A 38-year-old Turkish female patient was hospitalized for macroscopic hematuria persisting for 6 months. In the patient’s medical, family, and psychosocial history, it was found that she had undergone an operation due to multinodular goiter 7 years ago, her parents were healthy and had no history of chronic disease, and she was a housewife with two children. There were no symptoms related to the adrenergic system such as hypertension, palpitation, syncope, or flushing. Urogenital system examination findings were normal.
In the laboratory tests, the fasting blood sugar was 107 mg/dl, and hemoglobin was slightly elevated at 14.2 g/dl. Microscopic hematuria was observed in the urine analysis. Urinary system ultrasonography showed that both kidney sizes and calyceal system were normal, and a solitary tumoral formation of approximately 5 × 6 cm in size, starting from the bladder dome and extending to the right lateral wall was detected (Fig. 1).
After the detection of the mass in the bladder, cystoscopy was planned under general anesthesia. Cystoscopy confirmed a 5 × 6 cm solitary tumoral lesion starting from the bladder dome and extending to the right lateral wall. A sample was obtained with a transurethral resectoscope for pathological diagnosis. During the resection of the lesion, blood pressure increased to 206/124 mmHg and heart rate increased to 189/minute. After the procedure, the patient’s blood pressure and heart rate were monitored carefully. Her vital signs were stable in the follow-ups. The cardiology clinic suggested an endocrinology consultation, stating that her vital signs were stable and that she might have a paraganglioma.
Subsequently, an endocrinological evaluation was performed for extra-adrenal pheochromocytoma (Fig. 2). A 24-hour urine analysis was performed at the endocrinology consultation. It was determined that metanephrine and normetanephrine levels were elevated to 974 mcg and 1857 mcg, respectively. Hence, the observed mass was in favor of extra-adrenal pheochromocytoma. In the staging tomography performed by the urology clinic, an open operation was decided upon. Therefore, to prevent the recurrence of hypertensive crisis and the development of vascular collapse, it was planned to perform the operation after 3 weeks of treatment with the alpha-blocker doxazosin. The doxazosin starting dose was 2 mg within the 3 weeks, which was increased to 16 mg.
The resection pathology was reported as cystitis. Mass boundaries and neighborhoods were evaluated with contrast-enhanced computerized tomography (CT). Contrast-enhanced tomography sections revealed a hyperdense lobulated mass lesion with a heterogeneous internal structure starting from the anterior wall of the bladder and extending to the right lateral wall, measuring approximately 66 × 55 mm (Fig. 3). There was no evidence of lymphadenopathy.
After endocrinological evaluation and alpha-blocker treatment, we decided to perform an open partial cystectomy. During the dissection of the mass, blood pressure increased to 216/117 mmHg and heart rate to 223/minute. The mass was excised from the bladder to a tumor-free area of approximately 1 cm (Fig. 4). When the excision was completed, blood pressure values decreased to 56/24 mmHg and heart rate to 24/minute. Vital signs stabilized after intravenous hydration and administration of sympathomimetic agents. In the postoperative period, blood pressure values continued to be normal and no surgery-related complications developed. The transurethral catheter was removed on the tenth postoperative day.
The pathological evaluation of the tumor confirmed the diagnosis of vesical paraganglioma. Chromogranin, synaptophysin, S100, and gata-3 were positive on immunohistochemical staining (Fig. 5). There was no evidence of local tumor invasion.
The first control after the operation was carried out in the first month. On physical examination, the wound site was evaluated and wound healing was considered normal. Her blood pressure was 117/46 mmHg, complete blood count and biochemical values were normal, and there was no pathological finding on the urinary ultrasonography. No additional medication or recommendation was specified in the endocrinological consultation.
The follow-up of the patient continues at 3-month intervals. It is planned to evaluate the possibility of recurrence and metastasis with contrast-enhanced CT and cystoscopy in the sixth month of the case course.