Precocious Puberty Due to a Hypothalamic Hamartoma in a Patient Surviving to Late Middle Age

Wolman, Lionel; Balmforth, G. V.

doi:10.1097/00006254-196402000-00028

Cited by 11 publications

(15 citation statements)

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“…Histologically the hamartoma consists of small (hypothalamic) ganglion cells arranged in a somewhat haphazard fashion and surrounded by normal glial tissue [I], Advances have been made over the past 11 years in clarifying the mechanisms by which these tumors cause the syndrome of precocious puberty. One early hypothesis suggested a mechanism of hypothalamic 'overdrive' via direct neural connections of the tumor with the hypothalamus [8]; another proposed mechanical stimulation of the caudal hypothalamus by the tumor mass [6]. Bierich [1] however, detected unusually high levels of LHRH in the CSF of 3 patients, and Judge er at.…”

Section: Discussionmentioning

confidence: 99%

Clinical and Surgical Aspects of Hypothalamic Hamartoma Associated with Precocious Puberty in a 15-Month-Old Boy

Kammer¹,

Perlman²,

Humphreys³

et al. 1980

Pediatr Neurosurg

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A case is reviewed of precocious puberty associated with hypothalamic hamartoma in a 15-month-old boy. The authors believe this to be the first documented case in which significant reductions occurred in the level of serum testosterone and in the result of the luteinizing hormone-releasing hormone (LHRH) infusion test following surgical removal of the turner. Such surgery appears to be safe when a planned micro-surgical course is employed.

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Section: Discussionmentioning

confidence: 99%

Clinical and Surgical Aspects of Hypothalamic Hamartoma Associated with Precocious Puberty in a 15-Month-Old Boy

Kammer¹,

Perlman²,

Humphreys³

et al. 1980

Pediatr Neurosurg

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“…46) Myelinated and unmyelinated fibers have been identified among the cellular components 3,15,46) and some of these nerve fiber bundles connect to hypothalamic nuclei. 63,74) The lesion may contain ependymal cell lines, suggesting a primordial diverticulum from the third ventricle. 2) Ultrastructural …”

Section: Pathologymentioning

confidence: 99%

“…32) An abnormal afferent neuronal network originating in the HH may stimulate activity in normal GnRH cells. 63,74) Another hypothesis of mechanical compression suggests that the hypothalamic system that normally inhibits gonadotropinsecreting cells is disarranged by compression of the hypothalamus by the HH. 3,6,54) Treatment I.…”

Section: Cppmentioning

confidence: 99%

“…15,46) Between 1934 and 1963, only around 30 cases have been described. 74) Despite their rarity, HHs have become well known because of the characteristic symptoms of centraltype precocious puberty (CPP) and gelastic seizures. 2,5,8,11,72,76) Due to advances in magnetic resonance (MR) imaging, the number of cases of HH has been on the rise.…”

Section: Introductionmentioning

confidence: 99%

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Hypothalamic Hamartoma

Arita

Kurisu

Kiura

et al. 2005

Neurol. Med. Chir.(Tokyo)

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The incidence of hypothalamic hamartomas (HHs) has increased since the introduction of magnetic resonance (MR) imaging. The etiology of this anomaly and the pathogenesis of its peculiar symptoms remain unclear, but recent electrophysiological, neuroimaging, and clinical studies have yielded important data. Categorizing HHs by the degree of hypothalamic involvement has contributed to the accurate prediction of their prognosis and to improved treatment strategies. Rather than undergoing corticectomy, HH patients with medically intractable seizures are now treated with surgery that targets the HH per se, e.g. HH removal, disconnection from the hypothalamus, stereotactic irradiation, and radiofrequency lesioning. Although surgical intervention carries risks, total eradication or disconnection of the lesion leads to cessation or reduction of seizures and improves the cognitive and behavioral status of these patients. Precocious puberty in HH patients is safely controlled by long-acting gonadotropin-releasing hormone agonists. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities may lead to earlier intervention in young HH patients and prevent the decline in their cognitive abilities and quality of life. This review of hypothalamic hamartomas presents current classifications, pathophysiologies, and treatment modalities.

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“…A presença de hamartomas hipotalâmicos quase sempre leva a puberdade precoce 10 24 , revendo a literatura, descreveram as bases anatômicas segundo as quais os hamartomas manteriam conexões com o hipotálamo através de fibras nervosas, influenciando a sua função. Estas fibras "neurosecretoras" penetrando pela superfície da adeno-neurohipófise e pela pars tuberalis, bem como nos plexos dos vasos do sistema porta hipofisário, constituiriam a base morfológica para a ação dos hamartomas sobre a função reguladora na puberdade.…”

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Puberdade precoce causada por tumores intracranianos: relato de 4 casos

Brandt¹,

Carvalho²

1974

Arq. Neuro-Psiquiatr.

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A puberdade é considerada precoce quando o desenvolvimento sexual se inicia antes dos 9 anos de idade. Em 90% das meninas e em 50% dos meninos com puberdade precoce não é encontrada causa, sendo então denominada idiopática. Nestas, o mecanismo de interação hipófise-gônada é ativado precocemente, por motivos desconhecidos. A hipófise secreta gonadotrofinas que irão estimular o desenvolvimento e a maturação das gônadas. Estas secretam andrógenos e estrógenos em quantidades crescentes, logo atingindo os níveis normais para o adulto. Ovulação e menstruação aparecem na mulher e espermatogênese no homem.Tumores do ovário, testículo e supra-renal produzem puberdade precoce que se diferencia do tipo idiopático porque os hormônios sexuais em níveis adultos inibem a produção de gonadotrofinas hipofisárias. Conseqüentemente a ovulação e a espermatogênese não ocorrem. Entretanto, do ponto de vista laboratorial, nem sempre é possível distinguir a puberdade precoce idiopática da secundária a tumores das gônadas ou supra-renais. Este grupo compreende 85% dos casos em que a causa é conhecida.Lesões intracranianas com repercussão no eixo hipotálamo-hipofisário são responsáveis pela puberdade precoce em 15% dos casos de causa conhecida 1 . As características endocrinas são as mesmas da forma idiopática. Assim, antes de um caso de puberdade precoce ser diagnosticado como idiopático, lesões intracranianas deverão ser excluídas. Entre estas citam-se traumatismos, malformações, infecções, hidrocefalia e tumores intracranianos ". Dentre os últimos destacam-se o teratoma e o hamartoma. Segundo Jenkins 8 , pode ser demonstrado um tumor hipotalâmico em 8% dos casos de puberdade prepoce de causa hipotalâmica.

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Precocious Puberty Due to a Hypothalamic Hamartoma in a Patient Surviving to Late Middle Age

Cited by 11 publications

References 0 publications

Clinical and Surgical Aspects of Hypothalamic Hamartoma Associated with Precocious Puberty in a 15-Month-Old Boy

Clinical and Surgical Aspects of Hypothalamic Hamartoma Associated with Precocious Puberty in a 15-Month-Old Boy

Hypothalamic Hamartoma

Puberdade precoce causada por tumores intracranianos: relato de 4 casos

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