title: Adrenocortical cancer: mortality, hormone secretion, proliferation and urine steroids - experience from a single centre spanning three decades.
abstract: BACKGROUND
  Adrenocortical carcinoma (ACC) is a rare malignant disease with a poor prognosis. Our aims were to study survival and to explore prognostic markers.
  
  
  METHODS
  We retrospectively investigated the medical records of all 50 ACC patients at a single centre diagnosed between 1985 and 2012 and followed them up until 31/12/2014.
  
  
  RESULTS
  Of this cohort, twenty six (52 %) were females. Adrenalectomy was performed in 48 patients (96 %), and twenty seven (54 %) were treated with adjuvant cytotoxic agents. The tumor sizes ranged from 6 to 20 cm. Overall survival time was 5.5 years (0.3-19.8), the two and five-year survival was 64 and 40 %, respectively. In ENSAT stage II 25/48 patients had a median survival of 7.0 years (0.7-15.5), in stage III 8/48 this was 1.9 (0.4 - 19.8), and in stage IV 15/48 it was 1.2 (0.3-3.6) years. Seventeen patients (34 %) were still alive at the end of 2014. The total follow-up time was 8.4 (0.3-19.8) years. Cell proliferation measured with Ki-67 had a median value of 15 % (2-80) and the urinary steroid profile was clearly pathologic in 29 of 43 (67 %) tested patients. The proliferation index did not significantly predict mortality (Ki-67 ≤ 10 vs. >10 %, 9.0 vs. 3.2 years, P = 0.0833), but resection margins did (R1 vs. R2, P = 0.0066; R0 vs. R2, P < 0.0001). The urinary steroid profile did not predict mortality (normal vs. pathologic urine profile: median survival 6.6 vs. 3.3 years, P = 0.261).
  
  
  CONCLUSIONS
  The prognosis was generally poor and macroscopically positive resection margins resulted in a worse prognosis. However, some patients were still alive many years following primary surgery with no sign of residual disease.
authors.affiliation: Department of Clinical Science and Education, Karolinska Institutet, Södersjukhuset, Stockholm, Sweden. jan.calissendorff@sodersjukhuset.se.|Endocrine Section, VO Internmedicin, Södersjukhuset, Sjukhusbacken 10, 118 83, Stockholm, Sweden. jan.calissendorff@sodersjukhuset.se.
authors.forename: Jan
authors.identifier: http://orcid.org/0000-0003-2088-5814
authors.initials: J
authors.lastname: Calissendorff
authors.affiliation: Department of Clinical Science and Education, Karolinska Institutet, Södersjukhuset, Stockholm, Sweden.
authors.forename: Freja
authors.initials: F
authors.lastname: Calissendorff
authors.affiliation: Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.|Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Henrik
authors.initials: H
authors.lastname: Falhammar
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000378:metabolism / Q000652:urine*; D000306:Adrenal Cortex Neoplasms / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000315:Adrenalectomy; D018268:Adrenocortical Carcinoma / Q000378:metabolism / Q000401:mortality / Q000473:pathology / Q000601:surgery; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000450:Aldosterone / Q000378:metabolism; D049109:Cell Proliferation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006728:Hormones / Q000378:metabolism*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D006728:Hormones; D000450:Aldosterone
keywords: Adrenalectomy; Adrenocortical carcinoma; Ki-67; Mortality; Prognosis; Survival; Urinary steroid profile