A 43-year-old male patient was admitted to the Department of Urology with frequent micturition syncope concomitant with hypertension for several weeks on 4 November 2020. The patient was free of diabetes mellitus and other chronic cerebrovascular disorders. The patient also had no history of psychological, genetic, or other disorders and no family history of malignant neoplasms. The patient was taking irbesartan and amlodipine for hypertension and angina pectoris for more than 1 year. The physical examination was normal. Clinical laboratory tests including blood routine examination, blood sugar, blood lipid, serum electrolytes, liver function, and renal function were also normal. Routine urine examination of red blood cells was 5.5/HPF (normal range ≤3/HPF). Serum endocrine biomarkers were as follows: normetanephrine (NMN) 18,063.8 pmol/L (normal range ≤709.7 pmol/L), metanephrine (MN) 194.9 pmol/L (normal range ≤420.9 pmol/L), norepinephrine (NE) 8,590.5 pmol/L (normal range 413.9–4,434.2 pmol/L), epinephrine (E) 151.8 pmol/L (normal range ≤605.9 pmol/L), aldosterone (ALD) 232.19 pg/ml (normal range 40–310 pg/ml), and cortisol (COR) 347.00 nmol/L (normal range 160–660 nmol/L). Urine endocrine biomarkers were as follows: NMN in 24-h urine 5,630 nmol/24 h (normal range <312 nmol/24 h) and MN in 24-h urine 79 nmol/24 h (normal range <216 nmol/24 h). The computed tomography urography (CTU) scan indicated a bladder tumor with a sub-circular soft tissue lesion (4.8 cm × 3.7 cm) in the right anterior wall of the bladder with heterogeneous enhancement in the arterial phase and decreased enhancement of venous phase and excretory phase (; ). Magnetic resonance imaging (MRI) also indicated bladder tumor with an approximately circular signal of high T2-weighted imaging (T2WI) (4.9 cm × 3.8 cm) in the right anterior wall of the bladder (; ). Cystoscopic findings revealed a cauliflower-shaped mass with the right anterior wall of the bladder directed inward and a tip in the right wall, approximately 0.8 cm in diameter (; ). The preoperative diagnosis was suspicious bladder PGL concomitant with bladder tumor. The patient received preoperative phenoxybenzamine with aggressive volume repletion for 7 days. The patient successfully underwent laparoscopic partial cystectomy combined with TURBT ( ). The postoperative pathology report confirmed bladder PGL (T2N0M0, Stage II) concomitant with urothelial papilloma ( ), with immunohistochemistry (IHC) positive for Ki-67 (15%), chromogranin A (CgA), succinate dehydrogenase B (SDHB), somatostatin receptor 2 (SSTR2), and synapsin (Syn) of bladder PGL as well as CK20 (umbrella cells) and Ki-67 (1%) of urothelial papilloma (GAPP score of 6), and negative for S-100 of bladder PGL (; ). The final diagnosis was intermediate-risk functional bladder PGL concomitant with urothelial papilloma. The patient achieved enhanced recovery after surgery and returned to normal clinical manifestations, including normal urination and no syncope. The patient was admitted to the Cancer Center because a skull mass was found on the 8th month after surgery without regular follow-up. The 18F-FDG positron emission tomography (PET)/CT findings indicated multiple high uptake liver (2.8 cm × 2.4 cm) with a maximal standardized uptake value (SUVmax) of 17.7 and bilateral pulmonary nodules with a maximum diameter of 0.5 cm (), as well as high uptake masses and osteolytic bone destruction in the right parietal skull bone, vertebral body, and ilium (). These results revealed that the metastatic localizations of bladder PGLs were in the liver, lung, and bones. The patient underwent 18F-DOTATATE PET/CT targeted imaging as a result of the positive expression of SSTR2. The results showed multiple high uptakes of liver and lung nodules with a SUVmax of 65.0 and 2.2, respectively ( ), as well as a high uptake mass with osteolytic bone destruction of the right parietal skull and iliac bone ( ). These results of 18F-DOTATATE PET/CT confirmed the same diagnosis. The level of vanillylmandelic acid (VMA) in 24-h urine was 24.80 mg/24 h (normal range ≤12.00 mg/24 h) (). The patient received six courses of CVD chemotherapy with cyclophosphamide (1.4 g day 1), vincristine (2 mg day 1), and dacarbazine (0.4 g days 1–5) on 3 August 2021, every 16–26 days (mean 22 days). Considering the positive expression of SSTR2 in PGL tissues and the high uptake of octreotide on 18F-DOTATATE PET/CT in multiple metastases, the patient was simultaneously subjected to octreotide LAR (30 mg intramuscularly every 4 weeks) based on the recommendation of NCCN guidelines and the PROMID studies (,, ). The results of CT scans showed no significant progression in the size of the lung, liver, skull, and ilium metastases ( ). The levels of VMA in 24-h urine slowly declined during this period (). Therefore, octreotide LAR plus CVD chemotherapy could achieve stable disease of multiple metastatic bladder PGL. After finishing the sixth course of CVD chemotherapy and the latest treatment of octreotide LAR on 13 December 2021, the patient continued octreotide therapy (30 mg intramuscularly every 3 months) until now to control hormonally functional PGL. CT scans showed no significant progression of metastatic masses in the lung, liver, skull, and ilium ( ), and the level of VMA in 24-h urine was 17.10 mg/24 h after 6 months (). The patient maintained a state of stable disease during the period of 6-month follow-up. The flowchart of timeline for diagnosis and the treatment process is shown in.