IMAGES
IN THE
MEDICAL SCIENCES
Bladder Paraganglioma Somkiat Sangwatanaroj, MD, Daraka Khovinthasate, MD, Lilly Pathomyok, MD, Apirak Santingamkun, MD, Thiti Snabboon, MD* and Sompongse Suwanwalaikorn, MD *Division of Endocrine and Metabolism, Department of Medicine, Chulalongkorn University, Bangkok, Thailand (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 1
CASE PRESENTATION
70-year-old woman was admitted to the hospital with flash pulmonary edema and severe hypertension after voiding. She noticed the progressive symptoms of palpitation, sweating and headache after voiding for 4 months and a palpable suprapubic mass. Her blood pressure was 110/70 mm Hg but after voiding increased to 200/120 mm Hg. Results from other physical examinations were unremarkable except for a soft tissue mass discovered at the suprapubic region which had a diameter of 3 cm. Bladder paraganglioma was confirmed by elevated 24-hour urine metanephrine and normetanephrine, 48.9 mg (normal level, 52–341) and 589 mg (normal level, 88–444), respectively. MRI revealed a 7.4 cm 3 8 cm 3 7 cm lobulated mass at the postero-superior aspect of the urinary bladder with a hypersignal on T2W1 (Figure 1), which was compatible with findings from I-131-metaiodobenzylguanidine with SPECT/CT. Anterior pelvic exenteration with ileal conduit was performed without any complications. Sympathetic paraganglioma or extra-adrenal pheochromocytoma of the urinary bladder is rare with approximately 200 reported cases or ,10% of extra-adrenal pheochromocytoma. It accounts for ,0.06% of all bladder tumors and ,1% of all pheochromocytoma.1 Most tumors are solitary and localized at the dome or trigone.2 Its appearance on cystoscopy is a yellowish granulated and lobulated submucosal lesion. Presence of inter-
A
The American Journal of the Medical Sciences
mittent painless hematuria and micturition syncope or triad symptoms of pheochromocytoma after voiding or irrigating during cystoscopy should alert the physician about this tumor. Biphasic CT scan of homo- or heterogeneous hyperenhancing lesion or gadolinium-DTPA contrast-enhanced dynamic MRI of hyperintense signal changes on T2-weighted MRI sequences can help differentiate from other bladder tumors. Biopsy should be avoided because it can exacerbate the crisis. Diagnosis of bladder pheochromocytoma is based on elevated levels of catecholamine metabolites in plasma/urine, especially during the attacks, and an imaging including CT/MRI aiding with I-131-metaiodobenzylguanidine scan. In addition, the images will allow the physicians to assess the extent of disease and metastatic sites. Complete surgical removal of the mass is considered the definitive treatment after stringent blood pressure control with a-adrenergic blocking agent and adequate blood volume expansion. REFERENCES 1. Thrasher JB, Rajan AR, Perez LM, et al. Pheochromocytoma of the urinary bladder: contemporary methods of diagnosis and treatment options. Urology 1993;41:435–39. 2. Beilan J, Lawton A, Hajdenberg J, et al. Pheochromocytoma of the urinary bladder: a systematic review of the contemporary literature. BMC Urol 2013;13:22.
Volume 350, Number 1, July 2015
63
IN THE
MEDICAL SCIENCES
Bladder Paraganglioma Somkiat Sangwatanaroj, MD, Daraka Khovinthasate, MD, Lilly Pathomyok, MD, Apirak Santingamkun, MD, Thiti Snabboon, MD* and Sompongse Suwanwalaikorn, MD *Division of Endocrine and Metabolism, Department of Medicine, Chulalongkorn University, Bangkok, Thailand (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 1
CASE PRESENTATION
70-year-old woman was admitted to the hospital with flash pulmonary edema and severe hypertension after voiding. She noticed the progressive symptoms of palpitation, sweating and headache after voiding for 4 months and a palpable suprapubic mass. Her blood pressure was 110/70 mm Hg but after voiding increased to 200/120 mm Hg. Results from other physical examinations were unremarkable except for a soft tissue mass discovered at the suprapubic region which had a diameter of 3 cm. Bladder paraganglioma was confirmed by elevated 24-hour urine metanephrine and normetanephrine, 48.9 mg (normal level, 52–341) and 589 mg (normal level, 88–444), respectively. MRI revealed a 7.4 cm 3 8 cm 3 7 cm lobulated mass at the postero-superior aspect of the urinary bladder with a hypersignal on T2W1 (Figure 1), which was compatible with findings from I-131-metaiodobenzylguanidine with SPECT/CT. Anterior pelvic exenteration with ileal conduit was performed without any complications. Sympathetic paraganglioma or extra-adrenal pheochromocytoma of the urinary bladder is rare with approximately 200 reported cases or ,10% of extra-adrenal pheochromocytoma. It accounts for ,0.06% of all bladder tumors and ,1% of all pheochromocytoma.1 Most tumors are solitary and localized at the dome or trigone.2 Its appearance on cystoscopy is a yellowish granulated and lobulated submucosal lesion. Presence of inter-
A
The American Journal of the Medical Sciences
mittent painless hematuria and micturition syncope or triad symptoms of pheochromocytoma after voiding or irrigating during cystoscopy should alert the physician about this tumor. Biphasic CT scan of homo- or heterogeneous hyperenhancing lesion or gadolinium-DTPA contrast-enhanced dynamic MRI of hyperintense signal changes on T2-weighted MRI sequences can help differentiate from other bladder tumors. Biopsy should be avoided because it can exacerbate the crisis. Diagnosis of bladder pheochromocytoma is based on elevated levels of catecholamine metabolites in plasma/urine, especially during the attacks, and an imaging including CT/MRI aiding with I-131-metaiodobenzylguanidine scan. In addition, the images will allow the physicians to assess the extent of disease and metastatic sites. Complete surgical removal of the mass is considered the definitive treatment after stringent blood pressure control with a-adrenergic blocking agent and adequate blood volume expansion. REFERENCES 1. Thrasher JB, Rajan AR, Perez LM, et al. Pheochromocytoma of the urinary bladder: contemporary methods of diagnosis and treatment options. Urology 1993;41:435–39. 2. Beilan J, Lawton A, Hajdenberg J, et al. Pheochromocytoma of the urinary bladder: a systematic review of the contemporary literature. BMC Urol 2013;13:22.
Volume 350, Number 1, July 2015
63
