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Adrenocortical carcinoma with pure aldosterone hypersecretion

Disease definition

A very rare surgically-correctable form of primary aldosteronism (PA) due to an aldosterone-secreting adrenal malignancy.

ORPHA:231625

Classification level: Disorder
  • Synonym(s):
    • Pure APAC
    • Pure aldosterone-producing adrenocortical carcinoma
    • Pure aldosterone-secreting adrenocortical carcinoma
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: C74.0  E26.0
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Summary

Epidemiology

The prevalence of adrenocortical carcinoma with pure aldosterone hypersecretion is unknown.

Clinical description

Pure APAC is characterized by renin suppression, unilateral aldosterone hypersecretion and moderate to severe hypertension that may be associated with hypokalemia, and the presence of a large adrenal tumor. Hypokalemia may be symptomatic and present as muscular weakness, cramps, paresthesia or palpitations with or without atrial fibrillation.

Etiology

Etiology is unknown.

Diagnostic methods

The diagnosis of carcinoma is suggested by CT scan, showing a tumor exceeding 4 cm in diameter, and possible metastases and/or invasion of the inferior vena cava.

Management and treatment

Open laparotomy enables resection of the tumor and possible metastases or adjacent organs. Unless unresectable tumor or metastases are present, adrenalectomy abolishes aldosterone hypersecretion and hypokalemia in most patients. Adjuvant mitotane therapy may be indicated based on tumor scoring using the Weiss score or ENS@T score.

Prognosis

The prognosis of pure APAC is the same as for others adrenocortical carcinomas: 5-year survival is 15% in patients with metastatic adrenocortical carcinomas.

Expert reviewer(s):  Pr Laurence AMAR - Pr Pierre-François PLOUIN - Dr Olivier STEICHEN - Last update: July 2011

  A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011) Greek (2011, pdf) Polski (2011, pdf)

Detailed information

Guidelines

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.