Tumor lysis syndrome (TLS) is a metabolic derangement that may be seen after the initial treatment of cancer and usually occurs in patients with bulky, rapidly proliferating, or treatment-responsive tumors such as acute leukemia or Burkitt lymphoma. However, it is rare in the treatment of the solid tumor, especially osteosarcoma. An 11-year-old girl was admitted with a 3-months history of severe right leg pain. She was diagnosed as osteosarcoma and was treated with cisplatin and adriamycin. Three days after treatment, the values of potassium, phosphorus, uric acid were 6.4 mEq/L, 5.6 mg/dL and 8.9 mg/dL, respectively. At the same time, the value of calcium was 6.2 mg/dL. Although she was treated with proper conservative treatment including dialysis, she had significant complications such as renal failure, heart failure, hypoxic brain damage. We report a case of TLS in a girl with huge osteosarcoma.
Burkitt Lymphoma ; Calcium ; Child ; Cisplatin ; Dialysis ; Doxorubicin ; Heart Failure ; Humans ; Hypoxia, Brain ; Leg ; Leukemia ; Osteosarcoma ; Phosphorus ; Potassium ; Renal Insufficiency ; Tumor Lysis Syndrome ; Uric Acid

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PPNET) arises from bone or soft tissue of many sites of body, but ES/PPNET, as primary lesion, in the soft tissue of neck is very uncommon. The authors experienced a case of ES/PPNET in a 17-year-old girl, which was manifested as isolated cervical mass in the soft tissue without any other suspected primary lesion or metastasis. We report the case with a brief review of the literature.
Adolescent ; Humans ; Neck ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral ; Sarcoma, Ewing

Solid pseudopapillary tumors of the pancreas (SPTPs) are rare tumors with limited malignant potential; they are mainly diagnosed in young women. Complete surgical resection of the tumor is usually possible and patients have an excellent prognosis. However, local invasion and distant metastases have been reported in about 10-20% of patients with SPTPs; these patients require supplementary management for long-term survival. Here, we report a pediatric patient with inoperable SPTP unresponsive to neoadjuvant chemotherapy using cisplatin, ifosfamide, and etoposide which had spread to other organs. Subtotal resection of the primary tumor, radiofrequency ablation, radiotherapy, and adjuvant chemotherapy using gemcitabine were done to control this progressive SPTP.
Chemotherapy, Adjuvant ; Cisplatin ; Deoxycytidine ; Etoposide ; Female ; Humans ; Ifosfamide ; Neoplasm Metastasis ; Pancreas ; Prognosis

Benign intestinal tumors are rare in infancy, however the authors describe an inflammatory myofibroblastic tumor (IMT) of the mesentery in a 3-month-old infant who was diagnosed incidentally. During operation, huge mass which was attached to proximal jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare condition was done to delineate the natural history of this tumor. Because of the risk of local recurrence and malignant transformation, IMT cases needed a long-term follow up.
Follow-Up Studies ; Humans ; Infant ; Jejunum ; Mesentery ; Myofibroblasts ; Natural History ; Recurrence

Influenza A (H1N1) infection has been noted to be common in the young and high-risk groups for influenza infection, including transplant candidates and recipients. However, the optimal dosage and duration of oseltamivir for severely immunocompromised patients have not been defined. We report the case of a patient with relapsed neuroblastoma who was infected with influenza A (H1N1) and suffered from skin and lung graft versus host disease after he had received allogeneic hematopoietic stem cell transplantation from a matched sibling donor. During the immunosuppressant therapy, he was diagnosed with influenza A (H1N1) infection by real time polymerase chain reaction (RT-PCR). He recovered after oseltamivir treatment with a dosage of 90 mg twice a day for two weeks, which was two times of the standard dose until influenza A (H1N1) RT-PCR was proven to be negative.
Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Humans ; Immunocompromised Host ; Influenza A Virus, H1N1 Subtype ; Influenza, Human ; Lung ; Neuroblastoma ; Oseltamivir ; Real-Time Polymerase Chain Reaction ; Siblings ; Skin ; Tissue Donors ; Transplants

Protein C (PC) is a vitamin K-dependent plasma protein synthesized as a zymogen by liver. It's active form has an anti-thrombotic activity by cleaving the coagulation factor Va and VIIIa. During the last few years, a large number of cases with protein C deficiency (PCD) which contain variable mutations have been documented. Those with hereditary protein C deficiency tend to have an increased risk of thromboembolism. Hypertension, diabetes, hyperlipidemia and glaucoma are the main underlying diseases of the retinal vein occlusion (RVO). Rarely, thrombophilia (antithrombin III, protein S, protein C deficiency) results in the RVO which usually occurs after adolescent period. We recently experienced a case of central retinal vein occlusion by a PCD.
Adolescent ; Factor Va ; Glaucoma ; Humans ; Hyperlipidemias ; Hypertension ; Liver ; Plasma ; Protein C ; Protein C Deficiency ; Protein S ; Retinal Vein ; Retinal Vein Occlusion ; Thromboembolism ; Thrombophilia ; Vitamins

BACKGROUND: Bidirectional traffic of cells at the feto-maternal interface has been shown during pregnancy and fetal cells have been found to persist in maternal peripheral blood for decades after childbirth. Fetal-microchimerism has been reported in women with scleroderma, which shares a number of characteristics with chronic graft versus host disease (GVHD), although its contribution to the disease pathogenesis remains unclear. We performed this study to determine the frequency of male microchimerism in peripheral blood of patients with scleroderma or normal healthy women with son.METHODS: PCR targeting the Y chromosome specific DYZ1 sequence was employed to test DNA extracted from peripheral blood mononuclear cells of 26 women with scleroderma and 10 healthy women who had given birth to at least one son.RESULTS: Male DNA was detected in 16 of 26 (61.5%) women with scleroderma. Whereas male DNA was not detected in any healthy women who had given birth to son.CONCLUSION: Although fetal microchimerism in women with scleroderma was documented, additional studies will be necessary to determine whether microchimerism plays a role in the pathogenesis of this or other autoimmune disease.
Autoimmune Diseases ; Chimerism ; DNA ; Female ; Graft vs Host Disease ; Humans ; Male ; Parturition ; Polymerase Chain Reaction ; Pregnancy ; Skin ; Skin Manifestations ; Transplants ; Y Chromosome

BACKGROUND: Hepatoblastoma is the most common primary malignant tumor of the liver in children. Complete surgical resection is the treatment of choice for cure. However, only 50% are eligible for resection at diagnosis. Recently combination of preoperative chemotherapy and surgery had led improved resectability and survival rate.METHODS: Between May, 2001 and November, 2010, 9 patients were diagnosed with initially unresectable hepatoblastoma at the department of pediatrics, Yeungnam University Hospital. Medical records were reviewed retrospectively. Initial evaluation included complete blood counts, liver function, serum AFP, cholesterol level and abdominal-CT scan. Preoperative chemotherapy was consisted of cisplatin and doxorubicin every 3-4 weeks. Second-line chemotherapy was cisplatin, vincristine and fluorouracil. The treatment response was analyzed by the Response Evaluation Criteria In Solid Tumors (RECIST) criteria.RESULTS: Among 9 patients, male:female was 4:5. Median age at diagnosis was 12 months (4-59 months). The most common presenting symptom was the abdominal mass. Laboratory findings revealed: median AFP 216,841 ng/dL (3,535-1,036,404 ng/dL), anemia: 4, thrombocytosis: 5, elevated AST/ALT: 8, hyperbilirubinemia: 1 and hypercholesterolemia: 5. The median tumor size was 11 cm (8-15 cm). No patient had metastasis. After median 4 (3-5) cycles of preoperative chemotherapy, all patients(100%) showed a partial response and underwent complete surgical resection. Postoperative chemotherapy was given for median 4 (3-5) cycles. The median follow up was 34 months (6-117 months) and all patients are surviving without events.CONCLUSION: Although this study includes limited number of cases, preoperative intensive chemotherapy and surgery for initially unresectable hepatoblastoma in children resulted in excellent outcomes.
Blood Cell Count ; Child ; Cholesterol ; Cisplatin ; Doxorubicin ; Fluorouracil ; Follow-Up Studies ; Hepatoblastoma ; Humans ; Liver ; Medical Records ; Neoplasm Metastasis ; Pediatrics ; Retrospective Studies ; Vincristine

BACKGROUND: Intracranial germ cell tumors are higher in the East Asia such as Korea and Japan than any other Western countries. By analyzing common clinical features of intracranial germ cell tumors in children, we will prevent from misdiagnosing and delaying in the establishment of diagnosis. Furthermore, we can choose appropriate therapeutic plans to improve patient's prognosis.METHODS: We retrospectively reviewed the medical records of 68 patients to investigate and analyze clinical characteristics of intracranial germ cell tumors in children.RESULTS: The average age of 68 patients was 14.8 years old, and the male to female ratio in all patients was 3:1. The most common symptom presented by 30 patients was headache regarded as a nonspecific symptom in brain tumors. Sixty four patients were diagnosed by histologic method called biopsy and most of them were come out into germinoma. Thirty five patients were included in low-risk group and 30 patients were in high-risk group. Intracranial germ cell tumors in this study were most commonly located in the pineal gland.CONCLUSION: There are a variety of types in intracranial germ cell tumors, and they have been accurately diagnosed by radiologic, histologic methods and elevated tumor markers. We concluded that it is necessary for early diagnosis to evaluate exhaustively in patients suspected of brain tumors.
Biopsy ; Brain Neoplasms ; Child ; Early Diagnosis ; Far East ; Female ; Germ Cells ; Germinoma ; Headache ; Humans ; Japan ; Korea ; Male ; Medical Records ; Neoplasms, Germ Cell and Embryonal ; Retrospective Studies ; Biomarkers, Tumor

BACKGROUND: Hemangiomas are the most common benign tumor of childhood. Uncertainties regarding their diagnosis or management often prompt life- or function-threatening complications or unnecessary treatment. The objective of this study is to analyze and understand the clinical features and treatment outcomes of hemangiomas for accurate diagnosis and treatment.METHODS: A retrospective chart review of 60 patients diagnosed as hemangioma in Kangbuk Samsung Hospital from January 2001 to January 2011 was performed.RESULTS: Hemangiomas were observed predominantly in female with the sex ratio of 1:2.2. They were present at birth in 32%, and within 2 months of age in 22% of the patients. In 45% of the patients, hemangiomas occurred on the head and neck, which were the most commonly involved site. 27% of the cases were clinically diagnosed, 40% of the cases were diagnosed after imaging studies, and 37% of cases were confirmed by histopathology. 40% of the patients were observed without treatment. 57% of the patients were treated with surgical excision, corticosteroid, interferon or propranolol. Eight patients were treated with oral propranolol, and seven of them demonstrated regression without major side effects.CONCLUSION: We confirmed that hemangiomas were preponderant in female and usually appeared in first 2 months of life, and the head and neck were the most favored site. We observed that propranolol was effective and safe for treating problematic infantile hemangiomas.
Female ; Head ; Hemangioma ; Humans ; Interferons ; Neck ; Parturition ; Propranolol ; Retrospective Studies ; Sex Ratio