BALTIMORE – Patients who have recurrent adrenocortical carcinoma appear to have one option to increase their survival: surgery that includes complete tumor resection – but it may a viable path only if the recurrence occurred a year or more after the initial resection and diagnosis, according to an retrospective study of patients at five French university hospitals. “Adrenocortical carcinoma (ACC) is a rare, malignant tumor that has a poor prognosis, and recurrence of the tumor is considerable with 75% recurrence at 5 years,” Dr. Claire Blanchard of the Digestive and Endocrine Surgery Clinic at the Central University Hospital, Nantes, France, reported at the annual meeting of the American Association of Endocrine Surgeons. “Complete resection of recurrence is the only curative treatment.”
The researchers conducted a retrospective study of patients with at least one recurrence, diagnosed between 1980 and 2014, after initial resection of ACC, comparing outcomes in 29 patients who underwent surgery with 30 who had non-operative treatment, mainly chemotherapy and radiation.
Dr. Claire Blanchard
Patients who had an operation for recurrence more often had local recurrence, 75% vs. 10% in the nonoperative group, and more frequently had a unique site of recurrence, 97% vs. 45%, than the nonoperative patients, Dr. Blanchard said.
These other demographic and tumor characteristics were similar between the operative and nonoperative groups, respectively: age, 49 years and 53 years; gender, 63% and 79% female; Weiss score, 6 and 7; Ki-67 protein index, 23% and 24%; tumor size, 99.2 mm and 115.5 mm; ENSAT stage, 65% and 45% stages I and II; and R0 resection status of the primary tumor at initial surgery, 83% and 71%.
The univariate analysis showed that appearance of the first recurrence more than 12 months after the initial diagnosis increased a patient’s chance of survival after treatment for recurrence, Dr. Blanchard said.
“Recurrences occurred at median delay of 12 months after the initial surgery,” Dr. Blanchard said. “In the 59 patients, 24 had local recurrences and 35 had distant metastases.”
Overall median survival after the first recurrence was 91 months for patients who had surgery vs. 15 months for those who did not. Overall median survival after initial resection of the primary tumor was 133 months, with a range of 14 to 252 months, in operated patients vs. 32 months, ranging from 21 to 43 months, in those who had no surgery, Dr. Blanchard said.
Of the 29 surgery patients in the surgery group, 22 had local-regional resections, 6 of whom had adjunctive radiation of the tumor bed.
“The type of resection in recurrent ACC depends on the location of the recurrence,” senior coauthor Dr. Eric Mirallié said. “In the case of local recurrence, we resected the adrenalectomy bed and all the adjacent invaded organs.”
In this series, 6 patients had resection of the tumor bed and 16 had adjacent organ resections; 8 patients (28%) had two or more operations for recurrences. These operations involved eight splenectomies, seven resections for abdominal nodules, six nephrectomies, three distal pancreatectomies, three segmental colectomies, and two minor hepatectomies. All operations were by laparotomy.
“In cases of distant recurrence, complete metastasectomy was performed,” Dr. Mirallié said. The series reported two right hepatectomies, one liver tumorectomy, one lung tumorectomy, and one brain tumorectomy.
“Nonoperative management is reserved for nonresectable patients with recurrent adrenocortical carcinoma,” Dr. Mirallié said. “Oral chemotherapy like mitotane was always given when possible. In cases of nonresectable local recurrence, radiotherapy can be used.”
During the discussion, Dr. Bradford K. Mitchell of Michigan State University, East Lansing, said that the benefit of improved survival in surgical patients in the study may have been a function of selection bias as patients who were not operated on may have had more advanced disease.
Dr. Blanchard and her coauthors had no financial relationships to disclose.