A 14-month-old girl presented with petechial skin lesions and polydipsia was diagnosed as Langerhans cell histiocytosis (LCH) and responded fairly well to multiple chemotherapies using vincristine, cyclophosphamide, and prednisone. 3 years later, relapses were more common with short periods of remissions in spite of using more intensive therapy with vinblatine and etoposide. At age of 4.5, sudden weight gain and abnormal behavior led to MRI study and revealed an hypothalamic mass. Radiation of 1, 800 cGy was given to the mass and followed by a 75% decrease in measuring and remission of the obesity. Although, there was no evidence of tumor progression in the hypothalamus, she died of sepsis due to systemic progression of the disease at age of 5. LCH commonly present with the symptoms of diabetes insipidus, but hypothalamic mass is not common. We report this case with a brief review of literatures.
Cyclophosphamide ; Diabetes Insipidus ; Drug Therapy ; Etoposide ; Female ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypothalamus ; Infant ; Magnetic Resonance Imaging ; Obesity ; Polydipsia ; Prednisone ; Recurrence ; Sepsis ; Skin ; Vincristine ; Weight Gain

Pheochromacytoma, although occasionally present with adrenal cortical hyperfunction, is rarely associated with nonfunctioning adrenal cortical tumor. To our knowledge, eight cases of phemchromocytoma associated with adrenocortical adenoma have been reported in the literature, including a case in a Korean adult female. An adrenal mass is considered an incidentaloma when there is no history or physical findings suggesting an adrenal functional disorder or tumor. The majority of adrenal masses are nonfunctioning adrenocortical adenomas. In our case, left adrenal mass was found incidentally by ultrasonography after birth, done because of mother's oligohydroamnios. Abdomial CT study revealed an adrenal tumor, and a surgical resection was performed. The specimen showed a coincident pheochromocytoma and adrenocortical adenoma. The patient seems to be the first case of coexistence of nonfunctioning pheochromocytoma and adrenocortical adenoma in one adrenal tumor. We report this case with the review of literatures.
Adrenocortical Adenoma* ; Adult ; Female ; Humans ; Infant* ; Parturition ; Pheochromocytoma* ; Ultrasonography

Hepatoblastoma is the most common primary malignant hepatic tumor in infancy and childhood. But congenital hepatoblastomas are extremely rare and show distinctive, but important features when compared with tumors diagnosed in children beyond the neonatal age. They have different clinical presentations, higher incidence of pure fetal histology, significant risk for systemic metastasis, and worse outcome. The treatment of congenital hepatoblastoma should be the same as in older children, i.e., primary chemotherapy with delayed resection. We report a case of congenital hepatoblastoma in a 29-day-old boy who was known to have a well-defined ovoid hypoechoic mass at liver demonstrated by fetal sonography.
Child ; Drug Therapy ; Hepatoblastoma* ; Humans ; Incidence ; Liver ; Male ; Neoplasm Metastasis

The presence of rare paraneoplastic syndrome, the opsoclonus-myoclonus-ataxia syndrome (OMA), may strongly signal the presence of neuroblastoma. We report a case of ganglioneuroblastoma presented with OMA. A 26 month-old girl was admitted due to progressive ataxic gait and myoclonic jerking of the limbs. Brain and spine MRI scans were normal and cerebrospinal fluid analysis showed no specific abnormal finding. Abdominal computed tomography (CT) demonstrated about 3x1.5 cm sized well enhancing solid mass originated from the right adrenal gland. Urinary vanillyl mandelic acid (VMA) was mildly elevated and urinary homovanillic acid (HVA) was normal. After complete resection of the tumor, she was diagnosed with ganglioneuroblastoma and her symptomatology had disappeared.
Adrenal Glands ; Brain ; Cerebrospinal Fluid ; Child, Preschool ; Extremities ; Female ; Gait ; Ganglioneuroblastoma* ; Homovanillic Acid ; Humans ; Magnetic Resonance Imaging ; Myoclonus ; Neuroblastoma ; Paraneoplastic Syndromes ; Spine

Hypereosinophilia has been associated with a variety of underlying disorders such as parasitic, fungal and mycobacterial infections, allergic disorders, collagen vascular diseases, or hypereosinophilic syndrome (HES). The association of acute lymphoblastic leukemia (ALL) and symptomatic eosinophilia is rare and only a few cases have been reported. HES probably occurs in less than 1% of all patients with ALL. The chromosomal translocation t (5; 14) (q31; q32) was cloned at the molecular level in ALL with eosinophilia. This translocation joined the immunoglobulin heavy chain region to the promoter region of the interleukin-3 (IL-3) gene in opposite transcriptional orientation. The IL-3 gene translocated with the immunoglobulin heavy chain gene may play a central role in the pathogenesis of this leukemia and the associated eosinophilia. We describe a 8-year-old boy who presented with hypereosinophilia and concurrent ALL with t (5; 14).
Child* ; Clone Cells ; Collagen ; Eosinophilia ; Humans ; Hypereosinophilic Syndrome ; Immunoglobulin Heavy Chains ; Interleukin-3 ; Leukemia ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Promoter Regions, Genetic ; Translocation, Genetic* ; Vascular Diseases

PURPOSE: Mesenchymal stem cells (MSC) can be isolated from bone marrow (BM) and when systemically administrated to different species, they undergo site-specific differentiation. In this study, we isolated MSC from human BM and generated a continuously growing colony of cell lines (SNU-hMSC) with SV40 large T antigen. The purposes of this study are to identify whether SNU-hMSC have the characteristics of MSC and their possibility of chondrogenic differentiation. METHODS: MSC were mobilized from BM and cultured in DMEM-LG media for 2 weeks. We obtained SNU-hMSC, by introducing a viral vector of SV40 large T antigen and culturing it in the selected media for 6 months. We identified specific cell markers of MSC via FACS analysis and analyzed expression of cytokines, chemokines and receptors by RT-PCR. To stimulate the proliferation of the cells, we processed the media with FGF, BMP-2 and IL-6. The each medium's cell counts were counted in day 7 and day 14. To differentiate SNU-hMSC, they were cultured in chondrogenic media. After 2 weeks, chondrogenic differentiation was evaluated with safranin-O staining and the expression of COMP, aggrecan and SOX-9. RESULTS: SNU-hMSC exhibited MSC markers. When the IL-6, BMP-2 and FGF were added to each medium, the cell numbers were significantly increased as compared with control. In the study of differentiation, SNU-hMSC exhibited strong safranin-O staining, and chondrogenic gene expression was observed. CONCLUSION: SNU-hMSC expressed markers and cytokines identical with MSC. SNU-hMSC maintained multipotency of differentiation.
Aggrecans ; Antigens, Viral, Tumor* ; Bone Marrow ; Cell Count ; Cell Line ; Chemokines ; Chondrocytes* ; Cytokines ; Gene Expression ; Humans* ; Interleukin-6 ; Mesenchymal Stromal Cells*

PURPOSE: Characteristic clinical findings of fever, skin rash with or without the evidence of fluid retention, mimicking engraftment syndrome (ES), have been observed during the pre-engraftment period in patients with hematopoietic stem cell transplantation (HSCT). We described these findings as pre-engraftment syndrome (pES) and analyzed the incidence and risk factors of pES in the pediatric patients who received HSCT with various stem cell sources. METHODS: Among 53 patients who received HSCT at Dong-A University Medical Center from Sep. 1997 to Mar. 2004, 37 patients with allogeneic HSCT were analyzed retrospectively to characterize the clinical syndrome. RESULTS: In 3 (21.4%) out of 14 patients with cord blood stem cell transplantation, non-infectious fever, skin rash and tachypnea developed on 4~15 days prior to neutrophil engraftment. Two of them spontaneously recovered just with fluid restriction and oxygen inhalation, however, one patient died of complicated pulmonary hemorrhage in spite of aggressive supportive therapy and steroid treatment. In 4 (17.4%) out of 23 patients with allogeneic bone marrow transplantation (BMT), non-infectious fever and skin rash developed on 4~5 days prior to neutrophil engraftment. All of them recovered with steroid treatment only. We could not find any risk factors for this syndrome, however, the speed of neutrophil engraftment was significantly faster in the patients with pES. CONCLUSION: We established a distinctive clinical syndrome during pre-engraftment period, which is very similar but different in occurrence timing from ES. The pES may be associated with enhanced engraftment, but has no impact on the other clinical outcomes.
Academic Medical Centers ; Bone Marrow Transplantation ; Cord Blood Stem Cell Transplantation ; Exanthema ; Fever ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Hemorrhage ; Humans ; Incidence ; Inhalation ; Neutrophils ; Oxygen ; Retrospective Studies ; Risk Factors ; Stem Cells ; Tachypnea

PURPOSE: In order to monitor the clinical outcome of pediatric allogeneic stem cell transplantation (SCT), serial evaluations of chimerism were performed to compare the risk of relapse or graft rejection between patients with complete chimerism (CC) and mixed chimerism (MC). METHODS: Between January, 1996 and April, 2004, 64 cases who underwent SCTs were prospectively enrolled. Serial genotyping of VNTR (variable number of tandem repeats) /STR (short tandem repeats) loci and/or X-chromosome-specific FISH (fluorescent in situ hybridization) were performed at regular intervals. RESULTS: The "informative loci" were found in all 64 patient/donor pairs. CC was persistently detected in 44 cases (68.7%), while MC was detected at least once in 20 (31.3%). In cases with malignancy (n=40), relapse was more frequently encountered in MC group (7/8) than in CC group (7/32) (P < .001), as was death (75% vs. 28%, P < .05). The Kaplan-Meier 5-year overall survival was higher in CC than in MC (69.1% vs. 16.6%; P < .05). In cases with non-malignancy, MC group showed higher rate of graft rejection than CC group (7/12 vs. 1/12, P < .01). Survival was not different between the two groups. The chimerism status was not influenced by sex, donor type, source of stem cells, and inclusion of radiation in conditioning. CONCLUSION: Detection and sequential assessment of MC might be an important tool to predict relapse of disease in malignant diseases as well as to portend graft rejection in non-malignant illnesses. Earlier intervention to circumvent those life-threatening complications should be pursued based on the chimerism analyses.
Child* ; Chimerism* ; Graft Rejection ; Humans ; In Situ Hybridization, Fluorescence* ; Polymorphism, Genetic* ; Prospective Studies ; Recurrence ; Stem Cell Transplantation* ; Stem Cells* ; Tissue Donors

PURPOSE: Cefepime is a new broad-spectrum antibiotics and is available as initial monotherapy in the management of pediatric cancer patients with febrile neutropenia. The aim of this study is to evaluate the efficacy of Cefepime monotherapy and piperacillin-tazobactam plus gentamicin combination therapy as an initial empirical antibiotics treatment in pediatric cancer patients with febrile neutropenia. METHODS: From January 2003 to July 2004, a total of 55 episodes of febrile neutropenia were analyzed retrospectively for this study. They were treated intravenous Cefepime (50 mg/kg every 8 hour) or piperacillin-tazobactam (90 mg/kg every 8hour) plus gentamicin (2.5 mg/kg every 8hour). Modification of antibiotics was done at 72~96 hour if fever persisted. Clinical responses were evaluated at the completion of therapy. RESULTS: Twenty-eight patients in the cefepime monotherapy and twenty-seven patients in the piperacillin-tazobactam plus gentamicin combination therapy were evaluable for efficacy. Modification of the empirical initial antibiotics therapy was done for 53% in the cefepime monotherapy group compared with 33.3% in the piperacillin-tazobactam plus gentamicin combination therapy group. The modification rate was not statistically significance between the treatment groups (P=0.130). And the overall success rate was 92.9% in cefepime monotherapy and 96.3% in piperacillin-tazobactam plus gentamicin combination therapy. So, there was no significant difference in success rate between two groups (P=0.514). A drug-related adverse event was reported in 1 case of piperacillin-tazobactam plus gentamicin combination therapy. There was no major adverse event. CONCLUSION: The empirical regimen of cefepime monotherapy is at least as effective as the regimen of piperacillin-tazobactam plus gentamicin combination therapy in pediatric malignancy patient with febrile neutropenia.
Anti-Bacterial Agents ; Febrile Neutropenia* ; Fever ; Gentamicins* ; Humans ; Retrospective Studies

PURPOSE: This study is aimed to find out the incidence of late endocrine complications in children who were treated for childhood cancer. METHODS: We reviewed the medical records of 60 patients who was treated for childhood cancer and evaluated various pituitary hormonal functions after completion of treatment from July 1985 to September 2004 at Kyungpook National University Hospital. RESULTS: Forty-four boys (73.3%) and sixteen girls (26.7%) were involved in the study and their ages ranged from 2 months to 14 years. Twenty-four patients were treated with chemotherapy with cranial irradiation and thirty-six were treated with chemotherapy only. The diagnoses included ALL (n=27, 45.0%), AML (n=2, 3.3%), malignant lymphoma (n=13, 21.7%), brain tumor (n=5, 8.3%), MDS (n=1, 1.7%), and other solid tumor (n=12, 20.2%). Forty-four (73.3%) out of the 60 patients had an additional endocrine abnormality. Growth hormone deficiency (GHD) was identified in 44 children (73.3%). Incidence of GHD was significantly higher among those who had received cranial irradiation (91.6% vs. 61.1%) (P=0.009) but this kind of difference was not found in ALL 27 patients (93.7% vs. 72.7%) (P=0.131). Only one patient was diagnosed as primary hypothyroidism, two patients were diagnosed as primary hypogonadism, and these three patients showed growth hormone deficiency. No patient had abnormalities of ACTH and cortisol. CONCLUSION: We thought that late endocrine complications were very common in childhood cancer survivors. Accordingly, we recommended that we should focus on endocrine abnormalities while treating and following up the patients.
Adrenocorticotropic Hormone ; Brain Neoplasms ; Child ; Cranial Irradiation ; Diagnosis ; Drug Therapy ; Female ; Growth Hormone ; Gyeongsangbuk-do ; Humans ; Hydrocortisone ; Hypogonadism ; Hypothyroidism ; Incidence ; Lymphoma ; Medical Records ; Survivors*