Elsevier

Surgery

Volume 119, Issue 2, February 1996, Pages 161-170
Surgery

The Italian registry for adrenal cortical carcinoma: Analysis of a multiinstitutional series of 129 patients

https://doi.org/10.1016/S0039-6060(96)80164-4Get rights and content

Background. Adrenal cortical carcinoma is an uncommon tumor with a poor prognosis. The low incidence of this tumor makes it difficult to achieve reliable data on clinical manifestations, natural history, and the impact of therapies. The purpose of this study was to evaluate such aspects in a large series.

Methods. A retrospective series of 129 cases (55 men and 74 women, mean age of 49 years) was collected from 18 surgical institutions. AT the time of diagnosis 45.7% of patients had endocrine symptoms. One hundred twenty-four patients underwent surgery, which was considered curative in 91 cases and palliative in 33. Sixty-three patients had local disease, 48 had regional disease, and 43 had distant metastases.

Results. This study confirmed a higher incidence in the 40- to 50-year-old population with a female prevalence; hormonal hyperincretion was more common in women, but it was not caused by advanced disease. The overall 5-year survival rate was 35%. Tumor stage and curative resection affected prognosis significantly. The influence of gender, side, age, and hormonal function has not been confirmed. Adjuvant therapies were ineffective in prolonging survival. Reoperated patients experienced better survival (mean, 41.5 months) than nonreoperated cases (mean, 15.6 months).

Conclusions. The poor prognosis of adrenal cortical carcinoma may be imroved by early diagnosis and complete resection. Radical surgery is the sole effective therapy, particularly in early stages. Surgical treatment of recurrence seems to improve survival and should be attempted systematically. Adjuvant therapies obtained contrasting results, and their role should be evaluated in prospective multicentric trials.

References (66)

  • PommierRF et al.

    An eleven-year experience with adrenocortical carcinoma

    Surgery

    (1992)
  • VenkateshS et al.

    Adrenal cortical carcinoma

    Cancer

    (1989)
  • NaderS et al.

    Adrenal cortical carcinoma

    Cancer

    (1983)
  • CohnK et al.

    Adrenocortical carcinoma

    Surgery

    (1986)
  • IcardP et al.

    Adrenocortical carcinoma in surgically treated patients: a retrospective study on 156 cases by the French Association of Endocrine Surgery

    Surgery

    (1992)
  • SoreideJA et al.

    Adrenal cortical carcinoma in Norway, 1970–1984

    World J Surg

    (1992)
  • DidolkarMS et al.

    Natural history of adrenal cortical carcinoma

    Cancer

    (1981)
  • LutonJP et al.

    Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy

    N Engl J Med

    (1990)
  • MacFarlaneDA

    Cancer of the adrenal cortex—the natural history, prognosis and treatment in a study of fifty-five cases

    Ann R Coll Surg Engl

    (1958)
  • SullivanM et al.

    Adrenal cortical carcinoma

    J Urol

    (1978)
  • HajjarRA et al.

    Adrenal cortical carcinoma—a study of 32 patients

    Cancer

    (1975)
  • HenleyDJ et al.

    Adrenal cortical carcinoma—a continuing challenge

    Surgery

    (1983)
  • GrondalS et al.

    Steroid profile in urine: a useful tool in the diagnosis and follow-up of adrenocortical carcinoma

    Acta Endocrinol

    (1990)
  • DegenhartHJ et al.

    In vitro studies on enzymatic activities in human adrenal tumors

    Horm Res

    (1982)
  • d'AgataRD et al.

    Steroid biosynthesis in human adrenal tumors

    Horm Metab Res

    (1987)
  • DoerrHG et al.

    Evidence of 11 beta-hidroxylase deficiency in childhood adrenocortical tumors—the plasma corticosterone/11-deoxycorticosterone ratio as a possible marker for malignancy

    Cancer

    (1987)
  • SilvaEG et al.

    Adrenocortical carcinoma: an ultrastructural study of 22 cases

    Ultrastruct Pathol

    (1982)
  • KarakousisCP et al.

    Adrenal carcinomas—histologic grading and survival

    J Surg Oncol

    (1985)
  • BelldegrunA et al.

    Incidentally discovered mass of the adrenal gland

    Surgery

    (1986)
  • GlazerHS et al.

    Nonfunctioning adrenal masses: incidental discovery on computed tomography

    AJR

    (1982)
  • ReinckeM et al.

    Diagnostik und therapie asymptomatischer nebennierentumoren

    Dtsch Med Wochenschr

    (1989)
  • CopelandPM

    The incidentally discovered adrenal mass

    Ann Surg

    (1984)
  • GeelhoedGW et al.

    Management of the adrenal “incidentaloma”

    Surgery

    (1982)
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