An 11-month-old girl with central precocious puberty caused by hypothalamic hamartoma
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Abstract:
Central precocious puberty (CPP) is caused by premature activation of the hypothalamic-gonadal axis, and must be treated adequately. In particular, CPP that occurs at a relatively young age or in boys is likely to be caused by an organic lesion. Hypothalamic hamartoma (HH) is the most common organic cause of CPP. The present case report describes an 11-month-old female infant who presented with vaginal bleeding and rapidly progressive secondary sex characteristics from the age of 6 months. She was diagnosed with CPP following the detection of HH via magnetic resonance imaging. The infant girl was successfully treated with gonadotropin-releasing hormone agonist. After 6 months, her breast had regressed and clinical and radiological follow-up demonstrated stable findings with no evidence of tumor growth or secondary sexual characteristics until the fourth year after the initiation of treatment. This patient is the one of the youngest infants presenting with CPP and HH in Korea; treatment was successful over a relatively long follow-up period. Keywords: Hypothalamic hamartoma, Precocious puberty, MenstruationKeywords:
Girl
Central precocious puberty
Hamartoma
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Central precocious puberty (CPP) presenting at a very young age is likely to have an underlying pathology. One of the pathologies is hypothalamic hamartoma (HH), a non-neoplastic tumour-like lesion located at the floor of the third ventricle, near the tuber cinereum. Two young children with CPP due to HH without gelastic seizures or mental retardation were successfully managed and described in this report.
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We described the clinical presentation,image changes,pathologic manifestation,and hormone data before and after surgical excision in a 2 years-old boy with typical central precocious puberty (CPP) resulted from hypothalamic hamartoma(HH).With literature review,the epidemiologic and clinical features,hormone profile,diagnosis,and therapeutic options of CPP induced by HH were discussed to update the general understandings for the management of this disease.
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Hypothalamic disease; Hamartoma; Central precocious puberty
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Hypothalamic disease
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Abstract Two histologically confirmed hypothalamic hamartomas, one in a 7-year-old boy and another in a 10-year-old boy, are reported. One patient had precocious puberty, epileptic laughter, and abnormal behavior; the other had cerebral seizures. Partial removal of the tumors had no effect on precocious puberty; however, behavior improved in the first patient, and seizure control improved in the second patient.
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Central precocious puberty
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Hamartoma
Gelastic seizure
Hypothalamic disease
Central precocious puberty
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Hypothalamic hamartomas are rare tumours of particular interest because of their unusual symptoms. Three cases of hypothalamic hamartomas are reported in children, who presented with precocious puberty and gelastic seizures.
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To investigate the diagnosis and treatment of hypothalamic hamartoma in children.Eighteen cases of hypothalamic hamartoma in children, including 9 boys and 9 girls, were examined with CT and MRI. Eleven cases underwent operation. Post-operation follow-up was conducted for 0.5 approximately 6 years.The main clinical features of hypothalamic hamartoma were precocious puberty and gelastic seizures, some combine with other kinds of seizures, mental retardation or congenital abnormalities. The effective rate of surgery was 91%; patients with simple precocious puberty were cured.Microsurgery is the first choice of treatment for hypothalamic hamartoma.
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Objective To investigate the diagnosis and microsurgical treatment of hypothalamic hamartoma in children.Methods A retrospective analysis was conducted on five cases with hypothalamic hamartoma in children from January 1998 to December 2006.Five children(two boys and three girls)were treated for precocious puberty and gelastic seizures secondary to hypothalamic hamartoma.The age ranged from 20 months to 9 years with a mean of 44.4 months.Two cases mainly displayed precocious puberry,1 with gelastic seizures and the other 2 cases with both of precocious puberty and gelastic secures.The sex hormone level of four cases was about 5 to several dozen times more than nolTnal before operation.Magnetic resonance imaging clearly demonstrated an isointense mass in the interpeduncular cistern(5 cases)and suprasellar cistem(2 cases)ranged from 13.9 to 21.0mm in diameter without enhanced pictures after injected Gd-DTPA.The hamartomas were totally removed via a right frontotemporal approach under microscope.Results All hypothalamic hamartomas were completely resected under microscope and confirmed by pathological examination.The symptoms and signs of precocious puberty completely regressed postoperatively,and sex hormone level descended to prepubertal level in all patients.The symptoms of gelastic seizures completely disappeared in two patients and obviously reduced from ten times per day before operation to one time per fortnight in another one patient.There was no any complication during 1 year follow-up in all patients.Conclusions Microsurgery can be deserved the first choice of treatment for hypothalamic hamartoma in children.
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Hamartoma; Epilepsy; Microsurgery
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Hypothalamic hamartoma is a rare congenital nonneoplastic heterotopia consisting of neurons, glial cells and fiber bundles. Clinically, most patients with hypothalamic hamartomas present with precocious puberty and/or gelastic epilepsy. We report an interesting case of hypothalamic hamartoma causing precocious puberty in a young male. The lesion was excised totally through frontotemporal craniotomy and transSylvian approach without any added morbidity.
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