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A 26-year-old young male presented with recent onset hypertension and left-sided varicocele. On physical examination, his blood pressure was elevated and varicoceles were visible. On evaluation with computed tomography of abdomen, a large left-sided para-aortic mass was seen. His 24-hour urinary metanephrines and normetanephrines levels were within normal limits. Subsequently, the mass was excised. Tumor was excised completely after carefully dissecting it from left kidney and final histopathology revealed paraganglioma and postoperatively varicoceles disappeared., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Extra-adrenal pheochromocytoma is called paraganglioma. Paraganglioma near the ureterovesical junction can be confused with urothelial carcinoma in a ureterocele. Urinary metanephrine can be an indicator for bladder paraganglioma. Metaiodobenzylguanidine scintigraphy is an excellent method not only for distinguishing bladder paraganglioma from other submucosal mesenchymal tumors but also for detecting multifocal lesions. In the present case, we did not perform a preoperative metaiodobenzylguanidine scan because the patient was asymptomatic and urinary metanephrine was negative. Partial cystectomy with ureteroneocystostomy was performed for curative treatment because the tumor was very close to the ureteral orifice., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Chromaffin cell tumors are derived from neural crest cells and can produce and secrete catecholamines. Classic symptoms include headache, palpitations, diaphoresis, and hypertension. Diagnosis is typically in adulthood, and metastatic disease is rarely found. Genetic testing is suggested with young age, extra-adrenal tumors, malignant disease, or family history of associated tumors. Our young patient's presentation of incontinence is unique, but several other atypical findings can be accounted for relative to her germline mutation. Because of the rarity of our patient's case, follow-up procedure is undefined. Documenting atypical cases such as this is central to clinician awareness and development of follow-up protocol., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Ten to twenty percent of paragangliomas occur at extra-adrenal locations and less than 1% are at the urinary bladder. The most common presenting symptom of bladder paraganglioma is hypertensive attacks precipitated by micturition and hematuria. Paraganglioma of the urinary bladder occurring at pregnancy is extremely rare. We present a case of bladder paraganglioma as an unusual cause of early preeclampsia. After termination of the pregnancy, surgical resection was performed and the histopathologic diagnosis of paraganglioma confirmed. At 24 months of follow-up the patient felt well and was normotensive without any foci of paraganglioma. Although rare, paraganglioma must be considered in the differential diagnosis of early preeclampsia.

Pheochromocytoma in patients with von Recklinghausen's neurofibromatosis is a well-known association. However, extra-adrenal pheochromocytoma with this association is rare. Herein we report a case of urinary bladder paraganglioma in a patient with neurofibromatosis.