• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.691-695
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• 2006

Hepatoblastoma is a hepatic tumor predominantly occurring in children. The usual site of metastasis is the lung. There are only several reports worldwide on the distant metastasis of hepatoblastoma to the central nervous system in children. Only one reported case showed survival of a patient after multiple resections of a recurrent brain lesion. Involvement of the cardiovascular system has been reported in the medical literature. Lesions almost always involve the right-side of the heart. We report a case of recurrent hepatoblastoma at multiple sites, including brain, left atrium of the heart and lung in a 6-year-old girl who was partially treated in the past at the age of 1.5 years; the patient had been event-free for four and a half years.

• Childhood Kidney Diseases
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• v.24 no.1
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• pp.42-46
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• 2020

Disturbances in water and salt balances are relatively common in children after brain tumor surgery. However, the coexistence of different diseases of water and sodium homeostasis is challenging to diagnose and treat. The coexistence of combined central diabetes insipidus (CDI) and cerebral salt wasting syndrome (CSWS) is rare and may impede accurate diagnosis. Herein, we report the case of an 18-year-old girl who underwent surgery for a germinoma and who presented prolonged coexistence of CDI and CSWS. The patient was diagnosed with panhypopituitarism with CDI at presentation and was treated with hydrocortisone, levothyroxine, and desmopressin. Postoperatively, she developed polyuria of more than 3L/day, with a maximum daily urine output of 7.2 L/day. Her serum sodium level decreased from 148 to 131 mEq/L. Polyuria was treated with desmopressin at incremental doses, and hyponatremia was managed with fluid replacement. At 2 months after surgery, she presented with hyponatremia-induced seizure. Polyuria and hyponatremia combined with natriuresis indicated CSWS. Treatment with fludrocortisone were initiated; then, her electrolyte level gradually normalized. CSWS is self-limiting and generally resolves within 2 weeks. However, the patient in this study still required treatment with vasopressin and fludrocortisone at 16-months after surgery. Hyponatremia in a patient with CDI may be erroneously interpreted as inadequate CDI control or syndrome of inappropriate antidiuretic hormone secretion, leading to inappropriate treatment. The identification of the potential combination of CDI and CSWS is important for early diagnosis and treatment.

• The Journal of Pediatrics of Korean Medicine
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• v.12 no.1
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• pp.55-61
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• 1998

Brainstem gliomas remain an important oncologic problem in the pediatric age group. These tumors constitute between 10 and 15% of all intracranial childhood tumors and despite advanced in the diagnosis and treatment of children with brain tumors, brainstem gliomas are still almost invariably rapidly lethal. We have few of clinical records of braintumors and admission case in the oriental medical hospital, classifications of tumor, symptoms and etc, specially in the pediatrics, so we introduce a case of a 7-year-old child with brainstem glioma which is diagnosised by MRI in our hospital.

• Investigative Magnetic Resonance Imaging
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• v.12 no.2
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• pp.188-196
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• 2008

Purpose : To describe the clinical, MR imaging, and pathologic findings of pediatric meningiomas. Materials and Methods : The authors retrospectively reviewed the medical records and MR images of 16 pediatric patients with pathologically proven meningioma. Mean patient age at diagnosis was 14 years (range, 3-18). MR images were reviewed for details of lesion sizes, locations, signal intensity (SI), marginal characteristics, internal architectures, enhancements, and dural and parenchymal changes. The findings of other imaging modalities and of pathological examinations were also analyzed. Results : Mean tumor size was 5.24 cm (range, 1.3-18.1 cm) and locations were supratentorial in 12 and infratentorial in 4. SI of masses were variable, that is, high in 9, iso in 4, and low in 3 on T2 weighted images (T2WI), and low (n=11), iso (n=4), or high (n=1) on T1WI images. All lesions were visualized as well-demarcated enhancing masses. Five of the tumors were heterogeneous with cystic or necrotic components. Dural attachment was observed in 11 patients and adjacent brain edema in 10. Tumors exhibited hyperdense (n=6) or isodense (n=4) on non-enhanced CT scans, and 3 of the 7 angiograms demonstrated blood supply from the internal carotid artery. Pathologic examinations revealed the following subtypes; transitional cell (n=4), meningotheliomatous (n=4), chordoid (n=2), fibrous (n=2), clear cell (n=1), hyalinized (n=1), rhabdoid papillary (n=1), and atypical (n=1). Conclusion : Pediatric meningiomas occur usually in teenagers, have diverse pathological types, and may produce atypical imaging findings, such as, a heterogeneous internal content or findings suggestive of intraaxial tumors.

• Journal of Music and Human Behavior
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• v.5 no.2
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• pp.67-92
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• 2008

The purpose of this study is to give an overview of the clinical process of songwriting with pediatric patients undergoing cancer treatment. Many literatures have proved that music therapy aids patients during their cancer treatment to relax, reduce anxiety and feelings of isolation, and promote self-esteem and quality of life and so on. However, among music therapy methods, I found songwriting proved to be very effective method. By using songwriting as therapy with pediatric patients undergoing cancer treatment, songwriting helped patients express difficulties of dealing emotionally with their illness and ongoing aggressive and invasive treatments. Also the process of songwriting established trust and relationship between therapist and patient which promoted normalization of hospital environment. In my case study, patient N has expressed her feelings and thoughts through writing songs about her current life which revolved around treatment of her brain tumor. Moreover, patient N found her creativity and developed ways of getting touch with her inner strength.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1110-1115
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• 2007

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was $7.8{\pm}3.6$ (2.1-14.7) years. Mean follow-up duration was $8.6{\pm}5.1$ (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

• Pediatric Infection and Vaccine
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• v.19 no.3
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• pp.141-148
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• 2012

Purpose : Ventriculoperitoneal (VP) shunt insertion is an important treatment modality in children with hydrocephalus. VP shunt infection is a major complication and an important factor that determines the surgery outcome. This 15-year study was performed to evaluate the epidemiology of VP shunt infections in pediatric patients treated at our center. Methods : A retrospective review of medical records was performed in patients 18 years old or younger who underwent VP shunt insertion surgery from April 1995 to June 2010. Results : Three hundred twenty-seven VP shunt surgeries were performed in a total of 190 pediatric patients (83 females, 107 males). The median age of the patients was 2.4 years (range, 0.02-17.9 years). Having a malignant brain tumor was the most frequent cause for VP shunt insertion. The shunt infection rate was 6.7% (22/327) per 100 operations and 9.5% (18/190) per 100 patients, and the incidence rate was 0.45 infection cases per 100 shunt operations-year. The most common pathogen was coagulase-negative staphylococcus (n=7) followed by methicillin resistant Staphylococcus aureus (n=1). Ten cases were treated with vancomycin and beta-lactam antibiotic (cephalosporin or carbapenem) combination therapy and 7 cases were treated with vancomycin monotherapy. The median duration of antibiotic treatment was 26 days (range, 7 to 58 days). Surgical intervention was performed in 18 cases (18/22, 81.8%). Conclusion : Epidemiologic information regarding VP shunt infections in pediatric patients is valuable that will help guide proper antibiotic management. Additional studies on the risk factors for developing VP shunt infections are also warranted.

• Clinical and Experimental Pediatrics
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• v.51 no.7
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• pp.766-770
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• 2008

Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.206-210
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• 2007

Turcot syndrome is characterized by the concurrence of a primary neuroepithelial brain tumor and multiple colorectal polyposis. We report a case of a 24-year-old woman diagnosed with Turcot syndrome. At first, the patient was diagnosed as having a medulloblastoma after a tumorectomy of the 4th ventricle mass. The patient underwent radiotherapy and chemotherapy. After high-dose chemotherapy, neutropenic fever and severe mucositis developed. For an evaluation of the persistent hematochezia and diarrhea, a colonoscopy was performed. It revealed pseudomembranous colitis and multiple polyps in the entire colon. According to the family history, her father had undergone a total colectomy due to colon cancer and polyposis of the entire colon. Her brother also was found to have multiple polyps in the colon by a colonoscopy. The patient was diagnosed with Turcot syndrome.

• 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.