• Journal of Korean Neurosurgical Society
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• v.30 no.9
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• pp.1115-1119
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• 2001

Objective : Management strategies for pediatric chiasmatic-hypothalamic gliomas(CHG) include surgery, irradiation, chemotherapy and a combination of these modalities. This study was performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. Material and Method : We have reviewed the results of management of 6 children with a diagnosis of CHG, who were observed closely during the last 8 years. The patients were aged 7 months to 15 years. Our patients presented with diencephalic syndrome, endocrine dysfunction, and progressive visual loss. None of these had evidence of neurofibromatosis-1. Treatment consisted of surgery alone(2), surgry and irradiation(2), surgery, irradiation and chemotherapy(1), and surgery and chemotherapy(1). Results : Four children had large exophytic suprasellar tumors and two showed diffuse midline lesions. Obstructive hydrocephalus was present in all patients. Pathologic examination revealed anaplastic astrocytoma in 1 and lowgrade astrocytoma in 5. Two patients, recently treated with radiation therapy following radical subtotal resection, showed significant tumor reductions and good clinical status. Four patients had partial tumor resection. Of these patients, two developed disease stabilization during follow-up period of 7 and 8 years, respectively. Life-threatening complications were noted in remaining two patients. Conclusion : CHG may follow an unpredicatable course and show a various reponse to each treatment modality. Further studies are indicated to define the optimal method of treatment of CHG in childhood.

• Journal of Korean Neurosurgical Society
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• v.58 no.5
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• pp.432-441
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• 2015

Objective : To assess the impact of the complete resection of craniopharyngioma (CP) in adults on oncologic and functional outcomes. Methods : We retrospectively analyzed 82 patients with CP who were surgically treated by the same neurosurgeon at our institution between January 1994 and December 2012. Results : Gross total resection (GTR) was achieved in 71 patients (86.6%), near total resection (NTR) in 7 patients (8.5%), and subtotal resection (STR) in 3 patients (3.7%). The disease-specific overall survival rate was 100% with the exclusion of 2 surgery-related mortalities. The overall recurrence rate was 12.2% (10 of 82 patients), however the recurrence rate according to extent of resection (EOR) was 9.9% (7 of 71 patients) after GTR, 14.3% (1 of 7 patients) after NTR, and 66.7% (2 of 3 patients) after STR. The overall recurrence-free survival (RFS) rates at 5 and 10 years were 87.0% and 76.8%, respectively. Postoperatively, most patients (86.3%) needed hormone replacement for at least 1 hypothalamic-pituitary axis. Vision improved in 56.4% of the patients with preoperative abnormal vision, but deteriorated in 27.4% of patients. Hypothalamic dysfunction developed in 32.9% of patients. There were no significant differences in the risks of pituitary dysfunction, visual deterioration, or hypothalamic dysfunction between the groups with complete vs. incomplete removal. The overall rate of postoperative complications was 22.0%, which did not differ between groups (p=0.053). Conclusion : The complete removal of a CP at first surgery can provide a chance for a cure with acceptable morbidity and mortality risks.

• Clinical and Experimental Reproductive Medicine
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• v.19 no.1
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• pp.81-85
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• 1992

The effects of pulsatile GnRH therapy have been studied for the treatment of different forms of hypogonadotropic hypogonadism, including idiopathic hypogonadotropic hypogonadism and hypogonadism developed as a result of treatment with combination of surgery and irradiation for pituitary or hypothalamic tumor. GnRH was administered subcutaneously in a dose of $10{\mu}g$ every 2 hours with the pulsatile infusion pump. With GnRH therapy, all patients improved secretion of LH, FSH and testosterone. Testicular volumes increased. Spermatogenesis was induced in 8 patients. Pulsatile GnRH therapy is an effective treatment for idiopathic hypogonadotropic hypogonadism and can have a role in hypogonadism previously treated with combination of surgery and irradiation for pituitary or hypothalamic tumor.

• Advances in Traditional Medicine
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• v.10 no.1
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• pp.37-43
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• 2010

Palmitic acid (PA) is one of free fatty acids, which is found from Gaultheria itoana Hayata and Sarcopyramis nepalensis. Although PA has a variety of pharmacological effects including mediates hypothalamic insulin resistance, induces IP-10 expression, and promote apoptotic activities, the anti-inflammatory mechanism of PA in mouse peritoneal macrophages remains unclear. In this study, we showed that PA exerted an anti-inflammatory action through suppression the production of tumor necrosis factor-$\alpha$, interleukin-6, cyclooxygenases-2 and nitric oxide in lipopolysaccaride-stimulated mouse peritoneal macrophages. Our study suggests an important molecular mechanism of PA, which might explain its beneficial effect in the regulation of inflammatory reactions.

• Journal of Korean Neurosurgical Society
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• v.29 no.5
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• pp.688-692
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• 2000

The authors report a case of ganglioglioma in unusual site, the hypothalamus. The tumor was completely removed and no recurrence was noted on the follow up. Ganglioglioma usually occurs in the temporal and frontal lobe, but is rarely found on the hypothalamus. We report this case with pertinent literatual reviews.

• Proceedings of the KSAR Conference
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• 2001.03a
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• pp.47-47
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• 2001

The neuropeptide vasopressin (VP) is a nine- amino acid hormone synthesized as preprohormone in the cell bodies of hypothalamic magnocellular neurons. The tumor in magnocellular neurons of the hypothalamus is associated with disfunctions of the cell bodies, leading to the diabetes insipidus. In order to study with the diabetes insipidus caused by a defect in VP synthesis and its secretion, we have produced the transgenic mice regulated by vasopressin promoter inserted to SV40 T antigen coding sequence (pVPSV.IGR2.1). One transgenic line expressing high levels of SV40 T antigen was propagated. The founder and all transgene positive adult animals have appeared with shorten mortality or apparent phenotypic abnormalities, including immune complex disease, and eventually die between 4 and 8 months of age. The mRNA and protein of SV40T antigen transgene were detected in brain of fetus as well as in brain, spleen, lung and lymph node in moribund at the age of 20 weeks. Histological analysis of transgenic mice showed that tumor developed in brain similar to primitive neuroectodermal tumors (PNET) in man. We also detected lymphomas in spleen and lymph node, and consequent tumor formation in various tissues of the transgenic mice. In pVPSV.IGR2.1, 21% mice showed brain tumor (PNET) at 5 weeks and 100% mice showed brain tumor after 15 weeks. In addition, Expression of apoptosis related genes (Bcl-28 & Bax) was increased over their age in mice with PNET as compared to control mice. Apoptosis related gene expression might be deregulated in mice with brain tumor. However, transgenic mice were not developed with the diabetes insipidus. These mice represent the first disease model to exhibit primitive neuroectodermal tumor in brain, as well as a unique model system for exploring the cellular pathogenesis of lymphomas.

• Clinical and Experimental Pediatrics
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• v.58 no.1
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• pp.28-32
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• 2015

Purpose: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. Methods: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. Results: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was $18{\pm}10.5$ months, and diagnosis after symptom onset was made at the mean age of $11{\pm}9.7$ months. The mean z score was $-3.15{\pm}1.14$ for weight, $-0.12{\pm}1.05$ for height, $1.01{\pm}1.58$ for head circumference, and $-1.76{\pm}1.97$ for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. Conclusion: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.

• Journal of Korean Neurosurgical Society
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• v.59 no.5
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• pp.533-536
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• 2016

Pilocytic astrocytomas (PAs) are World Heath Organization Grade I tumors and are most common in children. PA calcification is not a common finding and has been reported more frequently in the optic nerve, hypothalamic/thalamus and superficially located cerebral tumors. We present a cerebellar PA in a 3-year-old male patient with cystic components and massive calcification areas. The residual tumor grew rapidly after the first operation, and the patient was operated on again. A histopathological examination revealed polar spongioblastoma-like cells. Massive calcification is not a common feature in PAs and can lead to difficulties in radiological and pathological differential diagnoses.

• BMB Reports
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• v.57 no.4
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• pp.167-175
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• 2024

Cancer progression is driven by genetic mutations, environmental factors, and intricate interactions within the tumor microenvironment (TME). The TME comprises of diverse cell types, such as cancer cells, immune cells, stromal cells, and neuronal cells. These cells mutually influence each other through various factors, including cytokines, vascular perfusion, and matrix stiffness. In the initial or developmental stage of cancer, neurotrophic factors such as nerve growth factor, brain-derived neurotrophic factor, and glial cell line-derived neurotrophic factor are associated with poor prognosis of various cancers by communicating with cancer cells, immune cells, and peripheral nerves within the TME. Over the past decade, research has been conducted to prevent cancer growth by controlling the activation of neurotrophic factors within tumors, exhibiting a novel attemt in cancer treatment with promising results. More recently, research focusing on controlling cancer growth through regulation of the autonomic nervous system, including the sympathetic and parasympathetic nervous systems, has gained significant attention. Sympathetic signaling predominantly promotes tumor progression, while the role of parasympathetic signaling varies among different cancer types. Neurotransmitters released from these signalings can directly or indirectly affect tumor cells or immune cells within the TME. Additionally, sensory nerve significantly promotes cancer progression. In the advanced stage of cancer, cancer-associated cachexia occurs, characterized by tissue wasting and reduced quality of life. This process involves the pathways via brainstem growth and differentiation factor 15-glial cell line-derived neurotrophic factor receptor alpha-like signaling and hypothalamic proopiomelanocortin neurons. Our review highlights the critical role of neurotrophic factors as well as central nervous system on the progression of cancer, offering promising avenues for targeted therapeutic strategies.

• Proceedings of the KSAR Conference
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• 2002.06a
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• pp.92-92
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• 2002

The neuropeptide vasopressin (VP) is a nine- amino acid hormone synthesized as preprohormone in the cell bodies of hypothalamic magnocellular neurons. The tumor in magnocellular neurons of the hypothalamus is associated with disfunctions of the cell bodies, leading to the diabetes insipidus. In order to produce the disease models with a defect in VP synthesis and its secretion, we have produced the transgenic mice regulated by VP constructs containing 3.8 kbp of the 5'flanking region and all the exons and introns in the mouse VP gene, which was fused at the end of exon 3 to a SV40 Tag. The two VP-transgene constructs differed by the lengths of their VP gene 3' flanking regions (2.1 versus 3.6 kbp). (omitted)