What are the other Names for this Condition? (Also known as/Synonyms)

• Bladder Paraganglioma
• Paraganglioma of the Bladder
• PGL of the Urinary Bladder

What is Paraganglioma of the Urinary Bladder? (Definition/Background Information)

• Paraganglioma (PGL) of the Urinary Bladder is an uncommon neuroendocrine neoplasm that involves the urinary bladder wall
• Neuroendocrine tumors (NETs) are tumors showing neuroendocrine differentiation

Incidence:

• These tumors are extremely rare and account for less than 0.05% of all bladder tumors
• These tumors usually occur in middle-age; some tumors are seen in children
• Both males and females are affected
• All races and ethnic groups are at risk

Risk factors:

• A family history is known to increase one’s risk
• Other risk factors have not been well-characterized

Cause:

• The cause of tumor formation is unknown; however, several genetic mutations have been known to increase the risk for such tumors
• Germline mutations are observed when the affected individuals are typically younger, present multiple tumors and/or extra-adrenal tumors

Signs and symptoms: Ganglions are sympathetic nerves in origin and are usually functional. Functional paragangliomas result in clinical signs and symptoms related to increased catecholamine secretions and also due to mass effect.

• When the urinary bladder is involved it results in increased urinary frequency (micturition) and associated symptoms
• Increased adrenaline/noradrenaline secretions can result in sweating palpitation and anxiety
• Signs symptoms, such as paroxysmal hypertension and tachycardia (increased heart rate), can also be seen

Diagnosis:

• Blood and urine tests for catecholamines and dopamine
• Radiological studies: Specialized radiological scans using special chemicals may be used to detect the spread of the tumors
  • Ultrasound of abdomen
  • CT and MRI scan of abdomen
• Tissue biopsy of the tumor mass can help achieve a definitive diagnosis

Complications:

• Emotional stress
• Tumor metastasis to different organs and regions in rare cases
• Incomplete removal results in tumor recurrence

Treatment:

• The staging of the tumor is performed according to AJCC TNM classification
• Treatment depends on the size of the tumor
• It may require a combination of surgery, chemotherapy, and radiation therapy

Prevention: Presently, it is not possible to prevent the formation of Paraganglioma of the Genitourinary System.

Prognosis:

• It depends on the stage of the tumor
• Lower stage tumors have better prognosis than higher stage tumors
• Some studies have shown that age, size, extra-adrenal locations, and whether the tumor is secretory or not, may have a bearing on overall outcomes