 As a histological classification, a new working formulation for clinical disease published by the National Cancer Institute is widely used. Furthermore, lymphoma can be divided into B and T cell types by immunohistochemistry or gene rearrangement. Routine staging of nonHodgkin’s lymphoma includes a history, physical examination, blood studies, computerized tomography, magnetic resonance imaging, gallium scintigraphy and bone marrow biopsy. Clinical staging is based on the Ann Arbor staging system used for Hodgkin’s disease. While Hodgkin’s disease subsequently develops to neighboring lymph nodes, nonHodgkin’s lymphoma may not. Radiation treatment seems to be effective for lymphomas of any type of histology since residual lymphoma is rare after 20
FIG. 1. Biopsy specimen. A, lymphocytic infiltration. H & E, reduced from X40. B , tumor is composed of small cell lymphocytes, immunoblasts and atypical plasma cells. Reduced from X 150. 175
176
NONHODGKINS LYMPHOMA OF URETHRA
was Ann Arbor stage I, 50 Gy. radiation were given. For local or other recurrence chemotherapy should be done. Because of the rarity of these tumors, there is no consensus on treatment. Of the 14 reported patients, including ours, 11 had no evidence of disease after 6 months to 10 years. Excision, radiation and chemotherapy have been performed successfully in these patients. However, guidelines for treatment must be based on a large number of cases.
REFERENCES
FIG. 2. A, gene rearrangement of immunoglobulin heavy chain J H shows rearranged band (arrow). Southern blot analysis. B + H , BamH I + Hind 111. H,Hind 111. B , gene rearrangement of T cell receptor-Cpl. B, BamH I. E , EcoR V.
Gy. radiation. Therefore, 40 Gy. radiation have commonly been administered to the tumor. Since disease in our case
1. Whitmore, W. F., 111, Skarin, A. T.and Rosenthal, D. S.: Urological presentations of NonHodgkin’s lymphomas. J . Urol., 128 953, 1982. 2. Rajan, N., Allman, D., Scaglia, B., Banno, J., Stuart, R., Colombo, G. and Hernandez-Graulau, J.: NonHodgkin’s lymphoma of the male urethra. J . Urol., 153: 1916, 1995. 3. Guthman, D. A., Malek, R. S., Chapman, W. R. and Farrow, G. M.: Primary malignant lymphoma of the bladder. J . Urol., 144: 1367, 1990.
Vol. 156, 175-176, July 1996 Printed in U S A .
NONHODGKIN’S LYMPHOMA ARISING IN THE URETHRA OF A MAN HIROSHI KITAMURA, TSUGIO UMEHARA, MASAFUMI MIYAKE, TOSHIAKI SHIMIZU, KYUHEI KOHDA AND MASAKATSU AND0 From the Departments of Urology, Hematology and Pathology, Asahikawa Red Cross Hospital, Asahikawa, Japan
KEY WORDS:urethra; prostate; lymphoma, non-Hodgkin’s
Primary malignant lymphoma is uncommon in the lower urinary tract and especially rare in the urethra. Primary extranodal lymphoma of the urethra has been previously reported in only 12 women and 1 man. We report on the second man to have malignant lymphoma of the urethra. CASE REPORT
A 44-year-old man presented with asymptomatic intermittent gross hematuria but no complaints of fever, general malaise or weight loss. Medical history was noncontributory and there were no abnormal physical signs. Subcutaneous lymph nodes were not enlarged. Cystourethroscopy demonstrated a congested, swollen prostatic urethra. The lesion extended nearly to the external sphincter and 2 small mucosal changes were seen on the bladder trigone. Urine cytology revealed atypical lymphoid cells and malignant lymphoma was suspected. Serum and free prostate specific antigen was normal. Digital rectal examination demonstrated a normal sized, elastic, soft prostate with no palpable induration. Transurethral biopsy showed many small cell lymphocytes, immunoblasts and atypical plasma cells with large nuclei (fig. 1).The tumor was classified as follicular, predominantly small cleaved cell nonsodgkin’s malignant lymphoma with diffuse areas. The specimens involved no prostatic tissues but the 2 areas of bladder mucosal change showed similar findings. Immunohistochemical markers revealed a strong positive reaction for L26, and immunohistochemical staining for immunoglobulin light chain demonstrated strong cytoplasmic positivity for lambda but no staining for kappa. In addition, the Southern blot method revealed the presence of the gene rearrangement of immunoglobulin heavy chain J H but not of T cell antigen receptorC p l (fig. 2). These results indicated the lymphoma to be of B cell origin. Chest x-ray, computerized tomography from lung to pelvis, magnetic resonance imaging of the abdomen and pelvis, g d lium scintigraphy and bone marrow aspiration from the sternum showed no evidence of any masses, enlarged lymph nodes or other abnormal signs. A total of50 Gy. radiation therapy was administered to the tumor bed. &r radiation therapy repeat transurethral biopsy (resection) of the prostatic urethra con-
Accepted for publication February 9, 1996.
firmed complete remission. Followup 9 months later showed no local or other recurrence. DISCUSSION
NonHodgkin’s lymphoma may involve the genitourinary tract primarily or secondarily. However, more than 90% of these tumors arise at nodal sites initially and affect the urogenital system secondarily.’ Although lymphomatous involvement of the urogenital tract at autopsy is relatively common, the clinical manifestations produced by these changes are comparatively infrequent. The primary site of lymphoma origin may appear to be within the genitourinary tract with the testis as the most common genitourinary site. Testicular lymphoma comprises 1to 7% of all testicular tumors. However, primary lymphoma arising in the lower urinary tract is uncommon. Involvement of the urethra by malignant lymphoma is rare with only 13 cases of primary urethral lymphoma reported previously. Only 1 of the 13 patients was a man, who presented in acute urinary retention with a mass protruding from the urethral meatus.2 It is important to differentiate lymphoma in our case from primary lymphoma of the prostate or bladder. The prostate was not the primary site of lymphoma origin since the resected specimen did not involve any prostate tissues. The bladder was not the primary site since no solid or submucosal bladder tumor was noted. This finding is supported by Guthman et al, who observed a solid tumor or submucosal mass on cystoscopy in their cases of bladder lymphoma, possibly causing bladder wall displacement.3 As a histological classification, a new working formulation for clinical disease published by the National Cancer Institute is widely used. Furthermore, lymphoma can be divided into B and T cell types by immunohistochemistry or gene rearrangement. Routine staging of nonHodgkin’s lymphoma includes a history, physical examination, blood studies, computerized tomography, magnetic resonance imaging, gallium scintigraphy and bone marrow biopsy. Clinical staging is based on the Ann Arbor staging system used for Hodgkin’s disease. While Hodgkin’s disease subsequently develops to neighboring lymph nodes, nonHodgkin’s lymphoma may not. Radiation treatment seems to be effective for lymphomas of any type of histology since residual lymphoma is rare after 20
FIG. 1. Biopsy specimen. A, lymphocytic infiltration. H & E, reduced from X40. B , tumor is composed of small cell lymphocytes, immunoblasts and atypical plasma cells. Reduced from X 150. 175
176
NONHODGKINS LYMPHOMA OF URETHRA
was Ann Arbor stage I, 50 Gy. radiation were given. For local or other recurrence chemotherapy should be done. Because of the rarity of these tumors, there is no consensus on treatment. Of the 14 reported patients, including ours, 11 had no evidence of disease after 6 months to 10 years. Excision, radiation and chemotherapy have been performed successfully in these patients. However, guidelines for treatment must be based on a large number of cases.
REFERENCES
FIG. 2. A, gene rearrangement of immunoglobulin heavy chain J H shows rearranged band (arrow). Southern blot analysis. B + H , BamH I + Hind 111. H,Hind 111. B , gene rearrangement of T cell receptor-Cpl. B, BamH I. E , EcoR V.
Gy. radiation. Therefore, 40 Gy. radiation have commonly been administered to the tumor. Since disease in our case
1. Whitmore, W. F., 111, Skarin, A. T.and Rosenthal, D. S.: Urological presentations of NonHodgkin’s lymphomas. J . Urol., 128 953, 1982. 2. Rajan, N., Allman, D., Scaglia, B., Banno, J., Stuart, R., Colombo, G. and Hernandez-Graulau, J.: NonHodgkin’s lymphoma of the male urethra. J . Urol., 153: 1916, 1995. 3. Guthman, D. A., Malek, R. S., Chapman, W. R. and Farrow, G. M.: Primary malignant lymphoma of the bladder. J . Urol., 144: 1367, 1990.