Case Report: Octreotide plus CVD chemotherapy for the treatment of multiple metastatic paragangliomas after double resection for functional bladder paraganglioma and urothelial papilloma

Abstract

BackgroundMetastatic pheochromocytomas and paragangliomas are rare neuroendocrine tumors with a poor prognosis. Bladder paraganglioma concomitant with urothelial papilloma is even rarer. However, the rate of tumor response to cyclophosphamide–vincristine–dacarbazine (CVD) chemotherapy and 5-year overall survival for patients with metastatic PPGLs remained lower. We described, for the first time, a case of a patient with multiple metastatic bladder PGL who received octreotide LAR combined with CVD chemotherapy after urological surgery and then octreotide therapy was continued during follow-up.Case presentationA 43-year-old male patient was admitted to the urology department for frequent micturition syncope concomitant with malignant hypertension. Preoperative findings were elevated levels of normetanephrine in 24-h urine or plasma. CT and MRI indicated diagnosis of suspicious bladder paraganglioma. Transurethral resection of bladder tumor combined with laparoscopic partial cystectomy was performed successfully after preoperative phenoxybenzamine with aggressive volume repletion for 7 days. The result of postoperative pathology was immediate-risk functional bladder paraganglioma (T2N0M0, Stage II) concomitant with urothelial papilloma, and the immunohistochemistry results of PPGL were positive for Ki-67 (15%), SDHB, CgA, and SSTR2. The patient achieved enhanced recovery with normal urination and no syncope after surgery. However, the results of 18F-FDG and 18F-DOTATATE PET/CT found that the metastatic localizations of bladder PGLs were in the liver, lung, and bones at the 8th month after surgery. The patient received octreotide long-acting repeatable plus six courses of CVD chemotherapy for 6 months, and then octreotide therapy was continued every 3 months until now. Metastatic localizations were stable in CT scans, and vanillylmandelic acid in 24-h urine was maintained at lower levels during follow-up.ConclusionOctreotide long-acting repeatable plus CVD chemotherapy after surgery could achieve stable disease in the case with multiple metastatic bladder PGLs, and the following octreotide therapy could maintain a state of stable disease during the period of 6-month follow-up.

Unrecognised paraganglioma of the urinary bladder precipitating hypertensive crisis

“…Tumour recurrence, risk of malignancy and submucosal location can lend themselves to the difficulty of successful endoscopic resection. Conversely, cyclophosphamide/vincristine/dacarbazine chemotherapy has been seen to stabilise metastatic disease 31 32…”

Middle mediastinal paraganglioma: A case report and review of the literature

“…However, some patients can be completely cured after combination chemotherapy with cyclophosphamide, vincristine and dacarbazine, and stabilization of multiple metastatic paragangliomas of the bladder with octreotide in combination with cyclophosphamide, vincristine and dacarbazine chemotherapy has also been reported. [11,12] The temozolomide is also a classic drug for chemotherapy of paraganglioma. Therefore, it has been suggested that chemotherapy can be used as an adjuvant treatment for malignant paragangliomas.…”