- Email: [email protected]
Granulocytic Sarcoma Presenting as a Diffuse Renal Mass before Hematological Manifestations of Acute Myelogenous Leukemia
0022-534 7/94/1526-2092$03.00/0 Vol. 152, 2092-2093, December 1994 Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1994 by AMERICAN
UROLOGICAL AssocIATION, INC.
GRANULOCYTIC SARCOMA PRESENTING AS A DIFFUSE RENAL MASS
BEFORE HEMATOLOGICAL MANIFESTATIONS OF ACUTE MYELOGENOUS LEUKEMIA MICHAEL D. BAGG, JOHN N. WETTLAUFER, DIANA S. WILLADSEN, VINCENT HO, DAVID LANE AND J. BRANTLEY THRASHER* From the Department of Surgery, Division of Urology and Departments of Radiology and Hematology I Oncology, Madigan Army Medical Center, Tacoma, Washington
ABSTRACT
Granulocytic sarcoma is a rare tumor composed of granulocytic precursor cells. The most common sites of involvement include the bones, soft tissue, lymph nodes and skin. Granulocytic sarcoma usually develops as a delayed manifestation of the disease process in patients with known acute myelogenous leukemia. To our knowledge we report the first case of renal granulocytic sarcoma presenting as the first sign of acute myelogenous leukemia. Computerized tomography and magnetic resonance imaging revealed diffuse nonspecific global tumor infiltration of the kidney and ureter. Renal granulocytic sarcoma was diagnosed on open renal biopsy and acute myelogenous leukemia on bone marrow biopsy. There has been partial response to combination chemotherapy. Renal granulocytic sarcoma may diffusely involve the kidney as the only manifestation of acute myelogenous leukemia and it should be included in the differential diagnosis of the many disease entities with global renal involvement since initial treatment is combination chemotherapy. KEY WORDS: leukemia, myelocytic, acute; sarcoma; kidney; ureter
Granulocytic sarcoma is a rare tumor composed of immature cells of the granulocytic series. I It was first described by Burns in 1811 and subsequently termed chloroma by King because the tumor had a greenish color when it was exposed to air due to the high concentration of the enzyme myeloperoxidase. 2 Generally these solid masses of leukemia cells occur in patients with known acute myelogenous leukemia with initial presentation related to cytopenia or leukocytosis. 3 However, there have been a few reports of granulocytic sarcoma in which blood or bone marrow manifestations of acute leukemia have not been documented. 4 These leukemic masses are frequently found in bones, periosteum, lymph nodes, skin and epidural structures. The infiltrating cells are identical to those in bone marrow. Kidney involvement has been reported in several autopsy studies and in a case of documented acute myelogenous leukemia. I We report a case of granulocytic sarcoma of the kidney that was confirmed before hematological manifestations of acute leukemia. CASE REPORT
A 56-year-old white man was evaluated in the emergency room and general outpatient clinic for intermittent right flank pain 1 week in duration. Physical examination at that time was significant for mild diffuse epigastric tenderness and fullness of the right upper quadrant. Urinalysis and complete blood count were normal except for mild leukocytosis of 12,900/ml. 3 with a normal machine differential, including 5.8% lymphocytes, 4.9% monocytes, 88.6% neutrophils, 0.2% eosinophils and 0.5% basophils. Computerized tomography (CT) of the abdomen revealed diffuse enlargement of the right kidney with a delayed right nephrogram. There was infiltration of the renal collecting system by a soft tissue density mass from the level of the renal pelvis to the ureterovesical junction with associated pyelocaliectasis (fig. 1). Accepted for publication March 18, 1994. The views of the authors do not purport to reflect the position of the United States Army or Department of Defense. * Requests for reprints: Madigan Army Medical Center, HSHJ-SU, Tacoma, Washington 98431-5000.
FIG. 1. CT of abdomen reveals diffuse enlargement ofright kidney with associated pyelocaliectasis.
Cystoscopy revealed no evidence of intravesical involvement. A retrograde pyelogram was attempted but it was unsuccessful due to inability to cannulate the right ureteral orifice. Magnetic resonance imaging of the abdomen supported CT findings, showing a mass iso-intense to renal parenchyma, which diffusely enlarged the kidney and extended contiguously down the course of the right ureter. Superiorly the mass extended into the anterior pararenal space adjacent to the inferior vena cava (fig. 2). After gadolinium diethylenepentaacetic acid administration there was mild enhancement that was less than that of the normal renal paren-
2092
GRAl'TULOCYTIC SARCOMA PRESENTING AS RENAL MASS
2093
kemia (French, American and British classification M2). Initial treatment included combination chemotherapy, consisting of doxorubicin, arabinosylcytosine and intrathecal methotrexate. After 12 months of chemotherapy the disease remains in remission with moderate radiographic evidence of response. DISCUSSION
FIG. 2. Magnetic resonance imaging of abdomen after gadolinium diethylenepentaacetic acid administration shows right renal mass with mild enhancement of normal renal parenchyma.
chyma, which is consistent with tumor infiltration and replacement of the kidney. Right renal exploration revealed a grayish tumor involving the right kidney and adherent to the inferior vena cava, aorta, psoas muscle and periureteral tissue. On frozen section numerous lymphocytes consistent with a malignant lymphoma were seen. Nephrectomy was not performed due to the diagnosis and diffuse involvement of the retroperitoneal structures, including the great vessels. However, permanent histological examination revealed increased immature granulocytes and numerous blasts (fig. 3). Giemsa stain demonstrated neutrophilic and eosinophilic granules within the myelocytic precursors consistent with a granulocytic sarcoma. Convalescence was uneventful. Complete blood count remained normal, including the machine differential. However, review of the peripheral smear revealed less than 2% blasts. Bone marrow biopsy demonstrated predominantly blasts and promyelocytes with more than 10% of them mature beyond the promyelocyte stage with occasional Auer rods, which are fused primary granules. Primary granules are the first granules to appear during maturation of a granulocyte. Bone marrow biopsy was consistent with acute myelogenous leu-
To our knowledge our case represents the first report of granulocytic sarcoma of the kidney documented before manifestations of acute myelogenous leukemia. It is rare for a patient to present with a granulocytic sarcoma before hematological manifestations of acute myelogenous leukemia. In a report of 950 patients with granulocytic sarcoma Krause noted only 6 diagnosed with the tumor before hematological manifestations of acute myelogenous leukemia. 5 The differential diagnosis of this renal granulocytic sarcoma includes lymphoma, sarcoma, transitional cell carcinoma, leukemia, an atypical infectious process and other primary collecting system malignancies, including adenocarcinoma of the collecting ducts and squamous cell carcinoma. All of these entities may present with diffuse renal involvement on imaging. Breatnach et al described CT characteristics of atypical presentation of transitional cell carcinoma, including global enlargement of the kidney, long segment ureteral involvement by tumor and retroperitoneal lymph node involvement out of proportion to the main soft tissue mass. 6 Our case demonstrated global enlargement of the involved kidney and long segment ureteral infiltration from the level of the renal pelvis to the distal ureter. The importance of differentiating granulocytic sarcoma and malignant lymphoma from these other disease entities relates to the fact that these tumors are treated with systemic chemotherapy. 7 Therefore, a high index of suspicion is recommended in such cases to avoid unnecessary radical extirpation. 7 In summary, granulocytic sarcoma of the kidney is a rare lesion that can present before hematological manifestations of acute myelogenous leukemia. A high index of suspicion is necessary to differentiate granulocytic sarcoma from other lesions globally involving the kidney, renal pelvis and ureter. If granulocytic sarcoma is suspected, a diligent search for hematological manifestations, including bone marrow biopsy, may prevent unnecessary surgery. Dr. Mark Brissette provided pathology slides and prints. REFERENCES
1. Klein, B., Falkson, G. and Simpson, I. W.: Granulocytic sarcoma
2,
3. 4. 5. 6. 7. FIG. 3. Granulocytic sarcoma with immature granulocytes (arrows). H & E, reduced from X400.
of the kidney in a patient with acute myelomonocytic leukaemia. A case report. S. Afr. Med. J,, 70: 696, 1986. Liu, P. I., Ishimaru, T,, McGregor, D. H., Okada, H. and Steer, A: Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia. Hiroshima-Nagasaki, 1949-1969. Cancer, 31: 948, 1973. Chan, Y. F.: Granulocytic sarcoma (chloroma) of the kidney and prostate. Brit. J. Ural., 65: 655, 1990. Neiman, R. S., Barcos, M., Berard, C., Bonner, H., Mann, R., Rydell, R. and Bennett, J.: Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer, 48: 1426, 1981. Krause, J. R.: Granulocytic sarcoma preceding acute leukemia: a report of six cases. Cancer, 44: 1017, 1979. Breatnach, E., Stanley, R. J. and Carpenter, J. T., Jr.: Intrarenal chloroma causing obstructive nephropathy: CT characteristics. J. Comp. Assist. Tomogr., 9: 822, 1985. Thrasher, J. B., Perez, L. M. and Anderson, E. E.: Clinical uroradiologic conference. Number 12. Sixty-one-year-old woman with multiple bilateral renal masses. Urology, 40: 542, 1992.
THE JOURNAL OF UROLOGY
Copyright© 1994 by AMERICAN
UROLOGICAL AssocIATION, INC.
GRANULOCYTIC SARCOMA PRESENTING AS A DIFFUSE RENAL MASS
BEFORE HEMATOLOGICAL MANIFESTATIONS OF ACUTE MYELOGENOUS LEUKEMIA MICHAEL D. BAGG, JOHN N. WETTLAUFER, DIANA S. WILLADSEN, VINCENT HO, DAVID LANE AND J. BRANTLEY THRASHER* From the Department of Surgery, Division of Urology and Departments of Radiology and Hematology I Oncology, Madigan Army Medical Center, Tacoma, Washington
ABSTRACT
Granulocytic sarcoma is a rare tumor composed of granulocytic precursor cells. The most common sites of involvement include the bones, soft tissue, lymph nodes and skin. Granulocytic sarcoma usually develops as a delayed manifestation of the disease process in patients with known acute myelogenous leukemia. To our knowledge we report the first case of renal granulocytic sarcoma presenting as the first sign of acute myelogenous leukemia. Computerized tomography and magnetic resonance imaging revealed diffuse nonspecific global tumor infiltration of the kidney and ureter. Renal granulocytic sarcoma was diagnosed on open renal biopsy and acute myelogenous leukemia on bone marrow biopsy. There has been partial response to combination chemotherapy. Renal granulocytic sarcoma may diffusely involve the kidney as the only manifestation of acute myelogenous leukemia and it should be included in the differential diagnosis of the many disease entities with global renal involvement since initial treatment is combination chemotherapy. KEY WORDS: leukemia, myelocytic, acute; sarcoma; kidney; ureter
Granulocytic sarcoma is a rare tumor composed of immature cells of the granulocytic series. I It was first described by Burns in 1811 and subsequently termed chloroma by King because the tumor had a greenish color when it was exposed to air due to the high concentration of the enzyme myeloperoxidase. 2 Generally these solid masses of leukemia cells occur in patients with known acute myelogenous leukemia with initial presentation related to cytopenia or leukocytosis. 3 However, there have been a few reports of granulocytic sarcoma in which blood or bone marrow manifestations of acute leukemia have not been documented. 4 These leukemic masses are frequently found in bones, periosteum, lymph nodes, skin and epidural structures. The infiltrating cells are identical to those in bone marrow. Kidney involvement has been reported in several autopsy studies and in a case of documented acute myelogenous leukemia. I We report a case of granulocytic sarcoma of the kidney that was confirmed before hematological manifestations of acute leukemia. CASE REPORT
A 56-year-old white man was evaluated in the emergency room and general outpatient clinic for intermittent right flank pain 1 week in duration. Physical examination at that time was significant for mild diffuse epigastric tenderness and fullness of the right upper quadrant. Urinalysis and complete blood count were normal except for mild leukocytosis of 12,900/ml. 3 with a normal machine differential, including 5.8% lymphocytes, 4.9% monocytes, 88.6% neutrophils, 0.2% eosinophils and 0.5% basophils. Computerized tomography (CT) of the abdomen revealed diffuse enlargement of the right kidney with a delayed right nephrogram. There was infiltration of the renal collecting system by a soft tissue density mass from the level of the renal pelvis to the ureterovesical junction with associated pyelocaliectasis (fig. 1). Accepted for publication March 18, 1994. The views of the authors do not purport to reflect the position of the United States Army or Department of Defense. * Requests for reprints: Madigan Army Medical Center, HSHJ-SU, Tacoma, Washington 98431-5000.
FIG. 1. CT of abdomen reveals diffuse enlargement ofright kidney with associated pyelocaliectasis.
Cystoscopy revealed no evidence of intravesical involvement. A retrograde pyelogram was attempted but it was unsuccessful due to inability to cannulate the right ureteral orifice. Magnetic resonance imaging of the abdomen supported CT findings, showing a mass iso-intense to renal parenchyma, which diffusely enlarged the kidney and extended contiguously down the course of the right ureter. Superiorly the mass extended into the anterior pararenal space adjacent to the inferior vena cava (fig. 2). After gadolinium diethylenepentaacetic acid administration there was mild enhancement that was less than that of the normal renal paren-
2092
GRAl'TULOCYTIC SARCOMA PRESENTING AS RENAL MASS
2093
kemia (French, American and British classification M2). Initial treatment included combination chemotherapy, consisting of doxorubicin, arabinosylcytosine and intrathecal methotrexate. After 12 months of chemotherapy the disease remains in remission with moderate radiographic evidence of response. DISCUSSION
FIG. 2. Magnetic resonance imaging of abdomen after gadolinium diethylenepentaacetic acid administration shows right renal mass with mild enhancement of normal renal parenchyma.
chyma, which is consistent with tumor infiltration and replacement of the kidney. Right renal exploration revealed a grayish tumor involving the right kidney and adherent to the inferior vena cava, aorta, psoas muscle and periureteral tissue. On frozen section numerous lymphocytes consistent with a malignant lymphoma were seen. Nephrectomy was not performed due to the diagnosis and diffuse involvement of the retroperitoneal structures, including the great vessels. However, permanent histological examination revealed increased immature granulocytes and numerous blasts (fig. 3). Giemsa stain demonstrated neutrophilic and eosinophilic granules within the myelocytic precursors consistent with a granulocytic sarcoma. Convalescence was uneventful. Complete blood count remained normal, including the machine differential. However, review of the peripheral smear revealed less than 2% blasts. Bone marrow biopsy demonstrated predominantly blasts and promyelocytes with more than 10% of them mature beyond the promyelocyte stage with occasional Auer rods, which are fused primary granules. Primary granules are the first granules to appear during maturation of a granulocyte. Bone marrow biopsy was consistent with acute myelogenous leu-
To our knowledge our case represents the first report of granulocytic sarcoma of the kidney documented before manifestations of acute myelogenous leukemia. It is rare for a patient to present with a granulocytic sarcoma before hematological manifestations of acute myelogenous leukemia. In a report of 950 patients with granulocytic sarcoma Krause noted only 6 diagnosed with the tumor before hematological manifestations of acute myelogenous leukemia. 5 The differential diagnosis of this renal granulocytic sarcoma includes lymphoma, sarcoma, transitional cell carcinoma, leukemia, an atypical infectious process and other primary collecting system malignancies, including adenocarcinoma of the collecting ducts and squamous cell carcinoma. All of these entities may present with diffuse renal involvement on imaging. Breatnach et al described CT characteristics of atypical presentation of transitional cell carcinoma, including global enlargement of the kidney, long segment ureteral involvement by tumor and retroperitoneal lymph node involvement out of proportion to the main soft tissue mass. 6 Our case demonstrated global enlargement of the involved kidney and long segment ureteral infiltration from the level of the renal pelvis to the distal ureter. The importance of differentiating granulocytic sarcoma and malignant lymphoma from these other disease entities relates to the fact that these tumors are treated with systemic chemotherapy. 7 Therefore, a high index of suspicion is recommended in such cases to avoid unnecessary radical extirpation. 7 In summary, granulocytic sarcoma of the kidney is a rare lesion that can present before hematological manifestations of acute myelogenous leukemia. A high index of suspicion is necessary to differentiate granulocytic sarcoma from other lesions globally involving the kidney, renal pelvis and ureter. If granulocytic sarcoma is suspected, a diligent search for hematological manifestations, including bone marrow biopsy, may prevent unnecessary surgery. Dr. Mark Brissette provided pathology slides and prints. REFERENCES
1. Klein, B., Falkson, G. and Simpson, I. W.: Granulocytic sarcoma
2,
3. 4. 5. 6. 7. FIG. 3. Granulocytic sarcoma with immature granulocytes (arrows). H & E, reduced from X400.
of the kidney in a patient with acute myelomonocytic leukaemia. A case report. S. Afr. Med. J,, 70: 696, 1986. Liu, P. I., Ishimaru, T,, McGregor, D. H., Okada, H. and Steer, A: Autopsy study of granulocytic sarcoma (chloroma) in patients with myelogenous leukemia. Hiroshima-Nagasaki, 1949-1969. Cancer, 31: 948, 1973. Chan, Y. F.: Granulocytic sarcoma (chloroma) of the kidney and prostate. Brit. J. Ural., 65: 655, 1990. Neiman, R. S., Barcos, M., Berard, C., Bonner, H., Mann, R., Rydell, R. and Bennett, J.: Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer, 48: 1426, 1981. Krause, J. R.: Granulocytic sarcoma preceding acute leukemia: a report of six cases. Cancer, 44: 1017, 1979. Breatnach, E., Stanley, R. J. and Carpenter, J. T., Jr.: Intrarenal chloroma causing obstructive nephropathy: CT characteristics. J. Comp. Assist. Tomogr., 9: 822, 1985. Thrasher, J. B., Perez, L. M. and Anderson, E. E.: Clinical uroradiologic conference. Number 12. Sixty-one-year-old woman with multiple bilateral renal masses. Urology, 40: 542, 1992.