Urol Radiol 12:61-64 (1990)

Urologic Radiology © Spfinger-VeflagNewYorkInc. 1990

Clinical Pathologic Conference Unilateral Ureteral Obstruction in a Patient with Acute Leukemia D a n e B l u m e n t h a l , 1 Paul R u s s o , 1 a n d J a m e s O r r 2 Departments of ~Surgery, Urology Service, and 2pathology, Memorial Sloan-Kettering Cancer Center, New York New York, USA

Case Report (Dr. P. Russo)

Radiologic Discussion (Dr. D. Blumenthal)

A 30-year-old female, 5 months status postcesarean section, presented to Memorial Sloan-Kettering Cancer Center with persistent fatigue. Physical examination revealed cervical and supraclavicular lymphadenopathy. There was no costovertebral angle tenderness or abdominal masses appreciated. The spleen was enlarged. Pelvic examination was normal. Complete blood count revealed a total leukocyte count of 13.8 x 103 #1 with 86% blasts. Hemoglobin was 5.8 g/100 dl and hematocrit was 19.6%. Serum creatinine was 0.9 mg/100 ml. A bone marrow aspiration and core biopsy revealed a hypercellular marrow with a predominance of undifferentiated blast cells consistent with acute undifferentiated leukemia. Immunohistochemical markers revealed a pattern of early myeloid differentiation. Cytogenetic analysis revealed several nondiagnostic chromosomal deletions. The patient was treated with systemic chemotherapy with daunorubicin and cystosine arabinoside for acute undifferentiated leukemia. Her clinical course was complicated by nadir sepsis and drug-induced rashes. Two cycles of chemotherapy, however, produced a complete clinical remission that lasted 4 months. Prior to a planned allogenic bone marrow transplant, the patient complained of a dull ache in her right flank. Physical examination revealed mild right costovertebral angle tenderness. In addition, several firm skin nodules were noticed on the anterior abdominal wall. Serum creatinine had risen to 2.8 mg/100 ml and creatinine clearance had decreased to 40 ml/min. A repeat bone marrow aspirate revealed 18-20% myeloid blasts, and biopsy of one skin nodule revealed a leukemic skin infiltrate (Fig. 1). Uroradiologic evaluation was initiated.

Plain a b d o m i n a l x - r a y r e v e a l e d n o e v i d e n c e o f calcification. A renal u l t r a s o u n d r e v e a l e d m a r k e d fight h y d r o n e p h r o s i s with a d e q u a t e renal p a r e n c h y m a a n d a n o r m a l - a p p e a r i n g left kidney. A b d o m i n a l a n d pelvic c o m p u t e d t o m o g r a p h i c (CT) scan with a n d witho u t c o n t r a s t d e m o n s t r a t e d a 2 - c m m a s s at the level o f the right m i d u r e t e r . T h e r e were n o a b n o r m a l calcifications (Fig. 2). T c - 9 9 D T P A r a d i o n u c l i d e scan s h o w e d p e r f u s i o n to b o t h k i d n e y s with d e l a y e d excretion on the right consistent with high grade, p r o x imal ureteral o b s t r u c t i o n . T h e r e was also d e l a y e d excretion n o t e d o n the left. C y s t o e n d o s c o p y revealed a n o r m a l u r e t h r a a n d bladder. R e t r o g r a d e p y e l o g r a p h y r e v e a l e d c o m p l e t e o b s t r u c t i o n o f the right ureter at the level o f S 1. A t t e m p t s to pass ureteral guidewires a n d stents were unsuccessful. A d o u b l e J ureteral stent was placed cystoscopically in the left renal pelvis w i t h o u t difficulty. A fight p e r c u t a n e o u s n e p h r o s t o m y tube was then placed a n d a h y d r o n e p h r o t i c drip was obtained. S e r u m creatinine decreased to 1.0 m g / 1 0 0 m l within 2 days. A n t e g r a d e n e p h r o s t o g r a m c o n f i r m e d the presence o f a h i g h - g r a d e stenosis in the p r o x i m a l right ureter with n o c o n t r a s t o b s e r v e d distal to L5 (Fig. 3). Because o f the presence o f l e u k e m i c skin nodules, the clinical p a t t e r n e m e r g i n g was felt to be t h a t o f leuk e m i c relapse with metastatic g r a n u l o c y t i c s a r c o m a . T h e differential diagnosis o f a soft tissue retroperitoneal m a s s associated with m y e l o g e n o u s leukemias, include g r a n u l o c y t i c s a r c o m a , abscess, hematoma, and lymphadenopathy.

Address reprint requests to: Paul Russo, M.D., Urology Service,

Memorial Hospital, 1275 York Avenue, New York, NY 10021, USA

Before you turn the page, what is your diagnosis?

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D. Blumenthal et al.: Unilateral Ureteral Obstruction

Fig. 1. Skin biopsy showing nodular proliferation of blasts similar to those in the marrow specimen (paraffin embedded, hematoxylin-eosin). Fig. 2. CT scan of abdomen showing a 2-cm mass (arrow) at level of right midureter. Stent present in left ureter. Fig. 3. Right antegrade nephrostogram demonstrating high-grade obstruction of proximal ureter.

Radiologic Diagnosis Metastatic granulocytic sarcoma (chloroma) to the retroperitoneum causing acute fight ureteral obstruction.

Hospital Course (Dr. P. Russo) Systemic reinduction chemotherapy with a 5-day course of mito×antione and cefoposide was administered. A 3-day course of intrathecal methotrexate was given after a spinal tap revealed leukemic blast cells in the cerebral spinal fluid. Whole brain irradiation was required for persistent blasts in the cerebral spinal fluid following intrathecal chemother-

apy. This reinduction course of chemotherapy was marked by persistent low-grade fevers, drug rashes, and a pulmonary hemorrhage treated with platelet transfusions. Renal function remained stable with right nephrostomy tube drainage. The creatinine clearance normalized. Repeat CT scan, 1 month after reinduction chemotherapy, demonstrated the disappearance of the previously noted mass in the region of the right midureter (Fig. 4). Repeat right antegrade nephrostogram 2 months after chemotherapy revealed a fully patent right ureter with free drainage of contrast material into the bladder (Fig. 5). In addition, all skin nodules disappeared.

D. Blumenthalet at.: UnilateralUreteral Obstruction

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Fig. 4. CT scan of abdomen (1 month after chemotherapy)demonstrating resolution of right midureteral soft tissue mass (arrow). Fig. 5. Repeatright antegradenephrostogram(2 months after chemotherapy) revealingresolutionof right ureteral obstructionwith contrast seen in the bladder. Focal ureteral mucosa irregularitypersists at site of previous obstruction.

The patient tolerated nephrostomy tube clamping without side effects and an intravenous urogram showed prompt visualization of both collecting systems without residual ureteral obstruction. The nephrostomy tube and left ureteral stent were removed and renal function remained normal. During this period of complete clinical remission, the patient underwent bone marrow transplantation without further urologic complications.

Discussion (Dr. Blnmenthal) Granulocytic sarcoma (GS, chloroma) is a malignant neoplasm composed of poorly differentiated myeloid cells present in extramedullary sites [ 1, 2]. It is most often associated with myelogenous leukemia. The tumor has been termed chloroma because of its frequent greenish color felt to be secondary to a high concentration of myeloperoxidase emanating from the tumor cells [3]. The tumors are typically firm and nodular [1]. Granulocytic sarcoma most often involves the cranium, orbit, and axial skeleton [4]. Other sites of granulocytic sarcoma involvement include lymph nodes, skin, breasts, arms, and the genitourinary tract [3, 5].

Granulocytic sarcoma is more frequent in children and young adults than older adults [2]. These tumors are often clinically silent and are discovered unexpectedly at postmortem examination [6]. Studies indicate the incidence of granulocytic sarcoma in patients with acute granulocytic leukemia to range from 3.1-8% [4, 6]. Although most commonly associated with acute myeloid leukemia, granulocytic sarcoma may also be found in patients with other forms of leukemia, myelofibrosis, myeloid metaplasia, and polycythemia vera [3]. Granulocytic sarcoma has been reported to involve the prostate [7], kidney [8], and bladder [9]. Belasco and colleagues reported a case of ureteral displacement in a child caused by granulocytic sarcoma in the form of a pelvic mass [ 10]. In contrast to our patient, granulocytic sarcoma is reported to cause ureteral displacement more commonly than obstruction [2, 10]. Voiding dysfunction secondary to a neurogenic bladder caused by granulocytic sarcoma induced spinal cord compression has been reported [4, 6]. Urinary incontinence or retention may result. Chaitin and colleagues pointed out that irritative voiding symptoms may result from bladder infiltration with granulocytic sarcoma [ 11 ].

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Obstructive symptoms have been reported in two cases ofgranulocytic sarcoma involving the prostate [7]. Treatment of genitourinary granulocytic sarcoma is directed at control of the systemic disease during this manifestation of relapse with systemic chemotherapy and palliation of local symptoms by local radiation therapy or urinary diversion. Chak and colleagues reported the effects of radiation therapy on localized granulocytic sarcoma [ 12]. Eightysix percent of patients with granulocytic sarcoma given 2000-2900 rad of external-beam irradiation achieved complete local response. Frame and colleagues reported two cases of granulocytic sarcoma involving the prostate which caused bladder outlet obstruction. In one case, transurethral resection of the prostate relieved the bladder outlet obstruction and 100 rad of external-beam irradiation to the prostate prevented recurrence. In the second case, combination chemotherapy (doxorubicin, vincristine, cystosine arabinoside, and prednisone) achieved systemic partial remission, but the bladder outlet obstruction resolved completely [7]. A case of biopsy-proven granulocytic sarcoma of the bladder responded completely to four courses of doxorubicin, vincristine, cytosine arabinoside, and prednisone [ 11 ]. In the present case, right percutaneous nephrostomy tube placement relieved the right ureteral obstruction and facilitated the normalization of renal function. After nephrostomy tube placement, subsequent combination chemotherapy induced a clinical complete remission and the granulocytic sarcomas, including the retroperitoneal mass responsible for the right ureteral obstruction, resolved. In this case, local radiation therapy to the retroperitoneum was not necessary to alleviate the ureteral obstruction. Percutaneous nephrostomy is an effective means of preserving renal function and monitoring the response to therapy via antegrade studies when granulocytic sarcoma causes complete ureteral obstruction.

D. Blumenthal et al.: Unilateral Ureteral Obstruction

Final Clinical Diagnosis Retroperitoneal granulocytic sarcoma causing right ureteral obstruction. Acknowledgment. Dr. Russo is a recipient of the American Cancer Clinical Oncology Career Development Award and Richard Molin Foundation Award for Cancer Research.

References 1. Sowers J J, Moody DM, Nadich TP, Ball MR, Laster DW, Leeds NE: Radiographic features of granulocytic sarcoma (choloroma). J Comput Assist Tomogr 3:226-233, 1979 2. Mason TE, Demarce RS, Margolis CI: Granulocytic sarcoma (chloroma) two year preceding myelogenous leukemia. Cancer 31:423-432, 1973 3. Neiman RS, Barcos M, Berard C, Bonnet H, Mann R, Rydell R, Bennett J: Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer 48:1426-1437, 1981 4. Liu PI, Ishimaru T, McGregor DH, Okada H, Steer A: Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia, Hiroshima-Nagasaki, 19491969. Cancer 31:948-955, 1973 5. Breatnach E, Stanley R J, Carpenter JT: Case report. Intrarenal chloroma causing obstructive nephropathy: CT characteristics. J Comput Assist Tomogr 9:822-824, 1985 6. Muss HB, Moloney WC: Chloroma and other myeloblastic tumors. Blood 42:721-728, 1973 7. Frame R, Head D, Lee R, Craven C, Ward J: Granulocytic sarcoma of the prostate. Cancer 59:142-146, 1987 8. Klein B, Falkson G, Simson IW, Stevens K, DeVilliers FJ, Keogram PG, Thatcher CJ: Granulocytic sarcoma of the kidney in a patient with acute myelomonocytic leukemia. S Afr Med J 70:696-698, 1986 9. McLeod AJ, Lewis E, Cox D: Chloroma of the urinary bladder: sonographic findings. J Clin Ultrasound 12:434--435, 1984 10. Belasco JB, Bryan JH, McMillan CW: Acute promyelocytic leukemia presenting as a pelvic mass. Med Pediatr Oncol 4: 289-295, 1978 11. Chaitin BA, Manning JT, Ordonex NG: Hematologic neoplasms with initial manifestations in lower urinary tract. Urology 23:35-42, 1984 12. Chak LY, Sapozink MD, Cox RS: Extramedullary lesions in nonlymphocytic leukemia: results of radiation therapy. Int J Radiat Oncol Biol Phys 9:1173-1176, 1981

Unilateral ureteral obstruction in a patient with acute leukemia.

Urol Radiol 12:61-64 (1990) Urologic Radiology © Spfinger-VeflagNewYorkInc. 1990 Clinical Pathologic Conference Unilateral Ureteral Obstruction in a...
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