Treatment of Childhood Pheochromocytoma and Paraganglioma

Treatment options for childhood paraganglioma and pheochromocytoma include the following:

Treatment of paraganglioma and pheochromocytoma is surgical. For secreting tumors, alpha- and beta-adrenergic blockade must be optimized before surgery. A single-institution study reviewed the experience of laparoscopic partial adrenalectomy for bilateral pheochromocytoma in patients with von Hippel-Lindau disease.[2] In eight patients, all 16 adrenalectomies were performed laparoscopically. Fourteen of the procedures were partial adrenalectomies, and two patients required a contralateral total adrenalectomy because of tumor size and diffuse multinodularity. Two patients had new ipsilateral tumors identified after a median follow-up of 5 years (range, 4–6 years), with one patient who underwent repeat partial adrenalectomy. There were no deaths during the study period.

For patients with metastatic disease, responses have been documented to some chemotherapeutic regimens such as gemcitabine and docetaxel or different combinations of vincristine, cyclophosphamide, doxorubicin, and dacarbazine.[3-5] Chemotherapy may help alleviate symptoms and facilitate surgery, although its impact on overall survival is less clear.

Responses have also been obtained with high-dose 131I-MIBG and sunitinib.[6,7]

Specific consensus guidelines for the diagnosis and management of pheochromocytoma and paraganglioma in patients with germline SDHB and SDHD pathogenic variants have been published.[8,9]