Craniopharyngiome

The craniopharyngiome is an embryonic tissue malformation (i.e. appeared before the birth) of the hypophyseal area. The reasons of this malformation are still unknown. The tumor, often cystic, is visible with the IRM (Imagerie by magnetic resonance) and of benign nature. It develops starting from the remainders of the pocket of Rathke, in and above the pituitary gland. The craniopharyngiome is located in the vicinity direct of very important zones of the brain for the somatic and psychic development. The proximity of the Optical nerve can lead to a reduction of the field of vision, even a blindness.

The parts close to the brain like the Pituitary gland and the Hypothalamus are at the origin of the many formation Hormones responsible for the growth, regulation of the weight, puberty, metabolism of water. Often, the first complaints of the patients are in relation to a hormonal deficit. In addition, near this area are synthesized various Protéines which plays a big role in the nychthemeral rate/rhythm (day/night), the concentration, the food behavior of the patients.

The treatment of a craniopharyngiome lately diagnosed at a child or a teenager is primarily surgical. The decision of the procedure (way initially, exerèse complete or partial) will depend on the characteristics of the tumor. Most of the time, the craniopharyngiome cannot be entirely removed, of fear of injuring the surrounding structures.

Even if ablation is complete, there exists a risk of repetition. In these cases, a new intervention can be necessary and/or a treatment by Radiothérapie. With share of rare cases where the pituitary gland is touched little, the patients will have to take regularly and with life hormones in the form of tablets, of nasal spray, or injections (subcutaneous or intramuscular).

More half of the patients develop an important overweight. The possible lesions of the Optical nerve are irreversible. One also describes disorders of the memory and attention. These after-effects involve school difficulties, sometimes even descolarisation with desocialisation.

A center of reference of the rare diseases endocriniennes of the growth exists in France. Its first goal is to improve the diagnosis and the assumption of responsibility of these patients.

Symptoms:

  • hormonal Deficit

  • Fall of the vision
  • Polyurie (to urinate more 10l/jour)
  • Polydypsie (to drink more 8l/jour)
  • Turbid of the field of vision
  • important Cephalgias with repetition
  • Delay of growth
  • Delay of puberty
  • repeated Vomiting

Associations of patients

  • Craniopharyngiome-Solidarity (France)

  • Association of the Parents of Children having Problems of Growth (France)

  • Kraniopharyngeom-Gruppe (Germany)

Center Reference

  • Center of Reference of Endocriniennes the Rare Diseases of the Growth (France)

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