Surgery plays key role in treating some malignant pheochromocytoma and paraganglioma

SAN DIEGO — Surgery is important in managing some malignant pheochromocytomas and paragangliomas, according to a poster presented at The Endocrine Society annual meeting.

Surgical resection could effectively normalize or reduce levels of catecholamines and metanephrines and improve hormone-related symptoms and hypertension and, whether complete or incomplete, is associated with survival despite tumor recurrence.

“This work gives an idea about outcomes of surgery for this rare disease, as available data has been extremely limited,” Veljko Strajina, MD, of the Mayo Clinic, Rochester, Minnesota, told Endocrine Today. “Even though the study is limited by small number of patients and retrospective nature, it is a contribution to informed decision making in a multidisciplinary approach to malignant pheochromocytoma or paraganglioma.”

Through a review of records from 1994 to 2014, Strajina and colleagues identified 34 patients undergoing surgery for malignant pheochromocytoma or paraganglioma (PPGL) at Mayo Clinic. Rochester Campus.

Metastases were demonstrated at the time of resection in regional lymph nodes (20), bone (12), liver (10), lung (5), peritoneum (2) and ovary (1). The median follow-up was 6 years, and the survival rate was 93% (median, 11 years) more than 5 years.

Complete resection was achieved in 16 patients (47%), with disease-free survival time 4.5 years on average, but up to 12 years; resection completeness did not significantly affect survival (P = .43).

At final follow-up, 8 patients (23%) were disease free. Median disease-free survival was shorter in patients with germline succinate dehydrogenase gene mutations (P < .01). Neither overall nor disease-free survival correlated with primary tumor location or metastases number or site(s).

High preoperative fractionated metanephrines or catecholamines were reported in 22 patients (65%) but normalized in 11 of 22 patients (50%) postoperatively, with 15 of 19 preoperatively symptomatic patients (79%) having symptom relief.

The number of antihypertensive medications was significantly reduced postoperatively among 22 patients with hormone-producing tumors; 11 remained off all, six required one and one required two. Four patients required phenoxybenzamine and a beta-adrenergic blocker.

“We see durable survival and even durable disease-free survival, with control of hormone excess in a substantial number of patients,” Strajina said. “However, the data also caution us that there is a high recurrence rate and complete resection may not be achieved in substantial number of patients.” – by Allegra Tiver

Reference:

Strajina V. Poster PP12-3. Presented at: The Endocrine Society Annual Meeting; March 5-8, 2015, San Diego.

Disclosure: Strajina reports no relevant financial disclosures.