Published online Sep 26, 2022. doi: 10.12998/wjcc.v10.i27.9834
Peer-review started: April 25, 2022
First decision: June 8, 2022
Revised: July 1, 2022
Accepted: August 15, 2022
Article in press: August 15, 2022
Published online: September 26, 2022
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.
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 trans
Clinical management of paraganglioma is challenging for endocrinologists and oncologists. Prospective studies are required to develop standardized therapeutic strategies for malignant paragangliomas.
Core Tip: Paragangliomas are widely distributed and have diverse clinical manifestations. Although most paragangliomas are benign, the malignancy is involved in approximately 10% of paragangliomas. The traditional treatment of malignant paragangliomas is surgery to the primary site. Surgery followed by adjuvant radiation is used less frequently, and chemotherapy is typically reserved for the distant disease. This study indicated the diagnostic features and therapeutic strategies of malignant paraganglioma. Therapeutic strategies for malignant paragangliomas are lacking. After the initial treatment, the patient’s progression-free survival reached 21 mo. Subsequently, the patient progressed and was treated again with chemoradiotherapy, surgery, and targeted therapy.