Extramedullary relapse in acute lymphoblastic leukemia (ALL) usually occurs in the central nervous system and testes those are known as sanctuary sites. Only a few cases of isolated breast relapse after allogeneic hematopoietic stem cell transplantation (HSCT) have been reported in pediatric ALL, with little known about the optimal treatment and prognosis of such cases. Herein, we report a pediatric case of isolated breast relapse with early T-cell precursor ALL after HSCT. We also describe a literature review of six pediatric cases, including our case.

Ewing sarcoma is a solid tumor involving the bone and/or surrounding soft tissue, which requires multidisciplinary treatment, primarily through a combination of surgery and chemotherapy and, in some cases, radiation therapy. We present the case of a patient who was diagnosed with stage I Ewing sarcoma without metastasis who had a lesion in one of the left 3rd metacarpal bones. The patient’s family members were active Jehovah’s Witnesses; therefore, the patient’s guardian refused blood transfusions for religious reasons. The medical staff explained the fatal side effects of pancytopenia after chemotherapy, and when life-threatening anemia, thrombocytopenia, and neutropenia occurred, the patient’s guardian agreed that the intensity of chemotherapy needed to be either reduced or stopped. The patient’s treatment regimen was as follows: VDC (vincristine, doxorubicin, cyclophosphamide) and IE (ifosfamide, etoposide) were alternately administered as neoadjuvant chemotherapy, followed by complete tumor excision and more chemotherapy. As red blood cell transfusion was impossible, only erythropoietin (EPO; EpokineⓇ, HK inno.N, Seoul, Korea) and granulocyte colony-stimulating factor (G-CSF; GrasinⓇ , Kyowa Kirin, Seoul, Korea) were administered as adjuvant therapy. During the course of treatment, hemoglobin levels decreased to as low as 6.7 g/dL; however, treatment without transfusion was possible with the administration of EPO and G-CSF, with minimal changes in the chemotherapy schedule. With 6 months follow-up post-treatment, the patient is still alive with no relapses observed on positron emission tomography-CT and bone scans.

Acquired hemophilia A (AHA) is a rare bleeding disorder, especially in adolescents and young adults (AYAs) attributable to the development of autoantibodies against coagulation factor VIII (FVIII). AHA diagnosis is difficult; patients lack any history of coagulopathy. We report here on an AYA with AHA who responded well to treatment. A 19-year-old woman visited our hospital with painful swelling of the right lower leg. She had no past or familial history of a bleeding disorder. The initial laboratory data revealed a prolonged activated partial thromboplastin time and an uncorrected mixing test result. The FVIII activity was below 1% and the FVIII antibody level 22.4 Bethesda units. She was diagnosed with AHA and treated with recombinant activated coagulation factor VII, activated prothrombin complex concentrates and an oral steroid. After 9 months, FVIII antibody level was negative and the FVIII activity was normalized. AHA is very rare especially in AYAs, but physicians must be suspicious about the disorder and plan specialized coagulation tests to diagnose the disease. An early diagnosis of acquired bleeding disorders should be done for initiating the adequate treatment immediately by both controlling the acute bleeding episode and eliminating FVIII antibodies.

Acute lymphoblastic leukemia (ALL) is the most common malignancy of children.Gastrointestinal complications can occur during treatment of these children due to disease infiltration in gut or treatment-related toxicity. Intestinal obstruction is one of these complications and this occurs mostly due to constipation and impacted stool. Intussusception is a very rare entity in children with ALL. Left sided colo-colic intussusception is even rarer. Most of the intussusceptions are ileo-colic and are associated with a lead point. We present a case of left sided colo-colic intussusception in an 8-year-old child with ALL. There was no lead point or leukemic infiltrate in the resected part of the intussusception. Our child had typhlitis leading to intussusception which is a very rare occurrence. Awareness regarding this complication with ALL is important for prompt diagnosis and treatment.

Wiskott-Aldrich syndrome (WAS) is characterized by eczema, recurrent infections and microthrombocytopenia, with the latter being the most consistent finding in the course of the disease. The present case report describes a pediatric patient with WAS and severe bleeding phenotype resulting from a very low platelet count. In order to limit platelet transfusions and related risk of allo-immunization, use of recombinant activated factor VII (rFVIIa) for treatment of bleeding was decided. In addition, prophylactic treatment with eltrombopag was attempted, in order to increase platelet count and limit bleeding episodes. Management of the patient with the above mentioned agents led to a significant improvement in his clinical course and overall quality of life. This is the first report on off-label use of rFVIIa in a WAS patient. With regards to the off-label use of thrombopoietic agents in pediatric WAS patients, limited data is available and is reviewed in the report.

Health insurance big data provides real-world evidence of unmet needs in clinical practice and breakthroughs in the medical industry that will impact the future of health care. Big data is expected to revolutionize the current medical paradigm and usher in an era of personalized medicine. In Korea, the Health Insurance Review and Assessment Service and the National Health Insurance Service established large-capacity healthcare big data open systems in 2011 and 2013, respectively, and are providing researchers with secured healthcare big data. However, concerns have been raised regarding the quality of big data-based research. Thus, numerous obstacles remain in leveraging big data research to change medical practice. This paper describes the understanding and practical applications of healthcare big data in pediatric cancer research, ranging from clinical research design using health insurance big data to medical writing.

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