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Mycosis fungoides causing ureteral obstruction
MYCOSIS FUNGOIDES CAUSING URETERAL OBSTRUCTION BARRY
SEIDMAN,
HOWARD HOWARD MENDLEY
M.D.
SCHIFF, M.D. BRUCKNER, M.D. A. WULFSOHN,
M.D.
From the Departments of Urology and Neoplastic The Mount Sinai Hospital, New York, New York
Disease,
ABSTRACT - Ureteral obstruction due to malignant disease is an entity which is commonly encountered. We present a case of ureteral obstruction secondary to mycosis fungoides, a rare T-cell lymphoma. We believe this is the first case of this entity to be reported.
Mycosis fungoides is a rare T-cell lymphoma of the skin, with the potential to spread to other organ systems. There is no universal agreement regarding the benign or malignant nature of the initially diagnosed lesion, but most agree that it is primarily a lymphomatous skin condition.’ Frequently, progression to extracutaneous sites, i.e., lymph nodes, lung, spleen, liver, and kidney is found.* We herein report a case of ureteral obstruction in a patient with mycosis fungoides. To our knowledge, this is the first report of ureteral involvement in this condition. Case Report A sixty-two-year-old white man presented with a four-day history of mild left flank pain. He had a history of skin lesions noted six years previously, and skin biopsy three years later revealed mycosis mngoides. One year prior to admission, the patient had a positive inguinal node biopsy for mycosis fungoides. Over the next six months the patient underwent total body skin electron beam radiation, followed by total nodal radiotherapy (1,500 rad). After these treatments, central nervous system involvement was discovered, with malignant cells found in the cerebrospinal fluid. He then received 3,500 rad to the brain.
170
Pertinent physical findings included multiple skin lesions compatible with mycosis fungoides, and right inguinal adenopathy. Abdominal examination revealed mild left costovertebral angle tenderness. Urinalysis revealed 6 to 8 white blood cells, and blood urea nitrogen and creatinine were within normal limits. Findings on urine culture were negative. Chest x-ray film was consistent with interstitial lung disease but did not show enlarged mediastinal lymph nodes. Intravenous urography revealed left hydronephrosis, and bulb retrograde pyelography confirmed the left hydronephrosis, and narrowing of the ureter at the level of LB to L5 suggesting encasement (Fig. 1A). Trap retrograde films showed delayed drainage of contrast at thirty minutes (Fig. 1B). Percutaneous needle aspiration of the left periureteral tissues was done under fluoroscopic control. Cytologic examination of the aspirate was interpreted as being malignant. The patient was given high-dose methotrexate therapy &er which there was resolution of the skin lesions and the hydronephrosis on follow-up urography. Comment Involvement of the genitourinary tract by lymphoma is well known. In addition to ureteral
UROLOGY
/
AUGUST
1982
/
VOLUME
XX,
NUMBER2
imrolvement, invasion of kidney, 3 bladder, 4 and testes5 has been described. Lateral displacement of the ureters by enlarged para-aortic lymph nodes is typically seen on intravenous pyelography. Reticulum cell sarcoma and lymphosarcoma more commonly involve the genitourinary tract than Hodgkin disease. 5 Obstruction may be produced by extrinsic compression from metastatic disease and diffuse malignant retroperitoneal fibrosis,“* direct invasion of the ureter,” and rarely by a primary intrinsic lesion. lo There are no previous reports of ureteral involvement by mycosis finrgoides, but consideration of the natural history of the disease with subsequent extracutaneous metastases, should lead one to anticipate this complication. In the case presented extracutaneous metastases were documented prior to the development of the ureteral obstruction, and aspiration biopsy of the periureteral tissue was positive for malignant cells. It was also noted that there was prompt resolution of the hydronephrosis and ureteral tapering on the follow-up intravenous urogram. As with other lymphomas producing ureteral obstruction, instrumentation or drainage of the obstructed system should be reserved for cases which have symptomatic or infected hydronephrosis. In the present case the response to chemotherapy was rapid, and resolution of the
UROl.OGY
/
AUGUST
1982
/
VOLUME
XX,
NUMBER
2
hydronephrosis resulted. The necessity for drainage by percutaneous nephrostomy or placement of indwelling ureteral stent might be anticipated in a more resistant case. Temporary drainage might be required in bilateral obstruction with renal failure, while awaiting a response to chemotherapy or radiotherapy. 47 East 77th Street New York, New York 10021 (DR. SCHIFF) References 1. Winkelmann RK, and Caro WA: Current problems in mycosis fungoides and sezary syndrome, Annu. Rev. Med. 9% 251(1977). 2. Lutzner MA, et al: Cutaneous T-cell lymphomas: the sezary syndrome, mycosis fungoides, and related disorders, Ann. Intern. Med. 83: 534 (1975). 3. Martinez-Maldonado M, and Ramirez de Arellano GA: Renal involvement in mahgnant lymphomas, J. Ural. 95: 485 (1966). 4. S&in G, Keogh B, Moore RH, and Murphy GP: Secxmdary involvement of the bladder in malignant lymphoma, ibid. 118: 251 (1977). 5. Sussman EB, Hajdu SI, Lieberman PH, and Whitmore WF: Mahgnant lymphoma of the testis: a clinicopathologic study of 37 cases, ibid. 118: 1904 (1977). 6. Webb AJ, and Dawson-Edwards P: Malignant retroperitoneal fibrosis, Br. J. Surg. 54: 595 (1967). 7. Usher SM, Brendler H, and Ciavarra VA: Retrqleritoneal fibrosis secondarv to metastatic neonlasm, Urologv 9: 191 (1977). 8. Williams G, and Peet TND: Bilateral ureteral obstruction due to mahgnant lymphoma, ibid. 7: 649 (1976). 9. Goswami AP: Metastatic cancer to the ureter and kidney from malignant lymphoma. A review of the literature. J. Urol 117: 381 11977). 10. Braun E, Manley C, Liao K, and Boyarsky S: Intrinsic Hodgkin’s disease of the ureter, ibid. 107: 952 (1972).
171
SEIDMAN,
HOWARD HOWARD MENDLEY
M.D.
SCHIFF, M.D. BRUCKNER, M.D. A. WULFSOHN,
M.D.
From the Departments of Urology and Neoplastic The Mount Sinai Hospital, New York, New York
Disease,
ABSTRACT - Ureteral obstruction due to malignant disease is an entity which is commonly encountered. We present a case of ureteral obstruction secondary to mycosis fungoides, a rare T-cell lymphoma. We believe this is the first case of this entity to be reported.
Mycosis fungoides is a rare T-cell lymphoma of the skin, with the potential to spread to other organ systems. There is no universal agreement regarding the benign or malignant nature of the initially diagnosed lesion, but most agree that it is primarily a lymphomatous skin condition.’ Frequently, progression to extracutaneous sites, i.e., lymph nodes, lung, spleen, liver, and kidney is found.* We herein report a case of ureteral obstruction in a patient with mycosis fungoides. To our knowledge, this is the first report of ureteral involvement in this condition. Case Report A sixty-two-year-old white man presented with a four-day history of mild left flank pain. He had a history of skin lesions noted six years previously, and skin biopsy three years later revealed mycosis mngoides. One year prior to admission, the patient had a positive inguinal node biopsy for mycosis fungoides. Over the next six months the patient underwent total body skin electron beam radiation, followed by total nodal radiotherapy (1,500 rad). After these treatments, central nervous system involvement was discovered, with malignant cells found in the cerebrospinal fluid. He then received 3,500 rad to the brain.
170
Pertinent physical findings included multiple skin lesions compatible with mycosis fungoides, and right inguinal adenopathy. Abdominal examination revealed mild left costovertebral angle tenderness. Urinalysis revealed 6 to 8 white blood cells, and blood urea nitrogen and creatinine were within normal limits. Findings on urine culture were negative. Chest x-ray film was consistent with interstitial lung disease but did not show enlarged mediastinal lymph nodes. Intravenous urography revealed left hydronephrosis, and bulb retrograde pyelography confirmed the left hydronephrosis, and narrowing of the ureter at the level of LB to L5 suggesting encasement (Fig. 1A). Trap retrograde films showed delayed drainage of contrast at thirty minutes (Fig. 1B). Percutaneous needle aspiration of the left periureteral tissues was done under fluoroscopic control. Cytologic examination of the aspirate was interpreted as being malignant. The patient was given high-dose methotrexate therapy &er which there was resolution of the skin lesions and the hydronephrosis on follow-up urography. Comment Involvement of the genitourinary tract by lymphoma is well known. In addition to ureteral
UROLOGY
/
AUGUST
1982
/
VOLUME
XX,
NUMBER2
imrolvement, invasion of kidney, 3 bladder, 4 and testes5 has been described. Lateral displacement of the ureters by enlarged para-aortic lymph nodes is typically seen on intravenous pyelography. Reticulum cell sarcoma and lymphosarcoma more commonly involve the genitourinary tract than Hodgkin disease. 5 Obstruction may be produced by extrinsic compression from metastatic disease and diffuse malignant retroperitoneal fibrosis,“* direct invasion of the ureter,” and rarely by a primary intrinsic lesion. lo There are no previous reports of ureteral involvement by mycosis finrgoides, but consideration of the natural history of the disease with subsequent extracutaneous metastases, should lead one to anticipate this complication. In the case presented extracutaneous metastases were documented prior to the development of the ureteral obstruction, and aspiration biopsy of the periureteral tissue was positive for malignant cells. It was also noted that there was prompt resolution of the hydronephrosis and ureteral tapering on the follow-up intravenous urogram. As with other lymphomas producing ureteral obstruction, instrumentation or drainage of the obstructed system should be reserved for cases which have symptomatic or infected hydronephrosis. In the present case the response to chemotherapy was rapid, and resolution of the
UROl.OGY
/
AUGUST
1982
/
VOLUME
XX,
NUMBER
2
hydronephrosis resulted. The necessity for drainage by percutaneous nephrostomy or placement of indwelling ureteral stent might be anticipated in a more resistant case. Temporary drainage might be required in bilateral obstruction with renal failure, while awaiting a response to chemotherapy or radiotherapy. 47 East 77th Street New York, New York 10021 (DR. SCHIFF) References 1. Winkelmann RK, and Caro WA: Current problems in mycosis fungoides and sezary syndrome, Annu. Rev. Med. 9% 251(1977). 2. Lutzner MA, et al: Cutaneous T-cell lymphomas: the sezary syndrome, mycosis fungoides, and related disorders, Ann. Intern. Med. 83: 534 (1975). 3. Martinez-Maldonado M, and Ramirez de Arellano GA: Renal involvement in mahgnant lymphomas, J. Ural. 95: 485 (1966). 4. S&in G, Keogh B, Moore RH, and Murphy GP: Secxmdary involvement of the bladder in malignant lymphoma, ibid. 118: 251 (1977). 5. Sussman EB, Hajdu SI, Lieberman PH, and Whitmore WF: Mahgnant lymphoma of the testis: a clinicopathologic study of 37 cases, ibid. 118: 1904 (1977). 6. Webb AJ, and Dawson-Edwards P: Malignant retroperitoneal fibrosis, Br. J. Surg. 54: 595 (1967). 7. Usher SM, Brendler H, and Ciavarra VA: Retrqleritoneal fibrosis secondarv to metastatic neonlasm, Urologv 9: 191 (1977). 8. Williams G, and Peet TND: Bilateral ureteral obstruction due to mahgnant lymphoma, ibid. 7: 649 (1976). 9. Goswami AP: Metastatic cancer to the ureter and kidney from malignant lymphoma. A review of the literature. J. Urol 117: 381 11977). 10. Braun E, Manley C, Liao K, and Boyarsky S: Intrinsic Hodgkin’s disease of the ureter, ibid. 107: 952 (1972).
171