Pubertas praecox due to hypothalamic hamartoma: report of two cases surviving surgical removal of the tumour.

Northfield, D. W. C.; Russell, Duncan S.

doi:10.1136/jnnp.30.2.166

Cited by 91 publications

(34 citation statements)

References 7 publications

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“…Neurosurgeons were generally reluctant to remove a lesion from the hypothalamus. The first reports of surgery for HH were those of Northfield and Russell, 25 who described 2 patients in 1967 who underwent subfrontal approaches for precocious puberty. The first lesion was probably pedunculated.…”

Section: The Development Of Surgery For Hhmentioning

confidence: 99%

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

FOC

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The prognosis for patients with hypothalamic hamartoma has improved dramatically over the last 20 years, for 3 main reasons. First, because of improved understanding of the anatomy and pathophysiology of these varied lesions. Second, due to advances in brain imaging and refinements in microsurgery, including the anterior transcallosal interforniceal approach, endoscopic, and skull-base approaches. And third, because of increasing experience with stereotactic radiosurgery, interstitial radiotherapy, and radiofrequency lesioning. Patients with hypothalamic hamartoma should be managed in comprehensive epilepsy centers where the treatments are individualized and concentrated in the hands of surgeons who can perform the full range of surgery, including approaches to the third ventricle. Total seizure-freedom rates of 52% to 66% have been achieved with surgery.

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Section: The Development Of Surgery For Hhmentioning

confidence: 99%

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Rosenfeld¹

2011

FOC

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“…HHs are very rarely located in the interpeduncular or prepontine cisterns without direct connection to the hypothalamus. 6,53,54) HHs primarily consist of mature neurons intermingled with glial cells. 3,46) The neurons resemble those in the normal tuber cinereum, 75) although large dysplastic neurons are occasionally found in HH patients with seizures.…”

Section: Pathologymentioning

confidence: 99%

“…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. Treatment of seizures (Table 3) Antiepileptic drugs: Gelastic seizures in HH patients are medically intractable 4,9) and very few are controlled by antiepileptic drugs alone.…”

Section: Cppmentioning

confidence: 99%

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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“…Resection of HHs for the treatment of precocious puberty was initially described in 1967 by Northfield and Russell, 23 and since then there have been many cases reported in which removal of the hamartoma has resulted in successful control of this condition. 3,4,7,14,19,[21][22][23]26,28,29 Surgical approaches to remove HHs include the transcallosal interhemispheric interforniceal approach, endoscopic approach through the foramen of Monro, or skull base approaches.…”

Section: 1330mentioning

confidence: 99%

“…3,4,7,14,19,[21][22][23]26,28,29 Surgical approaches to remove HHs include the transcallosal interhemispheric interforniceal approach, endoscopic approach through the foramen of Monro, or skull base approaches. The surgical route is selected based on the patient's age and condition, on the anatomy of the HH, and the surgeon's expertise.…”

Section: 1330mentioning

confidence: 99%

Successful treatment of hyperphagia by resection of a hypothalamic hamartoma

Esquenazi

Sandberg

Rekate³

2013

PED

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Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a 7-year-old girl who was diagnosed with an HH due to precocious puberty and was treated medically with a gonadotropin-releasing hormone analog for 3 years. Despite normalization of her plasma levels of luteinizing hormone, follicle-stimulating hormone, and estradiol and arrest of her precocious puberty, the patient developed progressive weight gain associated with extreme hyperphagia and morbid obesity by the age of 10 years. Her compulsive eating patterns were refractory to counseling and other interventions attempted by her parents and physicians. After resection of the HH, her hyperphagia resolved and her weight stabilized. To the authors' knowledge, this is the first report describing resection of an HH for the purpose of treating hyperphagia and obesity.

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Pubertas praecox due to hypothalamic hamartoma: report of two cases surviving surgical removal of the tumour.

Cited by 91 publications

References 7 publications

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

The evolution of treatment for hypothalamic hamartoma: a personal odyssey

Hypothalamic Hamartoma

Successful treatment of hyperphagia by resection of a hypothalamic hamartoma

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