Two Cases of Precocious Puberty Associated with Hypothalamic Hamartoma

Object.Hypothalamic hamartoma is a nonneoplastic malformative mass of neurons and glia in the region of the hypothalamus. Because of its location, open surgery is associated with high morbidity and mortality rates. Gamma knife surgery (GKS) may be an efficient and safe treatment approach, which produces little morbidity. The authors describe the results of GKS in three patients with hypothalamic hamartomas.Methods.All patients were male, aged 3, 12, and 15 years. The lesions were classified according to the Valdueza scale: one was Type IIb and two were Type IIa. The patients presented with gelastic seizures (15–20 per day), generalized epilepsy, behavioral abnormalities, and alterations of the sleep cycle. Precocious puberty was present in one patient. The Type IIb tumor had a volume of 1.8 cm3, and the Type IIa tumors were 597 mm3and 530.1 mm3. The lesions received 12.5 Gy, 14 Gy, and 15 Gy, respectively, to the 50% isodose line. The patients were followed for 30 to 50 months. After 3 months, all patients showed improvement of their sleep, behavior, and epilepsy. At the present time, these patients are receiving low-dose antiepileptic agents and have achieved adequate social development and school integration.Conclusions.Gamma knife surgery appears to be a good, safe, and effective option for the treatment of selected hypothalamic hamartomas. No morbidity or mortality was associated with these three cases.

Download Full-text

Hypothalamic hamartoma simulating a suprasellar arachnoid cyst: resolution of precocious puberty following microsurgical lesion resection

Journal of Neurosurgery Pediatrics ◽

10.3171/2014.4.peds13371 ◽

2014 ◽

Vol 14 (1) ◽

pp. 101-107 ◽

Cited By ~ 1

Author(s):

Sunil Manjila ◽

Timothy W. Vogel ◽

Yunwei Chen ◽

Mark S. Rodgers ◽

Alan R. Cohen

Keyword(s):

Arachnoid Cyst ◽

Precocious Puberty ◽

Central Precocious Puberty ◽

Hypothalamic Hamartoma ◽

Suprasellar Region ◽

Cystic Changes ◽

Long Term Follow Up ◽

Suprasellar Arachnoid Cyst

Hypothalamic hamartomas (HHs) are rare developmental lesions arising from the inferior hypothalamus that may cause gelastic seizures and central precocious puberty. Cystic changes in HHs are rare, usually occurring in giant lesions. The authors describe an unusual case of cystic HH masquerading as a suprasellar arachnoid cyst in an 18-month-old girl presenting with precocious puberty. Microsurgical removal of the lesion led to complete resolution of the precocious puberty on long-term follow-up. This case is the first reported HH with pathological demonstration of corticotropin-releasing hormone immunostaining in the solid tumor and glial cells in the cyst wall of the lesion. The clinical and radiological characteristics of HHs are reviewed, along with the unique surgical strategies used to manage cystic lesions in the suprasellar region.

Download Full-text

The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma

Journal of Neurosurgery ◽

10.3171/jns.1999.91.2.0212 ◽

1999 ◽

Vol 91 (2) ◽

pp. 212-220 ◽

Cited By ~ 149

Author(s):

Kazunori Arita ◽

Fusao Ikawa ◽

Kaoru Kurisu ◽

Masayuki Sumida ◽

Kunyu Harada ◽

...

Keyword(s):

Mental Retardation ◽

Magnetic Resonance ◽

Precocious Puberty ◽

Third Ventricle ◽

Clinical Manifestations ◽

Hypothalamic Hamartoma ◽

Content Type ◽

The Third ◽

The Relationship ◽

Fine Print

Object. Hypothalamic hamartoma is generally diagnosed based on its magnetic resonance (MR) imaging characteristics and the patient's clinical symptoms, but the relationship between the neuroradiological findings and clinical presentation has never been fully investigated. In this retrospective study the authors sought to determine this relationship.Methods. The authors classified 11 cases of hypothalamic hamartoma into two categories based on the MR findings. Seven cases were the “parahypothalamic type,” in which the hamartoma is only attached to the floor of the third ventricle or suspended from the floor by a peduncle. Four cases were the “intrahypothalamic type,” in which the hamartoma involved or was enveloped by the hypothalamus and the tumor distorted the third ventricle.Six patients with the parahypothalamic type exhibited precocious puberty, which was controlled by a luteinizing hormone—releasing hormone analog, and one patient was asymptomatic. No seizures or mental retardation were observed in this group. All patients with the intrahypothalamic type had medically intractable seizures, and precocious puberty was seen in one. Severe mental retardation and behavioral disorders including aggressiveness were seen in two patients. The seizures were controlled in only one patient, in whom stereotactically targeted irradiation of the lesion was performed.This topology/symptom relationship was reconfirmed in a review of 61 reported cases of hamartoma, in which the MR findings were clearly described. The parahypothalamic type is generally associated with precocious puberty but is unaccompanied by seizures or developmental delay, whereas the intrahypothalamic type is generally associated with seizures. Two thirds of patients with the latter experience developmental delays, and half also exhibit precocious puberty.Conclusions. Classification of hypothalamic hamartomas into these two categories based on MR findings resulted in a clear correlation between symptoms and the subsequent clinical course.

Download Full-text

Giant solid-cystic hypothalamic hamartoma

Neurosurgical FOCUS ◽

10.3171/2011.1.focus10240 ◽

2011 ◽

Vol 30 (2) ◽

pp. E7 ◽

Cited By ~ 9

Author(s):

Christian Dorfer ◽

Gregor Kasprian ◽

Angelika Mühlebner ◽

Thomas Czech

Keyword(s):

Precocious Puberty ◽

Hypothalamic Hamartoma ◽

Gelastic Seizures ◽

Classification Schemes ◽

Hypothalamic Hamartomas ◽

Cystic Hamartoma

Hypothalamic hamartomas are rare lesions for which different classification schemes have been proposed. The authors report on an exceptionally large solid-cystic hamartoma that led to hydrocephalus, precocious puberty, and intractable gelastic seizures. They discuss potential mechanisms of the development of hypothalamic hamartomas.

Download Full-text

Pathogenesis of Precocious Puberty in Hypothalamic Hamartoma

Hormone Research in Paediatrics ◽

10.1159/000058097 ◽

2002 ◽

Vol 57 (2) ◽

pp. 31-34 ◽

Cited By ~ 15

Author(s):

Heike Jung ◽

Sergio R. Ojeda

Keyword(s):

Precocious Puberty ◽

Hypothalamic Hamartoma

Download Full-text