[Endocrine evaluation and evolution of intrasellar craniopharyngioma (CPIS): study of 8 cases].
BACKGROUND
Craniopharyngiomas generally develop either in the suprasellar region or in both suprasellar and intrasellar regions. Purely intrasellar craniopharyngiomas are rare in children; they have special clinical and radiological features and pose specific therapeutic problems.


METHODS
Eight patients (five girls and three boys), aged 7 to 17 years, were admitted from 1976 to 1992 with a diagnosis of intrasellar craniopharyngioma without extrasellar development. Search for endocrine deficiencies was performed in all these patients as well as radiological investigation (skull X-rays, CT scan and MRI).


RESULTS
Persistent delayed growth was the main complaint in seven out of the eight patients. Headache was seen in three patients. The tumor was fortuitously discovered after cranial traumatism in one. Initial endocrine investigations showed growth hormone deficiency in all patients; deficiency in thyrotropin was seen in five, in adenocorticotropin in six. Three patients had moderately increased levels of blood prolactin and four had delayed puberty with gonadotropin deficiency. Only one patient presented with diabetes insipidus. X-rays showed enlarged sella turcica in seven of the eight patients with calcifications in three and a cystic lesion in five. Two patients were operated on because of a doubtful diagnosis: their condition is stable 1.5 and 12 year later, respectively. The tumor volume remained stable with a follow-up of 1.5-9 years in five other patients; its volume gradualled increased in another patient requiring surgical removal 18 months after diagnosis. Antehypophysar deficiency generally increased in all patients, operated on or not, and five patients out of six have gonadotropin deficiency.


CONCLUSIONS
These intrasellar craniopharyngiomas remain generally stable so that the greatest care must be taken in proposing surgical treatment.
Bertherat
J
J
Service d'endocrinologie pédiatrique, hôpital Saint-Vincent-de-Paul, Paris, France.
Carel
J C
JC
Adamsbaum
C
C
Bougnères
P F
PF
Chaussain
J L
JL
D000293:Adolescent; D002648:Child; D003397:Craniopharyngioma / Q000097:blood / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D005260:Female; D006065:Gonadotropins, Pituitary / Q000097:blood*; D013006:Growth Hormone / Q000627:therapeutic use; D006801:Humans; D006966:Hyperprolactinemia / Q000150:complications*; D008297:Male; D011859:Radiography; D012189:Retrospective Studies; D012658:Sella Turcica / Q000000981:diagnostic imaging; D013972:Thyrotropin / Q000097:blood*
D006065:Gonadotropins, Pituitary; D013972:Thyrotropin; D013006:Growth Hormone
Evaluation endocrinienne et évolution des craniopharyngiomes à prédominance intrasellaire (CPIS): études de huit observations.