RESUMO
We report a boy with possible Proteus syndrome and precocious puberty. This appears to be the first report of this association.
Assuntos
Síndrome de Proteu/complicações , Puberdade Precoce/complicações , Criança , Humanos , Masculino , Síndrome de Proteu/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
The persistent müllerian duct syndrome represents a rare form of male pseudohermaphroditism secondary to anti-müllerian hormone deficiency. We describe 2 cases of phenotypically male postpubertal patients with a uterus and tubes. Both patients presented with bilateral cryptorchidism, 1 was seen for gynecomastia and 1 was seen for an abdominal mass that was found to be a testicular tumor.
Assuntos
Criptorquidismo/complicações , Transtornos do Desenvolvimento Sexual/diagnóstico , Glicoproteínas , Inibidores do Crescimento/deficiência , Hormônios Testiculares/deficiência , Adolescente , Adulto , Hormônio Antimülleriano , Transtornos do Desenvolvimento Sexual/complicações , Transtornos do Desenvolvimento Sexual/genética , Ginecomastia/complicações , Humanos , Masculino , Ductos Paramesonéfricos , Seminoma/complicações , Neoplasias Testiculares/complicaçõesRESUMO
Prolactinoma is rare in childhood and adolescence. The earliest known diagnosis was reported in a patient at 8 years of age. We report the case of a sellar tumor found in a 6 years old girl. After a long symptomatic period she was brought for treatment. At this time she had partial papillar atrophy, hyperprolactinemia, and diminished reserve of growth hormone and cortisol. Due to rapid visual deterioration, she was submitted to a frontotemporal craniotomy for suspected craniopharyngioma. The tumor tissue immunohistochemistry was positive for prolactin. It is concluded that prolactinoma must be considered in differential diagnosis of sellar tumors in childhood since it benefits from a less aggressive therapy.