Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty

1998 ◽  
Vol 88 (2) ◽  
pp. 340-345 ◽  
Author(s):  
Laura Stewart ◽  
Paul Steinbok ◽  
Jorge Daaboul
Keyword(s):  
Precocious Puberty ◽  
Growth Velocity ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Total Resection ◽  
Content Type ◽  
Follow Up Care ◽  
Fine Print

✓ Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (> 7 cm/year), bone age remained advanced (> +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery. Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.

scholarly journals Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty

1997 ◽  
Vol 3 (6) ◽  
pp. E2
Author(s):  
Laura Stewart ◽  
Paul Steinbok ◽  
Jorge Daaboul
Keyword(s):  
Precocious Puberty ◽  
Growth Velocity ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Long Term Results ◽  
Total Resection ◽  
Hypothalamic Hamartomas ◽  
Follow Up Care

Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (gt 7 cm/year), bone age remained advanced (gt +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery. Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.

Anthropometric, metabolic, and reproductive outcomes of patients with central precocious puberty treated with leuprorelin acetate 3-month depot (11.25 mg)

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Carolina O. Ramos ◽  
Ana P M Canton ◽  
Carlos Eduardo Seraphim ◽  
Aline Guimarães Faria ◽  
Flavia Rezende Tinano ◽  
...  
Keyword(s):  
Precocious Puberty ◽  
Adult Height ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Overweight And Obesity ◽  
Leuprorelin Acetate ◽  
The Mean ◽  
Hormone Analogs

Abstract Objectives Longer-acting gonadotropin-releasing hormone analogs (GnRHa) have been widely used for central precocious puberty (CPP) treatment. However, the follow-up of patients after this treatment are still scarce. Our aim was to describe anthropometric, metabolic, and reproductive follow-up of CPP patients after treatment with leuprorelin acetate 3-month depot (11.25 mg). Methods Twenty-two female patients with idiopathic CPP were treated with leuprorelin acetate 3-month depot (11.25 mg). Their medical records were retrospectively evaluated regarding clinical, hormonal, and imaging aspects before, during, and after GnRHa treatment until adult height (AH). Results At the diagnosis of CPP, the mean chronological age (CA) was 8.2 ± 1.13 year, and mean bone age (BA) was 10.4 ± 1.4 year. Mean height SDS at the start and the end of GnRHa treatment was 1.6 ± 0.8 and 1.3 ± 0.9, respectively. The mean duration of GnRHa treatment was 2.8 ± 0.8 year. Mean predicted adult heights (PAH) at the start and the end of GnRH treatment was 153.2 ± 8.6 and 164.4 ± 7.3 cm, respectively (p<0.05). The mean AH was 163.2 ± 6.2 cm (mean SDS: 0.1 ± 1). All patients were within their target height (TH) range. There was a decrease in the percentage of overweight and obesity from the diagnosis until AH (39–19% p>0.05). At the AH, the insulin resistance and high LDL levels were identified in 3/17 patients (17.6%) and 2/21 patients (9.5%), respectively. The mean CA of menarche was 12.2 ± 0.5 years. At the AH, PCOS was diagnosed in one patient (4.8%). Conclusions Long-term anthropometric, metabolic, and reproductive follow-up of patients with CPP treated with longer-acting GnRHa revealed effectivity, safety, and favorable outcomes.

Treatment of colloid cysts of the third ventricle by stereotaxic microsurgical laser craniotomy

1989 ◽  
Vol 70 (4) ◽  
pp. 525-529 ◽  
Author(s):  
Chad D. Abernathey ◽  
Dudley H. Davis ◽  
Patrick J. Kelly
Keyword(s):  
Third Ventricle ◽  
Total Removal ◽  
Total Resection ◽  
Content Type ◽  
The Third ◽  
Cyst Aspiration ◽  
Colloid Cysts ◽  
Mass Lesions ◽  
Fine Print

✓ The therapeutic strategies employed in the management of anterior third-ventricular mass lesions remain controversial. Resection by conventional craniotomy, whether via a transcallosal or transcortical approach, carries well-known risks and limitations. Alternatively, in this region traditional stereotaxy has been relegated to use with biopsy only or cyst aspiration procedures. Combining aspects of both conventional and stereotaxic techniques has allowed total removal of 12 colloid cysts in six women and six men ranging in age from 25 to 71 years. No mortality and minimal morbidity have been associated with the procedures. There has been no evidence of recurrence in an average follow-up period of 19 months. By coupling the benefits of stereotaxic precision and localization to the microsurgical management of colloid cysts, several rewards have been realized: 1) only a limited cortical dissection is needed; 2) the hazards of callosal or forniceal injury can be avoided; 3) the lesion is easily localized regardless of ventricular size; 4) hemostasis can be readily achieved with bipolar cautery or defocused laser power; and 5) most importantly, a total resection is possible with little risk to the patient. Stereotaxic microsurgical laser craniotomy provides a new option for the management of colloid cysts and other anterior third-ventricular lesions.

Scoliosis and hydrocephalus in myelocele patients

1979 ◽  
Vol 50 (2) ◽  
pp. 174-178 ◽  
Author(s):  
Peter Hall ◽  
Richard Lindseth ◽  
Robert Campbell ◽  
John E. Kalsbeck ◽  
Alonso Desousa
Keyword(s):  
Spinal Fusion ◽  
Early Recognition ◽  
Common Cause ◽  
Content Type ◽  
Etiological Role ◽  
Ventricular Shunting ◽  
Fine Print

✓ Developmental scoliosis is a common cause of increasing disability and deformity in long-term myelocele survivors, and is believed to result from a paralytic collapsing spine. The possible etiological role of compensated hydrocephalus and hydromyelia was assessed by determining the effect of ventricular shunting on 11 myelocele patients with developmental scoliosis. After successful shunting, one patient with a 47° curve continued to deteriorate. Three cases with curves greater than 60° were stabilized for short periods, but eventually required spinal fusion. Seven cases with curves less than 55° were improved from a mean scoliosis of 29° to 13° during a 20-month follow-up period. Several patients had pre-existing shunts that were found to be non-functional on shuntogram. These findings suggest that the spinal complications of hydrocephalus may be more common than previously recognized in myelocele patients and that advanced developmental scoliosis may be avoided by early recognition and ventricular shunting.

Postoperative surveillance imaging in children with cerebellar astrocytomas

1996 ◽  
Vol 84 (5) ◽  
pp. 721-725 ◽  
Author(s):  
Leslie N. Sutton ◽  
Avital Cnaan ◽  
Laura Klatt ◽  
H. Zhao ◽  
Robert Zimmerman ◽  
...  
Keyword(s):  
Predictive Value ◽  
Tumor Recurrence ◽  
Residual Tumor ◽  
Subtotal Resection ◽  
Surveillance Program ◽  
Content Type ◽  
Surveillance Imaging ◽  
Follow Up Care ◽  
Fine Print

✓ The standard follow-up care for children with cerebellar astrocytomas includes regular surveillance imaging of the brain with computerized tomography or magnetic resonance. The purpose of surveillance imaging is to detect asymptomatic tumor recurrence at an early stage and permit safer reoperation. The authors evaluated the effectiveness of an intensive surveillance program for cerebellar astrocytoma and tested different models of surveillance frequency and duration to arrive at a specific recommended program. Review of the records of 93 children with typical cerebellar astrocytomas who received follow-up care between 1975 and 1993 was performed. Immediate postoperative and surveillance images were classified as showing definite, equivocal, or no tumor based on the radiology report at the time the image was obtained. Various surveillance models were then tested for their predictive value for detecting tumor recurrence. Seventeen (18%) of the 93 children had tumor recurrence or progression. Eleven of these tumors were asymptomatic and detected only by surveillance image. Tumor recurred in only one patient with a total resection, whereas tumor progression occurred in five of 21 patients with equivocal postoperative images and in 11 of 14 patients with residual tumor. A model in which patients with possible or definite residual tumor after surgery undergo surveillance at 12, 18, 30, 42, and 66 months, and later have one additional image, yielded optimum predictive value for recurrence and/or progression with the fewest images. Patients with tumor recurrence were satisfactorily treated, and only one patient died. Children with totally resected cerebellar astrocytomas do not appear to benefit from routine surveillance, because the likelihood of recurrence is small. Surveillance is of benefit in those who may have subtotal resection based on the immediate postoperative imaging.

Gamma knife radiosurgery for hypothalamic hamartomas in patients with medically intractable epilepsy and precocious puberty

2000 ◽  
Vol 92 (4) ◽  
pp. 726-731 ◽  
Author(s):  
Frank Unger ◽  
Oskar Schröttner ◽  
Klaus Haselsberger ◽  
Eva Körner ◽  
Robert Ploier ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Gamma Knife ◽  
Precocious Puberty ◽  
Seizure Control ◽  
Gamma Knife Radiosurgery ◽  
Content Type ◽  
Hypothalamic Hamartomas ◽  
Gelastic Epilepsy ◽  
Fine Print

✓ Hamartoma of the hypothalamus represents a well-known but rare cause of central precocious puberty and gelastic epilepsy. Due to the delicate site in which the tumor is located, surgery is often unsuccessful and associated with considerable risks. In the two cases presented, gamma knife radiosurgery was applied as a safe and noninvasive alternative to obtain seizure control.Two patients, a 13-year-old boy and a 6-year-old girl, presented with medically intractable gelastic epilepsy and increasing episodes of secondary generalized seizures. Abnormal behavior and precocious puberty were also evident. Magnetic resonance (MR) imaging revealed hypothalamic hamartomas measuring 13 and 11 mm, respectively. After general anesthesia had been induced in the patients, radiosurgical treatment was performed with margin doses of 12 Gy to 90% and 60% of isodose areas, covering volumes of 700 and 500 mm3, respectively.After follow-up periods of 54 months in the boy and 36 months in the girl, progressive decrease in both seizure frequency and intensity was noted (Engel outcome scores IIa and IIIa, respectively). Both patients are currently able to attend public school. Follow-up MR imaging has not revealed significant changes in the sizes of the lesions.Gamma knife radiosurgery can be an effective and safe treatment modality for achieving good seizure control in patients with hypothalamic hamartomas.

Thoracoscopic resection of thoracic neurogenic tumors

2002 ◽  
Vol 96 (3) ◽  
pp. 304-308 ◽  
Author(s):  
Patrick P. Han ◽  
Curtis A. Dickman
Keyword(s):  
Gross Total Resection ◽  
Total Resection ◽  
Thoracoscopic Resection ◽  
Content Type ◽  
Neurogenic Tumors ◽  
Normal Tissues ◽  
Intercostal Neuralgia ◽  
Intraspinal Extension ◽  
Fine Print

Object. The authors describe the treatment and results of thoracoscopic resection performed in patients with neurogenic tumors. Methods. Seven patients with large intrathoracic paraspinal neurogenic tumors underwent a thoracoscopic procedure to achieve gross-total resection. All tumors were entirely intrathoracic and treated thoracoscopically except in one patient whose tumor had an intraspinal extension. Gross-total resection was achieved in all cases. Postoperatively, one patient developed Horner syndrome. The only other complication, transient intercostal neuralgia, resolved in all patients. Clinical examination and magnetic resonance imaging follow-up examination in all patients demonstrated no evidence of recurrent disease (mean follow up 12.5 months). Conclusions. Endoscopic transthoracic approaches can reduce approach-related soft-tissue morbidity and facilitate a patient's recovery by preserving the normal tissues of the chest wall, by avoiding rib retraction and muscle transection, and by reducing postoperative pain.

scholarly journals Central Precocious Puberty in Boys and Girls: Similarities and Differences

Sexes ◽  
2021 ◽  
Vol 2 (1) ◽  
pp. 119-131
Author(s):  
Cristina Mucaria ◽  
Nina Tyutyusheva ◽  
Giampiero I. Baroncelli ◽  
Diego Peroni ◽  
Silvano Bertelloni
Keyword(s):  
Precocious Puberty ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Gnrh Analog ◽  
Secondary Sexual Characteristics ◽  
Sexual Characteristics ◽  
The Central Nervous System

Central precocious puberty (CPP) is due to the premature activation of the hypothalamic–pituitary–gonadal axis, which is responsible for the appearance of secondary sexual characteristics. It occurs before the age of 8 and 9 in girls and boys, respectively. CPP shows higher incidence in females than in males. Causes of CPP are similar in both sexes, but the idiopathic form is more frequent in girls, while organic forms are more frequent in males. Recent studies demonstrated a role of some genetic variants in the pathogenesis of CPP. The diagnostic evaluation based on accurate physical examination, assessment of the pituitary–gonadal axis, pelvic sonography in girls, and determination of bone age. Magnetic resonance of the central nervous system should be done in all boys and selected girls. Since the 1980s, pharmacologic treatment involves the use of gonadotropin-releasing hormone (GnRH) analogs. These drugs are characterized by few side effects and long-term safety. Many data are available on the outcome of GnRH analog treated female patients, while poor data are reported in boys. Adult height is improved in both sexes.

scholarly journals Gonadotropin Suppression for 7 Years after a Single Histrelin Implant for Precocious Puberty

2022 ◽  
Author(s):  
Douglas Villalta ◽  
Jose Bernardo Quintos
Keyword(s):  
Standard Deviation ◽  
Precocious Puberty ◽  
Final Height ◽  
Central Precocious Puberty ◽  
Bone Age ◽  
Genetic Potential ◽  
Parental Height ◽  
Hormone Analogs ◽  
Lost To Follow Up

Abstract Gonadotropin releasing hormone analogs (GnRHas) are an effective treatment to address the compromise in height potential seen in patients with central precocious puberty. There is no evidence in the literature of a single GnRHa used for longer than 2 years before being removed or replaced. We describe a patient who was on continuous gonadotropin suppression for 7 years and despite this, achieved a height potential within one standard deviation of mid-parental height. A boy aged 10 years and 3 months presented to endocrine clinic with signs of precocious puberty and advanced bone age. Initial labs showed random LH 9.4 mIU/mL, FSH 16.3 mIU/mL, DHEAS 127 mcg/dl, and testosterone 628 ng/dL. He was initially started on Lupron injections before transitioning to a Histrelin implant. Follow-up laboratory results 5 months post-suppression showed pre-pubertal random LH 0.2 mIU/mL, FSH 0.1 mIU/mL, and testosterone 5 ng/dL. The patient was lost to follow-up and returned 5 years later presenting with gynecomastia and delayed bone age. He had continuous gonadotropin suppression with random LH 0.10 mIU/mL, FSH 0.16 mIU/mL, and testosterone 8 ng/dL. The Histrelin implant was removed and 4 months after removal labs showed random pubertal hormone levels with LH 5.6 mIU/mL, FSH 4.3 mIU/mL, and testosterone 506 ng/dl. The patient’s mid-parental height was 175.3 cm and the patient’s near final height was 170.6 cm which is within one standard deviation of his genetic potential. Further studies are needed to explore continuous gonadotropin hormone suppression with a single Histrelin implant beyond 2 years.

Primary cerebellar glioblastomas multiforme in children

1999 ◽  
Vol 90 (3) ◽  
pp. 546-550 ◽  
Author(s):  
Abhaya V. Kulkarni ◽  
Laurence E. Becker ◽  
Venita Jay ◽  
Derek C. Armstrong ◽  
James M. Drake
Keyword(s):  
Adjuvant Therapy ◽  
Tumor Resection ◽  
Immunohistochemical Analysis ◽  
Tumor Spread ◽  
Content Type ◽  
Craniospinal Radiation ◽  
Sick Children ◽  
Fine Print

✓ Primary cerebellar glioblastomas multiforme are exceedingly rare in children. The authors therefore retrospectively characterized the clinical behavior and pathological features of these tumors. A review of the database at the Hospital for Sick Children, Toronto, Canada revealed four patients with cerebellar tumors that displayed significant pleomorphism, hypercellularity, mitoses, and necrosis with pseudopalisading. The authors performed a detailed clinical, radiological, histological, and immunohistochemical analysis of the tumors in these four children (three boys and one girl; average age at presentation 7 years; range 21 months–15 years). Magnetic resonance imaging and computerized tomography most commonly revealed a large lesion with minimal edema, inhomogeneous contrast enhancement, and a discrete border. Tumor resection was subtotal in one patient and gross total in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was positive in varying degrees. Initial adjuvant therapy consisted of local radiation only (one patient), chemotherapy only (one patient), and radiation and chemotherapy (one patient). One patient received no adjuvant therapy. Tumor recurrence was documented in all patients: two local recurrences (at 3.5 and 7 months), one spinal recurrence (at 14 months), and one local recurrence with ventricular and spinal spread (at 8 months). Ultimately, three of the four patients developed leptomeningeal tumor spread. Patient follow up ranged from 8 to 17 months (mean 12.5 months). Three patients were dead at last follow up with a mean survival of 15 months.The prognosis for patients with cerebellar glioblastomas is extremely poor, and the tumor has a tendency for cerebrospinal fluid dissemination. The optimal management of patients harboring of these difficult-to-treat tumors, including the role of craniospinal radiation and chemotherapy, has not yet been achieved.

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