Clinical Review of Adrenocortical Carcinoma Cases – a Single Institution Experience

Presentation Number: SUN 400
Date of Presentation: April 2nd, 2017

Kenneth Tompkins*1, Katja Kiseljak-Vassiliades2, Lauren Fishbein3, Christopher D Raeburn3, Maria Albuja-Cruz3, Stephen Leong3 and Margaret E Wierman4
1Univ of Colorado School of Medicine, Aurora, CO, 2University of Colorado School of Medicine and Research Service VAMC, Aurora, CO, 3University of Colorado School of Medicine, Aurora, CO, 4University of Colorado School of Medicine and Research Service Veterans Affairs Medical Center, Denver, CO

Abstract

Adrenocortical carcinoma (ACC) is an uncommon endocrine malignancy that is associated with significant morbidity and mortality. Given the poor survival and limited understanding of mechanisms underlying ACC, additional research is urgently needed. In recent years, a multidisciplinary program combining efforts of endocrinologists, oncologists and endocrine surgeons has been initiated at our institution. Since 2013, we have seen 18 patients with ACC in our multidisciplinary clinic. Mean age at diagnosis was 50.6 years (range 16.5 to 80.8) with 12/18 (66%) female patients. Abdominal pain from local tumor growth was the most common presentation (7/18, 38%). Primary tumors ranged in size from 4.0 to 22.5 cm, with a median of 9.5 cm, and the majority were left sided (13/18, 72%). Hormone overproduction was seen in 11/18 (61%) at presentation. Half of patients had metastatic disease at initial diagnosis (9/18) and metastasis was eventually observed in (14/18, 77%), with the liver being the most common site. Surgical resection was undertaken in 15/18 (83%) patients. Of these, six had evidence of metastatic disease at time of initial surgery. Mitotane was initiated in 94% (17/18) of patients, and of those, only 35% (6/17) were documented to achieve therapeutic levels (>14 ug/mL). Nevertheless, 88% (15/17) patients treated with mitotane developed symptoms of adrenal insufficiency and were treated with prednisone BID over hydrocortisone QID for patient ease and tolerability. The mean time to initiation of prednisone was 3.7 months and fludrocortisone was 20.09 months after mitotane therapy began. Central hypothyroidism was another common side effect of mitotane therapy (13/18, 72%). Further treatments varied as 55% of patients (10/18) received chemotherapy and 39% (7/18) received radiotherapy. Seven patients (39%) died. Median overall survival (OS) from initial diagnosis was 56 months and from diagnosis of metastasis was 38 months. Although not statistically significant, median OS from initial diagnosis showed a trend to be higher in women vs men (56.4 vs 15.17 months, p=0.11). Hormone production was not associated with a difference in survival compared to nonfunctioning tumors (p=0.52). In summary, in our patient cohort, ACC was more prevalent in women than men, consistent with previous literature, and females may have a longer overall survival. To improve patient compliance, prednisone was used to treat adrenal insufficiency after mitotane therapy and was required within months after starting mitotane. A large portion of patients also required fludrocortisone although time to initiation after mitotane treatment was longer. Even with the aggressive standard of care, significant morbidity and mortality remains in patients with ACC. Additional research is needed to better understand the genetics, natural history and mechanisms underlying ACC to change the prognosis in our patients.

 

Nothing to Disclose: KT, KK, LF, CDR, MA, SL, MEW