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PubMed1995

Giant hypothalamic hamartoma: an unusual neonatal tumor.

A case of neonatal manifestation of giant hypothalamic hamartoma is reported. It is suggested that hypothalamic hamartoma should be included in the list of neonatal intracerebral tumors. Magnetic resonance imaging appearance similar to that of normal gray matter on T1-weighted images and slightly hyperintense on T2-weighted images, without enhancement after gadolinium injection, is suggestive of the diagnosis. Hypothalamic hamartomas are congenital malformations, consisting of disorganized mature neuronal elements in proportions similar to that of normal tissue [1]. They are clinically evidenced in infants ranging from 1 to 7 years of age [1-5]. This report describes a histologically proved giant hypothalamic hamartoma diagnosed in the neonatal period. Magnetic resonance imaging (MRI) is helpful to distinguish this congenital non-evolutive malformation from more aggressive neonatal tumors.

Female↗
PubMed1980

Clinical and Endocrinological findings in ectopic pinealoma and spongioblastoma of the hypothalamus.

Cystic spongioblastoma and ectopic pinealoma occurring simultaneously were found in a 16-year-old male patient, and produced destruction of the hypothalamus. The clinical course extended for over four years. The clinical picture was characterized by a defect of osmo- and thermoregulation and by defective function of diencephalic nuclei and the sympathetic nervous system. The releasing factors for ACTH, TSH, LH, and FSH were lacking and produced corresponding disturbances of pituitary function. Because of the lack of hypothalamic inhibiting factors the prolactin level was increased, and the HGH level was stimulated by arginine loading and inhibited in the glucose test. The intact neurones in the ventromedial nucleus of the hypothalamus could be seen on microscopical examination. Clinical and endrocrinological findings were more suggestive of the diagnosis than the radiological ones. Computerized tomography showed multiple "tumour" localizations without any displacement signs. The occurrence of ectopic pinealoma and spongioblastoma in the same case would suggest, from the pathological point of view, a common dysontogenetic origin developing from the local elements of the nervous tissue.

Adolescent↗
PubMedDate not supplied

Hypothalamic hamartomas and precocious puberty.

Ten children, five boys and five girls with true precocious puberty at an early age were found to have hypothalamic hamartomas on brain imaging. Very early onset of puberty, varying from a few weeks to three years of age, and rapid progression were characteristic. Accelerated growth velocity and markedly advanced bone age were evident in all. Gonadotropin and gonadal hormone levels were elevated above the prepubertal range. Six children had associated developmental delay or hyperactivity.

Child, Preschool↗
PubMed1990

Spontaneous regression of anterior pituitary deficits in a woman harboring a persistent hypothalamic mass.

Sellar or parasellar lesions can cause anterior pituitary dysfunction either by direct damage to the anterior pituitary gland or by compression of the pituitary stalk and mediobasal hypothalamus which contain the hypophysiotropic hormones. Without treatment, the pituitary deficits in such cases are not likely to improve. We describe a case in which deficits in anterior pituitary hormones spontaneously remitted in a woman who had a persistent hypothalamic lesion that was not amenable to surgery or radiotherapy. The factors that may predict spontaneous recovery of anterior pituitary function in such cases are discussed.

Adult↗
PubMed1995

Diabetes insipidus for five years preceding the diagnosis of hypothalamic Langerhans cell histiocytosis.

We report a case of an adult male with Langerhans cell histiocytosis (LCH), in which the hypothalamic involvement went undetected by radiology and who was diagnosed as having central diabetes insipidus for 5 years before the skin lesions and the hypothalamic mass became evident on a CT scan. The skin lesions spontaneously disappeared but relapsed 12 months later. The hypothalamic mass disappeared six months after low dose radiotherapy with persistence of diabetes insipidus and loss of thirst sensation. We did not observe relapse of the hypothalamic mass within the five years of post-radiotherapy follow-up. Despite the fact that patients with LCH may experience spontaneous remissions and exacerbations in their clinical manifestations, the patient's long-term evolution suggested the mass was cured. We would like to draw the attention of clinicians to the necessity of long-term follow-up in patients initially diagnosed of idiopathic central diabetes insipidus. Furthermore, low dose radiotherapy is a successful treatment for LCH-dependent masses in the hypothalamus; however, normalization or regression of CT abnormalities after radiotherapy did not affect the clinical diabetes insipidus status.

Adult↗
PubMed1991

Histiocytosis X of the hypothalamus.

An unusual case of cutaneous and hypothalamic histiocytosis X (HX) is reported. The hypothalamic involvement occurred as a tumor that mimicked a chiasm glioma on computed tomography angioscanning. Magnetic resonance imaging after gadolinium injection localized the tumor within the third ventricle floor. The HX origin of the tumor was confirmed by histological examination of hypothalamic biopsies obtained by transventricular endoscopy. The results of endocrine evaluation were consistent with anterior panhypopituitarism resulting from a multiple releasing-hormone secretory defect, but there was no diabetes insipidus. This unusual endocrine aspect has not been previously described in the field of hypothalamic HX. Lastly, the tumor was insensitive to low dose megavoltage radiation therapy. This unusual case stresses the superiority of magnetic resonance imaging over computed tomography scanning in the assessment of suprasellar tumors and emphasizes the usefulness of transventricular endoscopy in these cases.

Female↗
PubMed2004

[Hypopituitarism].

Hypopituitarism is usually caused by tumours of the hypothalamus-pituitary region, but may also arise as the consequence of pituitary inflammation, infiltration or hypoperfusion. Tumour mass reduction by surgical intervention or following drug treatment may improve pituitary function. However, neurosurgical tumour resection and radiation therapy may lead to a permanent manifestation of hypopituitarism. Diagnosis is established by endocrine testing, revealing the characteristic low serum concentrations of both peripheral hormone and associated regulatory hormone of pituitary origin. Hypopituitarism may involve from one to all endocrine axes regulated by the pituitary (in order of frequency: growth hormone deficiency>secondary hypogonadism>secondary hypothyroidism>secondary adrenal failure). The treatment of permanent hypopituitarism consists of replacement of the peripheral hormones (hydrocortisone, DHEA, thyroxine, testosterone or oestradiol, growth hormone). Quality of life is impaired in a considerable number of patients with hypopituitarism and mortality is increased, mostly due to cardiovascular and cerebrovascular causes, but also as a consequence of recurrent respiratory infections. Long-term care and monitoring of patients with hypopituitarism requires the experienced endocrinologist.

Adrenal Insufficiency↗
PubMed1997

Pituitary stalk meningioma: case report.

We report a 45-year-old woman with a meningioma which was in contact with only the pituitary stalk on MRI. As the pituitary stalk has no dura mater, we suggest this tumour may have originated from the arachnoid membrane of the pituitary stalk. Though some reports have shown that meningiomas can arise from sites lacking a dural component, this is the first report of a meningioma originating from the pituitary stalk.

Female↗
PubMed2000

Early spontaneous regression of a hypothalamic/chiasmatic mass in neurofibromatosis type 1: MR findings.

A patient with neurofibromatosis type 1 was found to have an enhancing mass in the hypothalamus and in the anterior optic pathway. A 3-month MR study showed a reduction in the size and enhancement of the mass. At a 9-month MR follow-up the mass disappeared and ceased to enhance. This report shows the unusual behaviour of a hypothalamic/chiasmatic mass confirming that in such asymptomatic cases the conservative management can be considered the treatment of choice.

Child, Preschool↗
PubMed2002

Hypothalamic lipoma adjacent to mamillary bodies.

INTRODUCTION: Intracranial lipomas are rare lesions of developmental origin. They are generally asymptomatic and localized in the midline. However, they may occasionally produce neurological symptoms such as seizures. Surgical treatment is rarely indicated. CASE REPORT: We report a child with a lipoma that is located on the ventral side of the mamillary bodies. The diagnosis was made using computerized tomography (CT) and magnetic resonance imaging (MRI). There have been no previous reports of lipomas in this location.

Adolescent↗
PubMed2004

Post-shunt ascites in infants with optic chiasmal hypothalamic astrocytoma: role of ventricular gallbladder shunt.

INTRODUCTION: We report a series of infants with optic chiasmal hypothalamic astrocytomas (OCHAs) who developed abdominal ascites following ventriculo-peritoneal (VP) shunting. The mechanism of ascites development among these patients with OCHA remains speculative and unclear. METHODS: We treated four infants with hypothalamic tumors who were shunted for hydrocephalus using standard VP shunts and who subsequently experienced symptomatic ascites. RESULTS: In three patients the gallbladder proved an effective alternative site for shunting prior to conversion to other sites, and in one patient the gallbladder shunt remains functional and revision-free. CONCLUSIONS: Several aspects of the gallbladder as a reservoir for CSF make this approach appealing. Ventricular gallbladder shunting provided an effective (at least temporarily) receptacle for CSF in these patients.

Ascites↗
PubMed2006

Optic pathway glioma: outcome and prognostic factors in a surgical series.

OBJECTS: The goals of this study were to evaluate the surgical outcomes of optic pathway glioma (OPG) and to analyze the prognostic factors related to the progression-free survival. MATERIALS AND METHODS: A retrospective review was conducted on 33 patients who underwent surgery for OPG; these included 15 male and 18 female patients with a mean age of 8.3 years. The mean duration of follow-up was 52 months. RESULTS AND CONCLUSIONS: The preservation rate of ipsilateral vision was 25%, while that of contralateral vision was 83% (P<0.001). There was no remarkable endocrine improvement after surgery. The overall and progression-free survival rates at 5 years were 93.6 and 52.4%, respectively. In our study, the predictors for tumor progression were children younger than 5 years of age (p=0.023) and of female gender (p=0.022). Because of the variable course of OPG, treatment policy should be optimized individually according to patient's status.

Adolescent↗
PubMed1997

Basal interhemispheric supra- and/or infrachiasmal approaches via superomedial orbitotomy for hypothalamic lesions: preservation of hypothalamo-pituitary functions in combination treatment with radiosurgery.

Although several approaches to the hypothalamus have been used, none is able to give full views of the hypothalamus. The risk of permanent morbidity for hypothalamo-pituitary functions is still high, especially in patients with craniopharyngioma. Basal interhemispheric supra-chiasmal or infra-chiasmal approaches via superomedial orbitotomy were developed for better visualization of the hypothalamus. Operative techniques and results, including combination treatment with radiosurgery, are reported. Twelve patients with tumors compressing the hypothalamus upward or extending into the III ventricle, or both, were operated on: 3 tumors were removed totally, 6 tumors subtotally and 3 tumors partially. Six patients received radiosurgery for residual tumor. Four patients with hypopituitarism preoperatively required oral corticosteroids and thyroid hormones postoperatively. The basal interhemispheric approach via superomedial orbitotomy is useful for better visualization of the hypothalamus and preservation of hypothalamo-pituitary functions.

Adenoma↗
PubMed1998

Computer simulation of a neurosurgical operation: craniotomy for hypothalamic hamartoma.

Although magnetic resonance imaging has revolutionised the management of intracranial lesions with improved visualisation of anatomical structures, it only produces two-dimensional images, from which the clinician has to extrapolate a three-dimensional interpretation. Several approaches can be used to create 3D images; the discipline of image segmentation has encompassed a number of these techniques. Such techniques allow the clinician to delineate areas of interest. The resulting computer-generated outlines can be reconstructed in a three-dimensional arrangement. Although a plethora of "generic" segmentation techniques exist, we have developed a refined form, dependent on general and particular properties of the anatomical structures under investigation. High-contrast structures such as the ventricles and external surface of the head are found by using a localised adaptive thresholding technique. Less definable structures, with poor or nonexistent signal change across neighbouring structures, such as brain stem or pituitary, are found by applying an "energy minimisation"-based technique. To demonstrate the techniques we used the example of an 8-year-old boy with uncontrolled gelastic seizures due to a hypothalamic hamartoma, who is being considered for surgery. We were able to demonstrate the anatomical relationships between the hypothalamic hamartoma and adjacent structures such as optic chiasm, brain stem and ventricular system. We were subsequently able to create a video, reproducing the stages of craniotomy for excision of this tumour. By creating true 3D objects, we were able at any stage of the simulation to visualise structures situated contralaterally to the approaching surgical dissector. These 3D representations of the structures can be either invisible or opaque, in order to afford 3D localisation as the "virtual" surgical dissection proceeds. The clinical application of such techniques will enable surgeons to improve their understanding of anatomical relations of intracranial lesions and has obvious implications in image-guided surgery.

Child↗
PubMed2004

Clinico-pathological features of pilomyxoid astrocytoma of the optic pathway.

Five cases of pilomyxoid astrocytoma (PmA) characterized by a monophasic pattern with a myxoid background were selected for a clinicopathological study from 23 cases previously diagnosed as pilocytic astrocytoma (PA). All PmA patients were either infants or young children (mean age 2.1 years), and all tumors were located in the optic chiasm/hypothalamus region. All cases received chemotherapy, which reduced tumor size, and the location of the tumor became confined to the optic chiasm. In two cases, tumor recurrence occurred 3 and 7 years after chemotherapy. Histology of the recurrent tumors showed the biphasic pattern of classical PA. Hence, we conclude that PmA might be an infantile form of PA and speculate that a subset of PmA in the optic pathway/hypothalamus originates from the optic chiasm, possibly derived from radial glia existing in the embryonic optic chiasm.

Astrocytoma↗