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A phéochromocytome is a tumoral affection sometimes Maligne. This tumor develops starting from the cells chromaffines adrenal medulla.

This tumor is exceptional, but touches relatively young patients (20-50 years). Its malignant character is even rarer, but frightening, with the emission of hepatic metastases and osseous.

History

Its first description goes up with 1886 per Felix Fränkel in a patient deceased of what he thought of being a cancer of the glands Surrénale S. the term Phéochromocytome appears in 1912 and corresponds to a particular coloring of the fabrics surrénaliens after fixing with chromate.

Diagnosis

Private clinic

The phéochromocytome comprises: associated with a typical symptomatic triad but inconstant associating:

  1. of the pulsatile Cephalgia S

  2. of cardiac palpitations and Tachycardia
  3. of the Sweat S profuses

  • Of other symptoms is possible:

The clinical elements of the phéochromocytome can intriquer with other disease the such Maladie of Von Hippel-Lindau, a multiple Néoplasie endocrinienne, or a Syndrome of Cushing

Biology

Proportioning of the Catecholamine S: métanéphrine and normétanéphrine urinary of the 24:00

Other metabolic disorders

Seek of a multiple Néoplasie endocrinienne of the type 2 (NEM2):
  • search for a change of proto-oncogene RET
  • search for a Hyperparathyroïdie
  • search for a medullary cancer of thyroid the

Seek of a change of gene VHL (Maladie of Von Hippel-Lindau)

Imagery

  • Scintiscanning with the MIBG (Méta-iodobenzylguanidine)
  • Scanner (Tdm) or thoraco-abdominal IRM

Treatment

Surgical

Ablation of the suprarenal. Delicate surgery within sight of the risk of hypertensive push during the intervention.
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