A 25-year-old male was admitted to the Urology Ward of West China Hospital, Sichuan University, in March 2022. One month ago, a tumor close to the urinary bladder was incidentally found during ultrasonography in routine physical examination. The records at admission showed that the blood pressure of the patient was 126/73 mmHg, and the heart rate was 80 bpm when he was inpatient. No other chief complaints or marked symptoms were presented, such as headaches, blood pressure turbulence, or abdominal pain. The patient had no history of hepatitis and tuberculosis, nor did he have a history of allergies, trauma, surgery, familial disease, alcoholism, or smoking. The physical examination showed a soft abdomen, and no mass, regional lymphadenopathy, or pain was reported.
An abdominal CT scan showed an additional left para-aortic tumor with enhanced density ( Figure 1 ). The 131I-mataiodobenzyl-guanidine (MIBG) scintigraphy results showed two radio-uptake enhancing regions in the left abdomen and pelvis after 48 and 72 h ( Figure 1E ). Laboratory tests showed an increase in the level of norepinephrine (10.37 nmol/L), normetanephrine (7.01 nmol/L), and 3-methoxytyramine (23.97 nmol/L). The characteristics, diagnosis, and therapies of the disease were discussed. The patient presented with normal blood pressure and heart rate. However, significantly increased serum catecholamines were detected. A preliminary diagnosis of multifocal PGLs was confirmed by an additional metaiodobenzylguanidine (MIBG) scan.
Laparoscopic resection of the tumor adjacent to the bladder wall and the para-aortic tumor was recommended. In order to avoid hypotensive episodes during surgery, the patient was treated with pre-operative adrenergic blockade (10 mg of the alpha adrenoreceptor blocker phenoxybenzamine). The tumor adjacent to the bladder measured 4.0 x 3.1 x 2.5 cm, and the para-aortic tumor measured 5.6 x 5.2 x 4.6 cm. The patient's postoperative course was uneventful and the patient was discharged 4 days post-surgery.
The final diagnosis was confirmed by histopathology of the specimens: The envelopes of the tumors were intact; the cystic-solid tumor close to the aorta presented a grey-white nodular morphology with hemorrhage and necrosis, cysts were individual and smooth, with yellow-brown tissues inside ( Figure 2A ); and the other nodular tumor showed a similar morphology ( Figure 2B ). Further immunochemistry results reported Syn (+), CgA (+), S-100 (+), GATA3 (+), PCK (-), HMB45 (-), and MART-1 (-), and the Ki-67 positive index was 1% ( Figures 2C-F ). Combined with the morphological features, the tumors were considered as PGLs. The patient's consent has been obtained before the acquisition of relevant data and materials.