Background: Paragangliomas and extra-adrenal pheochromocytomas are uncommon neuroendocrine tumors with ubiquitous distribution. Malignant paraganglioma is a relatively rare entity. We report the treatment and pathological characteristics of a patient with malignant paraganglioma, and summarize the latest advances in the treatment of malignant paraganglioma based on a literature review.
Case summary: A 45-year-old Chinese woman presented to the hospital due to pain in the waist (right side) and right buttock, and was diagnosed as malignant paraganglioma after the placement of ureteral stent, implantation of ileus catheter, and transvaginal removal of the vaginal mass. After relief of intestinal obstruction, the patient received intravenous chemotherapy and peritoneal perfusion chemotherapy. Although her pelvic mass disease was stable, she developed multiple liver metastases and bone metastases. Due to the development of spinal cord compression, she underwent orthopedic surgery, followed by radiotherapy, and molecular targeted therapy with apatinib, but with poor disease control.
Conclusion: Clinical management of paraganglioma is challenging for endocrinologists and oncologists. Prospective studies are required to develop standardized therapeutic strategies for malignant paragangliomas.
Keywords: Case report; Chemotherapy; Malignant paraganglioma; Radiotherapy; Targeted therapy.
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