Adrenocortical Carcinomas: A Clinicopathological Analysis of 77 Cases
Annikka Weissferdt, Alexandria Phan, Saul Suster, Cesar A Moran. University of Texas Health Science Center at Houston, Houston, TX; MD Anderson Cancer Center, Houston, TX; Medical College of Wisconsin, Milwaukee, WI
Background: Adrenocortical carcinomas are rare malignant tumors of the adrenal cortex with an incompletely understood pathogenesis and aggressive clinical behavior. Their low incidence has prevented large scale research into these tumors and uniform treatment strategies are still lacking. We report the clinicopathological features of 77 primary adrenocortical carcinomas, in one of the largest series of these tumors to date.
Design: Seventy-seven cases of primary adrenocortical carcinomas from the surgical pathology files of the MD Anderson Cancer Center and the Medical College of Wisconsin were analyzed and clinical information including presentation, treatment and follow up was obtained from the referring pathologists or institutional clinical files.
Results: The patients were 52 females and 25 males with an age range from 17 to 76 years (mean 46.8). Hormone-related symptoms were present in 23 patients. The tumor weight ranged from 26 to 3260g (mean 457.1). Thirty-eight tumors were right sided and 39 were left sided. Histologically, 30 tumors were of the conventional type, 30 tumors showed oncocytic features, 7 tumors were classified as myxoid, 7 as rhabdoid and 3 as sarcomatoid adrenocortical carcinoma. Ten patients received adjuvant chemotherapy. Clinical follow-up available for 56 patients showed that 23 patients were alive with a follow-up period from 1-259 months (mean 141.6) and 33 patients had died 4 to 439 months after diagnosis (mean survival 43.4).
Conclusions: Adrenocortical carcinomas are tumors predominantly affecting patients in the 5th decade of life with a female predilection. They can show a wide range of morphologic variability with conventional and oncocytic types being the most common. Standard chemotherapy as adjuvant therapy for adrenocortical carcinomas is still lacking. These tumors run an aggressive clinical course with a mean survival of 43.4 months. Larger studies are needed to guide future therapies and improve patient outcome.
Wednesday, March 6, 2013 1:00 PM
Poster Session VI # 53, Wednesday Afternoon