Adrenocortical carcinoma: presentation and outcome of a contemporary patient series

Abstract

Background Adrenocortical carcinoma (ACC) is a rare endocrine carcinoma with poor 5-year survival rates of < 40%. According to the literature, ACC is rarely an incidental imaging finding. However, presentation, treatment and outcome may differ in modern series. Design and methods We studied all patients ( n = 47, four children) from a single centre during years 2002–2018. We re-evaluated radiologic and histopathological findings and assessed treatments and outcome. We searched for possible TP53 gene defects and assessed nationwide incidence of ACC. Results In adults, incidental radiologic finding led to diagnosis in 79% at median age of 61 years. ENSAT stage I, II, III and IV was 19%, 40%, 19% and 21%, respectively. Nonenhanced CT demonstrated > 20 Hounsfield Units (HU) for all tumours (median 34 (21–45)), median size 92 mm (20–196), Ki67 17% (1–40%), Weiss score 7 (4–9) and Helsinki score 24 (4–48). ACC was more often found in the left than the right adrenal ( p < 0.05). One child had Beckwith-Wiedemann and one a TP53 mutation. In adults, the primary tumour was resected in 88 and 79% received adjuvant mitotane therapy. Median hospital stay was significantly shorter in the laparoscopic vs. open surgery group (4 (3–7) vs. 8 (5–38) days, respectively; p < 0.001). In 3/4 patients, prolonged remission of > 5 to > 10 years was achieved after repeated surgery of metastases. Overall 5-year survival was 67%, and 96% vs. 26% for ENSAT stage I–II vs. III–IV ( p < 0.0001). ENSAT stage and Ki67 predicted survival, type of surgery did not. Mitotane associated with better survival. Conclusions Contemporary ACC predominantly presents as an incidental imaging finding, characterised by HU > 20 on nonenhanced CT but variable tumour size (20–196 mm). Malignancy cannot be ruled out by small tumour size only. The 5-year survival of 96% in ENSAT stage I–III compares favourably to previous studies.

Mitotane Concentrations Influence Outcome in Patients with Advanced Adrenocortical Carcinoma

“…Our study shows that less than a quarter of patients were alive after 3 years of follow-up, with a median survival of 35 months. These data confirm the high mortality of advanced ACC [12,14]; however, they also follow the trend of a better prognosis observed in the most recent studies [13]. The present study shows that the lung is the organ most commonly involved by metastatic spread, with a higher frequency than previously reported in clinical series [14] but with a similar rate of autoptic [15] or surgical series [16,17].…”

“…In the overall group, the achievement of target mitotane levels required a median time of 6 (3-9) months from the start of therapy while 14 patients (17.5%) never achieved levels ≥ 14 mg/L. The peak of plasma mitotane concentrations was 20.8 (14.8-25.0) mg/L, which was reached after a median of 11 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20) months.…”

“…ACC may present with metastatic disease at diagnosis in about one quarter of cases, or progress to advanced disease after an initial apparently complete resection [11,12]. The prognosis of advanced ACC is generally poor when surgery is unfeasible, with a reported 5-year survival less than 15% [11,13].…”

Open vs laparoscopic adrenalectomy for localized adrenocortical carcinoma

“…The recently published guidelines on the management of ACC suggest that laparoscopic adrenalectomy is acceptable in tumours smaller than 6 cm 6 . However, several studies demonstrated that in specialized referral centres laparoscopic surgery for ACC represents the appropriate surgical approach in patients with a tumour diameter of up to 10 cm if the principles of oncologic surgery are respected 10,16 . In support of these data, in the present study, the majority of patients in the LA group (57%) had a tumour larger than 6 cm (7‐11 cm) and only one of them experienced tumour recurrence, 28 months after the laparoscopic surgery.…”

18F-fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography for preoperative planning in a rare case of hyperfunctional bilateral adrenocortical carcinoma and review of literatures

“…showed that laparoscopic adrenalectomy was successful for the selected cases with tumor size ranging from 6 to 10 cm without local invasion and resulted in shorter hospitalization and showed comparable survival rate with open adrenalectomy. [ 8 ] Our patient demonstrated that laparoscopic adrenalectomy can be safely applied to bilateral ACC with tumor size exceed to 12.5 cm. However, the 1 cm metastatic paraaortic lymph node that noted on PET/CT images could not be depicted intraoperatively due to its small size.…”