ABSTRACT
Leukemia is a systemic disease affecting both the hematological system and the extramedullary sites alike. Myeloid sarcoma has been defined as a localized tumor mass of immature cells of granulocytic lineage in extramedullary sites. Myeloid sarcoma usually occurs with or after the onset of systemic leukemia but rarely before it. Localized myeloid sarcoma presenting before the onset of systemic leukemic disease has been termed primary myeloid sarcoma. The most common extramedullary sites affected by myeloid sarcoma are the central neurological system and the testes. The kidneys are the most common genitourinary organ affected by myeloid sarcoma; however, the urinary bladder can be involved. Here we report a rare case of primary myeloid sarcoma of the urinary bladder.
Yeng Kwang Tay, David Pan, Sree Appu, Mark Frydenberg
Submitted March 12, 2012 - Accepted for Publication April 4, 2012
KEYWORDS: Primary myeloid sarcoma, bladder, granulomatous sarcoma
CORRESPONDENCE: Yeng Kwang Tay, MBBS, Monash Medical Centre, Melbourne, Victoria, Australia ()
CITATION: UroToday Int J. 2012 August;5(4):art 40. http://dx.doi.org/10.3834/uij.1944-5784.2012.08.13
INTRODUCTION
Leukemia is a systemic disease affecting both the hematological system and the extramedullary sites alike. Myeloid sarcoma has been defined as a localized tumor mass of immature cells of granulocytic lineage in extramedullary sites. Myeloid sarcoma usually occurs with or after the onset of systemic leukemia but rarely before it. Localized myeloid sarcoma presenting before the onset of systemic leukemic disease has been termed primary myeloid sarcoma. The most common extramedullary sites affected by myeloid sarcoma are the central neurological system and the testes. The kidneys are the most common genitourinary organ affected by myeloid sarcoma; however, the urinary bladder can be involved. Here we report a rare case of primary myeloid sarcoma of the urinary bladder.
CASE REPORT
A 79-year-old man with a past history of atrial fibrillation, hypertension, and gastric reflux presented with a 1 week history of painless hematuria, incontinence, and lower urinary tract symptoms. On examination, there was a palpable mass in the suprapubic area extending to the umbilicus. A rectal examination demonstrated a fixed pelvic mass and a normal prostate. His renal function was impaired, with a creatinine level of 166 mg/dL. His serum PSA was within the normal range at 2.5 ng/ml. A computed tomography (CT) scan of his abdomen revealed bilateral hydronephrosis, a thickened bladder wall, and pelvic lymphadenopathy (Figure 1). Initial management included bilateral percutaneous nephrostomy and indwelling catheter insertion. His renal function improved quickly and he proceeded to cystoscopic resection biopsy. Intraoperatively, the bladder was grossly abnormal with an extensive tumor noted throughout the bladder, with edematous mucosa and a firm, yellow lamina propria and muscle layer. The bladder held a small capacity of approximately 50 mls. Examination under anaesthesia revealed a hard, rigid, malignant pelvis.
The patient’s pathology revealed acute myeloid leukemia of the bladder. His bone marrow biopsy was normal. A diagnosis of extramedullary leukemia, otherwise known as myeloid sarcoma, was reached (Figure 2).
The patient declined to receive chemotherapy but underwent 16 Gy of radiotherapy over 4 fractions to control his local symptoms. His bilateral urinary obstruction responded partially to radiotherapy for a short period of time, allowing the removal of percutaneous urinary diversion. The patient was treated conservatively and passed away 3 months later from renal failure.
DISCUSSION
Myeloid sarcoma, a tumor, is composed of myeloid lineage-derived cells, or myeloblasts. It is an extramedullary manifestation of acute myeloid leukemia. This rare entity was first described by Burns in 1811 and referred to as chloroma by King in 1853 due to its characteristic green color given by myeloperoxidase. The term chloroma was replaced by granulocytic sarcoma in 1966 by Rappaport [13]. The term myeloid sarcoma was recently favored [17]. Myeloid sarcoma most commonly presents as a complication of acute myeloid leukemia; however, it can, though rarely, present before any evidence of systemic leukemia. This is termed primary myeloid sarcoma. In the majority of reported cases, systemic leukemic disease follows after a mean period of 7 months [18], hence myeloid sarcoma should be regarded as a manifestation of systemic disease and should be treated as such.
The most common form of leukemia affecting the genitourinary tract is acute lymphoblastic leukemia and the less common acute myelocytic leukemia. The kidneys are the most commonly involved genitourinary organs in patients with myeloid sarcoma, and the pattern of involvement is commonly bilateral. Myeloid infiltration of the urinary bladder is very rare. It usually occurs in children; however, it can affect octogenarians [5]. There is a male dominance of 2:1. Bladder infiltration with myeloid cells is seen usually in the setting of primary localized disease, and there has been no report in the literature of a patient presenting with relapsing leukemia affecting the bladder.
The most common presenting symptom of primary myeloid sarcoma of the bladder is hematuria. This occurs because of diminution in the number of platelets with increased capillary permeability or stasis of the smaller blood vessels, with rupture and hemorrhage caused by local infiltration. Occasionally patients present with suprapubic pain, obstructive symptoms, and upper-tract compromise. There are a total of 13 cases in the literature describing leukemia infiltration of the bladder; of those, 4 cases had primary myeloid sarcoma of the urinary bladder [2,3,8,13] (Table 1).
To date, there is no effective therapy for myeloid sarcoma of bladder and it carries a poor prognosis. It is not known whether the prognosis of localized bladder leukemia differs from disseminated disease, but it should always be treated as early manifestations of more sinister systemic disease. Control of local disease is achieved with a combination of endoscopic bladder resection and palliative radiotherapy. Systemic chemotherapy with agents such as cytoxan, vincristin, cholorambucil, and fludarabine are used to defer the onset of systemic leukemia following the diagnosis of myeloid sarcoma. Long-term survival beyond 6 months with systemic disease is rare. Due to the rarity of the disease, there is no well-defined chemotherapy regimen specific for myeloid sarcoma.
CONCLUSION
Primary myeloid sarcoma of the bladder is an extremely rare disease. It should be regarded as an initial manifestation of systemic leukemia and treated as such with chemotherapy. Local symptoms should be palliated with a combination of endoscopic resection and radiotherapy. Long-term survival of this disease is rare.
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