Paraganglioma of the urinary bladder is a rare entity, and accounts for less than 0.5% of all bladder tumors. In the genitourinary tract, the urinary bladder is the most common site for paragangliomas. These tumors commonly present with hematuria and intermittent hyper-tension during micturition along with generalized symptoms due to raised catecholamines. Surgical removal is the treatment of choice, with most of the patients requiring at least a partial cystectomy. We herewith report a 38-year-old lady who presented with a two month history of pain abdomen, burning micturition, headache and uncontrolled hypertension. Detailed investigations revealed a pheochromocytoma of the urinary bladder, which was treated with partial cystectomy, following which her blood pressure stabilized.
"Pheochromocytomas of the urinary bladder are exceedingly rare tumors accounting for less than 0.05% of all bladder tumors and less than 1% of all pheochromocytomas. In the genitourinary tract, the urinary bladder is the most common site for pheochromocytomas (79.2%), followed by the urethra (12.7%), pelvis (4.9%), and ureter (3.2%) [82,83]. Furthermore, approximately 10% of all extra adrenal pheochromocytomas are malignant . "
[Show abstract][Hide abstract] ABSTRACT: Background
Pheochromocytoma (paraganglioma) of the urinary bladder is a rare tumor. Herein we sought to review the contemporary literature on pheochromocytomas of the urinary bladder in order to further illustrate the presentation, treatment options and outcomes of patients diagnosed with these tumors.
A comprehensive review of the current literature was conducted according to the PRISMA guidelines by accessing the NCBI PubMed database and using the search terms “paraganglioma, pheochromocytoma, bladder.” This search resulted in the identification of 186 articles published between January 1980 and April 2012 of which 80 articles were ultimately included in our analysis.
Pheochromocytomas usually occurred in young adult Caucasians (mean age, 43.3 years; range,11–84 years). According to the literature, the most common symptoms and signs of pheochromocytomas of the urinary bladder were hypertension, headache, and hematuria. Of the 77 cases that commented on catecholamine production, 65 patients had biochemically functional tumors. Approximately 20% of patients were treated by transurethral resection alone, 70% by partial cystectomy and 10% by radical cystectomy. The 75 patients with follow-up information had a mean follow-up of 35 months. At the time of last follow-up, 15 (14.2%) had disease recurrence, 10 (9.4%) had metastasis, and 65 (61.3%) were alive.
Pheochromocytomas of the urinary bladder tend to be functional and occur mostly in young adult Caucasians. Patients with localized tumors have an extremely favorable prognosis and may be managed by less aggressive modalities, whereas patients with metastatic disease have a significant reduction in survival rates despite aggressive treatment.
[Show abstract][Hide abstract] ABSTRACT: Background
Paraganglioma of the urinary bladder is a rare tumor. Herein we sought to describe a case of locally advanced paraganglioma of the urinary bladder managed by partial cystectomy and extended pelvic lymph node dissection.
The case of a 43-year old Haitian male with locally advanced paraganglioma of the urinary bladder is presented in detail. Through surgical extirpation, our patient was rendered disease-free. Eighteen months later the patient is doing well without symptoms but is noted to have subcentimeter bilateral pulmonary nodules and retroperitoneal lymph nodes. No further therapy has been initiated at this time.
Patients with localized tumors have an extremely favorable prognosis and may be managed by less aggressive modalities, whereas patients with metastatic disease have a significant reduced survival rate despite aggressive treatment.
BMC Research Notes 04/2013; 6(1):156. DOI:10.1186/1756-0500-6-156
[Show abstract][Hide abstract] ABSTRACT: Paragangliomas are rare tumors arising from neural crest tissue located outside the adrenal gland. Primary seminal vesicle paraganglioma is extremely rare entity.
A 26-year-old male patient presented with symptoms and signs of acute appendicitis where a CT of abdomen and pelvis showed an inflamed appendix and incidental finding of left seminal vesicle mass. The patient underwent uneventful laparoscopic appendectomy followed by transrectal ultrasound (TRUS) guided seminal vesicle biopsies. Histopathology revealed a neuroendocrine neoplasm consistent with paraganglioma. Surgical excision of the left seminal vesicle was carried out.
Paraganglioma of genitourinary tract is rare. The urinary bladder is the most common site, followed by the urethra, pelvis and ureter. Seminal vesicle paragangliomas were reported in association with other genitourinary organ involvement such as bladder and prostate. Isolated seminal vesicle paraganglioma is extremely rare and surgical excision remains the standard treatment for localized paraganglioma.
Primary tumors of seminal vesicle are rare and represent a diagnostic challenge. Differential diagnosis includes a list of benign and malignant tumors. Primary seminal vesicle paraganglioma is a rare but important diagnosis to be included in the differential diagnosis.
International Journal of Surgery Case Reports 07/2013; 4(10):822-824. DOI:10.1016/j.ijscr.2013.07.009
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