Abstract


AUTHORS: Raúl Chervin, MD1, Mariela Leal Reyna, MD2, Ester Matilde Pardes, MD, PHD3, Reynaldo Manuel Gomez, MD1, Valeria de Miguel, MD4, Andrea Paissan, MD4, Susana Nelida Lupi, MD3, Marcela Martinez, MD5, Martín Angel, MD6 and Susana Belli, MD6 view more

AUTHORS: Raúl Chervin, MD1, Mariela Leal Reyna, MD2, Ester Matilde Pardes, MD, PHD3, Reynaldo Manuel Gomez, MD1, Valeria de Miguel, MD4, Andrea Paissan, MD4, Susana Nelida Lupi, MD3, Marcela Martinez, MD5, Martín Angel, MD6 and Susana Belli, MD6
(1) Endocrinology, Hospital de Clínicas "José de San Martín" - University of Buenos Aires, Buenos Aires, Argentina, (2) Endocrinology, Complejo Médico Churruca-Visca, Buenos Aires, Argentina, (3) Endocrinology, Hospital Ramos Mejía, Buenos Aires, Argentina, (4) Endocrinology, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, (5) Endocrinology, Hospital Cesar Milstein, Buenos Aires, Argentina, (6) Endocrine Oncology, Instituto Alexander Fleming, Buenos Aires, Argentina

INTRODUCTION: Adrenal cortex cancer (ACC) is a rare tumor with dismal prognosis. Complete resection, although enabling prolonged survival, is associated with a high recurrence rate and poor response to post-surgical treatments. However, as a subset of patients has a better outcome than the one expected, it becomes critical that survival parameters continue being assessed in order to adjust treatments to risk / benefit ratio. OBJECTIVE: perform a multicenter retrospective analysis of ACC patients evaluating the diagnostic and therapeutic methods and correlating prognostic indicators with overall survival (OS) and disease-free survival (DFS). 

MATERIALS AND METHODS: 1) Register data of our patients with pathologic diagnosis of ACC treated between1996 and 2016. 2) Calculare OS and DFS and establish their correlations by the Kaplan Meier method. 

RESULTS: 40 patients (F30 M10). Mean age 47 years (22-69). 28% were incidentalomas and abdominal pain was the most common symptom. Size was 31-200 mm. 74% were hyperfunctioning, Cushing syndrome (CS) being the most frequent, 50% of them co-secreting androgens and / or estrogens (view ACCs HOMONAL PROFILE PDF file below). DHEAS, Androstenodione (A) and testosterone were increased within tumoral ranges in 71%, 100% and 83% of virilized women respectively and in 30%, 57% and 50% of women without hyperandrogenism as well. 50% of men had similar high levels of DHEAS and A. 17α-OH-P was increased 2 to 12 times in 10/13 patients. Adrenalectomy was performed in 39/40 patients, 20% by laparoscopy. 70% of tumors were in stages ENSAT I and II while 14% and 16% in III and IV. 82% of patients with ENSAT I, II and III ACC had complete surgical resection, 40% of them without residual microscopic tumor verification. Relapse occurred in 17 patients (55%) despite half of them having been treated adjuvantly with mitotane, EDP, thalidomide or radiotherapy. 88% of relapsed patients were re-operated and / or treated with the above mentioned therapies with resulting regression, stabilization or tumor progression. Second, 3th and 4th recurrences where observed in 41, 24 and 6%. Thirteen patients died of their cancer. Mean OS was 100 months (2-288) and DFS 114 months (3-288). The OS was longer in ENSAT I and II patients compared to ENSAT III and IV (p = 0.003). Patients with DFS > 12 months had longer OS than those with DFS ≤ 12 months (p = 0.001). CS patients had shorter OS compared to nonfunctioning ACC (p = 0.001). Ki-67 (n=18) did not correlate with OS nor with DFS. 

CONCLUSIONS: There was a majority of functioning tumors, predominantly CS co-secreting androgens and / or estrogens, with worse prognosis. Androgens, estrogens and 17α-OH-P were useful tumor markers in women with and without hyperandrogenism - likewise in men. The average OS was relatively long possibly due to a high proportion of ENSAT I and II patients. OS was greater in patients with longer DFS. No valid conclusions could be drawn about KI-67 since it was determined in less than 50% of patients.

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