Giant solid-cystic hypothalamic hamartoma

2011 ◽  
Vol 30 (2) ◽  
pp. E7 ◽  
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.

Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: experience in Mexico

2005 ◽  
Vol 102 ◽  
pp. 53-55 ◽  
Author(s):  
Marco A. Barajas ◽  
Maria G. Ramírez-Guzmán ◽  
Carlos Rodríguez-Vázquez ◽  
Vinicio Toledo-Buenrostro ◽  
Abel Cuevas-Solórzano ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Precocious Puberty ◽  
Intractable Epilepsy ◽  
Gamma Knife Surgery ◽  
Sleep Cycle ◽  
Hypothalamic Hamartoma ◽  
High Morbidity ◽  
Content Type ◽  
Hypothalamic Hamartomas ◽  
Fine Print

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.

Gamma knife surgery for hypothalamic hamartomas accompanied by medically intractable epilepsy and precocious puberty: experience in Mexico

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 53-55 ◽  
Author(s):  
Marco A. Barajas ◽  
Maria G. Ramírez-Guzmán ◽  
Carlos Rodríguez-Vázquez ◽  
Vinicio Toledo-Buenrostro ◽  
Abel Cuevas-Solórzano ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Precocious Puberty ◽  
Intractable Epilepsy ◽  
Gamma Knife Surgery ◽  
Sleep Cycle ◽  
Hypothalamic Hamartoma ◽  
High Morbidity ◽  
Content Type ◽  
Hypothalamic Hamartomas ◽  
Fine Print

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 mm3 and 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.

Neurosurgical treatment of hypothalamic hamartomas causing precocious puberty

1993 ◽  
Vol 78 (1) ◽  
pp. 77-82 ◽  
Author(s):  
A. Leland Albright ◽  
Peter A. Lee
Keyword(s):  
Precocious Puberty ◽  
Pubertal Development ◽  
Hypothalamic Hamartoma ◽  
Eye Muscle ◽  
Content Type ◽  
Inferior Surface ◽  
Patients Âgés ◽  
Hypothalamic Hamartomas ◽  
Neurosurgical Treatment ◽  
Eye Muscle Surgery

✓ Five children, three girls and two boys, were treated for precocious puberty secondary to hypothalamic hamartoma by resection of the hamartoma. The patients' ages at onset of pubertal development ranged from 6 to 19 months. The hamartomas ranged in size from 6 to 10 mm; four were pedunculated, one was sessile, and all were located below the tuber cinereum. The hamartomas were excised via a right subtemporal approach, with transection at the inferior surface of the hypothalamus; two were adherent posteriorly to the basilar artery and brain stem, and the adhesions were divided. Postoperatively, three children exhibited a transient oculomotor paresis and one other child required eye-muscle surgery. The symptoms and signs of precocious puberty completely regressed postoperatively in all patients. Preoperative hormone assays of testosterone, luteinizing hormone, and follicle-stimulating hormone were within the pubertal range in all five children; postoperative assays fell to prepubertal levels. The children have been followed for 0.5 to 10.5 years (mean 5.0 years) postoperatively, without evidence of recurrence of precocious puberty. One child has begun spontaneous puberty at a normal age. It is concluded that complete resection of hypothalamic hamartomas causing precocious puberty is curative.

scholarly journals Association of Morphological Characteristics with Precocious Puberty and/or Gelastic Seizures in Hypothalamic Hamartoma

2003 ◽  
Vol 88 (10) ◽  
pp. 4590-4595 ◽  
Author(s):  
H. Jung ◽  
E. Neumaier Probst ◽  
B. P. Hauffa ◽  
C.-J. Partsch ◽  
O. Dammann
Keyword(s):  
Precocious Puberty ◽  
Clinical Symptoms ◽  
Third Ventricle ◽  
Morphological Characteristics ◽  
Central Precocious Puberty ◽  
Male Gender ◽  
Hypothalamic Hamartoma ◽  
Sexually Dimorphic ◽  
Gelastic Seizures ◽  
The Third

Abstract The pathogenesis of central precocious puberty (PP) and/or gelastic seizures due to a hypothalamic hamartoma (HH) is still under debate. We evaluated the association of clinical symptoms with morphology and localization of the HH in 34 patients. The majority (86.4%) of HHs in patients with isolated PP (n = 22; 68.2% females) revealed a parahypothalamic position without affecting the third ventricle (91%). Half of them were pedunculated, and 40.9% showed a diameter less than 10 mm. In contrast, 11 of 12 patients with seizures, eight of whom were male, presented with a sessile intrahypothalamic hamartoma, 10 of which distorted the third ventricle. Logistic regression analysis revealed an increased relative risk (RR) for epilepsy in males (RR, 4.3; 95% confidence interval, 0.96–19). However, combination of the risk factor gender with intrahypothalamic position (RR, 19; 1.3–285) and distortion of the third ventricle (RR, 10; 0.6–164) reduced the risk associated with male gender to 1.1. The position of a HH and involvement of the third ventricle are likely to be more predictive for clinical characteristics than size and shape. Male gender was associated with an intrahypothalamic HH and epilepsy, suggesting a sexually dimorphic developmental pattern of this heterotopic mass.

Hypothalamic Hamartoma: Report of Two Cases

Neurosurgery ◽  
1985 ◽  
Vol 16 (2) ◽  
pp. 198-206 ◽  
Author(s):  
Masaharu Sato ◽  
Yukitaka Ushio ◽  
Norio Arita ◽  
Heitaro Mogami
Keyword(s):  
Precocious Puberty ◽  
Seizure Control ◽  
The Other ◽  
Abnormal Behavior ◽  
Hypothalamic Hamartoma ◽  
Partial Removal ◽  
Hypothalamic Hamartomas

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.

Gelastic seizures, precocious puberty, and hypothalamic hamartoma

Neurology ◽  
1986 ◽  
Vol 36 (3) ◽  
pp. 443-443 ◽  
Author(s):  
P. Curatolo ◽  
R. Cusmai
Keyword(s):  
Precocious Puberty ◽  
Hypothalamic Hamartoma ◽  
Gelastic Seizures

Stereotaxy for Hypothalamic Hamartoma with Intractable Gelastic Seizures: Technical Case Report

Neurosurgery ◽  
1999 ◽  
Vol 44 (6) ◽  
pp. 1347-1350 ◽  
Author(s):  
Masafumi Fukuda ◽  
Shigeki Kameyama ◽  
Manabu Wachi ◽  
Ryuichi Tanaka
Keyword(s):  
Case Report ◽  
Hypothalamic Hamartoma ◽  
Gelastic Seizures
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