PMC9895770_01
The results of preoperative examination. (A) The computed tomography urography (CTU) scan showed a roundish soft tissue lesion (4.8 cm x 3.7 cm) in the right anterior wall of bladder with heterogeneous enhancement of arterial phase. (B, C) MRI showed an approximately circular signal of high T2WI (4.9 cm x 3.8 cm) in the right anterior wall of bladder (B) with heterogeneous enhancement of arterial phase (C). (D) Cystoscopy showed inward right anterior wall.
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The computed tomography urography (CTU) scan indicated a bladder tumor with a sub-circular soft tissue lesion (4.8 cm x 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 ( Figure 1A ;  Supplementary Figures 1A-C ). C ;  Supplementary Figures 1D-F ). 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 ( Figure 1D ;  Supplementary Figure 1G ).
The intraoperative condition and postoperative pathology results. (A) Intraoperative condition of urothelial papilloma in TURBT. (B) Intraoperative condition of bladder pheochromocytomas in laparoscopy-assisted partial cystectomy. (C) The postoperative pathology tissues of bladder pheochromocytomas. (D, E) The results of postoperative pathology with HE staining in bladder pheochromocytomas (D) and urothelial papilloma (E). (F-I) The results in IHC staining of Ki-67+ (15%) (F), SDHB(+) (G), CgA(+) (H), and SSTR2(+) (I) in bladder pheochromocytomas.
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TURBT ( Figures 2A   -C ). The postoperative pathology report confirmed bladder PGL (T2N0M0, Stage II) concomitant with urothelial papilloma ( Figures 2D   , E ), 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 ( Figures 2F-I ;  Supplementary Figures 2A-D ).
The results of 18F-DOTATATE PET/CT for metastatic localizations of paragangliomas. (A-C) Multiple high uptake localizations in liver (A, B) and lung (C). (D, E) High uptake localizations with osteolytic bone destruction of right parietal skull bone (D) and ilium (E). (F) Whole-body SPECT/CT imaging.
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The 18F-FDG positron emission tomography (PET)/CT findings indicated multiple high uptake liver (2.8 cm x 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 ( Supplementary Figures 3A-C ), as well as high uptake masses and osteolytic bone destruction in the right parietal skull bone, vertebral body, and ilium ( Supplementary Figures 3D, E ). The results showed multiple high uptakes of liver and lung nodules with a SUVmax of 65.0 and 2.2, respectively ( Figures 3A   -C ), as well as a high uptake mass with osteolytic bone destruction of the right parietal skull and iliac bone ( Figures 3D   -F ).
The results of CT and VMA in 24-h urine during octreotide plus six courses of CVD chemotherapy and the flowchart of the timeline for diagnosis and treatment process. (A-D) The size of metastatic localizations of lung had no significant progression on two courses (A), four courses (B), and six courses of chemotherapy (C), and 6-month follow-up (D). (E-H) The size of metastatic localizations of liver had no significant progression on two courses (E), four courses (F), and six courses of chemotherapy (G), and 6-month follow-up (H). (I-L) The size of metastatic localizations of skull had no significant progression on two courses (I), four courses (J), and six courses of chemotherapy (K), and 6-month follow-up (L). (M-P) The size of metastatic localizations of illum had no significant progression on two courses (M), four courses (N), and six courses of chemotherapy (O), and 6-month follow-up (P). (Q) The line graph of VMA levels in 24-h urine changes in the perioperative and chemotherapeutic period. (R) The diagnosis and treatment flowchart.
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The level of vanillylmandelic acid (VMA) in 24-h urine was 24.80 mg/24 h (normal range <=12.00 mg/24 h) ( Figure 4Q ). The results of CT scans showed no significant progression in the size of the lung, liver, skull, and ilium metastases ( Figures 4A   -L ). The levels of VMA in 24-h urine slowly declined during this period ( Figure 4Q ). 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 ( Figures 4M   -P ), and the level of VMA in 24-h urine was 17.10 mg/24 h after 6 months ( Figure 4Q ). The flowchart of timeline for diagnosis and the treatment process is shown in  Figure 4R .