INTRODUCTION Pheochromocytomas are tumors of chromaffin cells, derived from the embryonic neural crest, usually originate from the adrenal medulla. 10% of these tumors occur at extra-adrenal sites and are known as paragangliomas. Paraganglioma is rare account for <0.06% of all urinary bladder tumors and 6% of all extra-adrenal pheochromocytomas. It arises from chromaffin tissue of the sympathetic nervous system within the layers of the bladder wall. Most common site is the dome or the trigone of the bladder. Bladder paraganglioma is an extremely rare tumor with a high recurrence rate. CASE REPORT A 20 year old female patient presented with complaints of burning sensation during micturation and intermittent hematuria since 1 month. she had a history of similar complaint in the past and found to have bladder growth, for which she underwent TURBT 6 months back. The histopathology report was suggestive of bladder Paraganglioma. On examination general condition and vitals were stables; abdomen was soft, nontender and no lump palpable. Serum Chromogranin A level was 184 ng/ml (<108) with raised 24 hour Urine Metanephrine - 1760 (600-1600). Ultrasonography of abdomen & pelvis showed a 4.4.x 1.9 cm lesion in region of trigone on left side of bladder with increased vascularity. CECT abdomen & pelvis showed a pedunculated heterogenously enhancing lesion arising from posterolateral wall of Urinary bladder on left side of size 2.5 x 2.5 x 1.3 cm without extravesical spread. DIAGNOSIS & TREATMENT Based on past history, laboratory and imaging investigation recurrent paraganglioma was diagnosed and the patient underwent partial cystectomy. Histopathological findings were compatible with the diagnosis, on gross examination the tumor showed 2.2x2x1.3 cm submucosal lesion. On microscopy the specimen showed presence of round to polygonal cells arranged in small nests or “zellballen” pattern, separated by highly vascularized fibrous septa; on immunohistochemistry staining was strongly positve for synaptophysin and chromogranin. S-100 staining shows diffuse positivity. DISCUSSION Paraganglioma of bladder are generally solitary and submucosal in nature. Most commonly seen in women of 2 to 3 decade of life. 15-20% of these tumour are malignant, 80-85 % of it are hormonally active. Both CT scanning and MRI are useful in the localisation of both the primary tumour and any metastases however Scanning with 131 Iodine metaiodinebenzylguinidine (MIBG) has been shown to have a very high sensitivity and specificity for phaeochromocytoma detection. CONCLUSION Paraganglioma of urinary bladder have a characteristic histological and immunochemistry feature and it’s a cornerstone for diagnosis, it is found that metastatic dissemination is the only real proof of malignancy. Surgery is the mainstay of the treatment that requires total excision. Bladder paragangliomas tends to recur and metastasis, so lifelong follow-up is required.
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