Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia / 소아과
Korean Journal of Pediatrics
;
: 137-140, 2011.
Article
Dans Anglais
| WPRIM
| ID: wpr-190233
ABSTRACT
Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were 10x6 cm and 7.5x4.5 cm, respectively. His height was 155.1 cm (standard deviation score [SDS], -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and 9alpha-fludrocortisone; thereafter, the reduced testis size has been maintained.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Puberté précoce
/
Testicule
/
Dexaméthasone
/
Prednisolone
/
Steroid 21-hydroxylase
/
Hyperplasie congénitale des surrénales
/
Choristome surrénalien
/
Hormone corticotrope
/
Granulocytes éosinophiles
/
Établissements de soins ambulatoires
Limites du sujet:
Enfant
/
Humains
/
Mâle
langue:
Anglais
Texte intégral:
Korean Journal of Pediatrics
Année:
2011
Type:
Article
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