We retrospectively analyzed the frequency of and risk factor for endocrine dysfunction in 104 patients (61 males, 43 females) with pediatric cancer who were alive for at least one year after cancer treatment between 1983 and 2014. The median age at initial diagnosis and the last follow-up were 4 years (range, 0–16 years) and 15 years (range, 1–33 years), respectively. The disease subtypes were central nervous system (CNS) solid tumors without the involvement of the pituitary gland (n=22) and non-CNS solid tumors (n=82). Forty-eight (46.2%) of the 104 patients had at least one endocrine dysfunction. Gonadal dysfunction was observed in 33 (45.8%) of the 72 evaluable patients: high doses of cyclophosphamide (≥7.5 g/m²) or ifosfamide (≥60 g/m²) and age at diagnosis (≥10 years) were the independent risk factors. Thyroid dysfunction was observed in 18 (18.9%) of the 95 evaluable patients; head and neck irradiation was an independent risk factor. Growth disturbance/growth hormone deficiency was observed in 16 (19.3%) of the 83 evaluable patients; however, no independent risk factors were identified. None of the patients had adrenal gland dysfunction. A higher risk of gonadal dysfunction was found in patients with osteosarcoma or other sarcomas, and neuroblastoma, whereas multiple endocrine dysfunctions were detected in patients with medulloblastoma and intracranial germ cell tumor. Treatment should be improved to reduce the risk of late adverse effects for patients with bone and soft tissue tumors or brain tumors who were treated with high-dose alkylators and radiation therapy and thus have an increased risk of endocrine dysfunction.

The identification of pathogenic fungi is difficult in pediatric oncology patients. We retrospectively analyzed the clinical courses and susceptibilities of 2 filamentous fungi and 5 yeasts isolated from 6 patients who were treated in our institution between January 2004 and December 2014. Aspergillus terreus was isolated repeatedly from the ear discharge of a patient with otitis externa during neutropenia. In accordance with susceptibility test results, the patient safely received stem cell transplantation with voriconazole (VRCZ) combined with micafungin (MCFG). All the yeasts detected in the patients with bloodstream infections were Candida species. Although the β-D-glucan titer increased and the clinical manifestation of splenic abscess worsened, we treated C. tropicalis infection with MCFG only on the basis of the susceptibility test results. Although C. glabrata is sensitive to MCFG, the C. glabrata from our patient showed a high minimum inhibitory concentration against MCFG. Recently, echinocandin resistance to Candida has been increasing in the United States. Therefore, the identification of pathogenic fungi and drug susceptibility test results will become more important for treating pediatric oncology patients.

We experienced managing the symptoms of two adolescents with cancer (an 18 year old with epithelial sarcoma and a 17 year old with rhabdomyosarcoma) who received continuous intravenous infusion of midazolam at home to relieve end-of-life distress. We obtained written informed consent not only from their families but also from the patients themselves. The estimated prognosis using the Palliative Prognostic Index was less than 3 weeks in both patients. The symptoms that required sedation were restlessness in end-of-life distress, insomnia, and anxiety in one patient, and refractory thirst, nausea, and vomiting in the other patient. The starting doses of midazolam were 3 and 1.25 mg/h, and we increased the flow velocity by 20 to 30% until pain is relieved if there is no respiratory depression. We succeeded in symptom management after increasing the doses by twofold. The duration of continuous sedation was less than 2 weeks and there were no drug-related lethal complications. Even at home, sedation by continuous intravenous injection of midazolam is considered safe if the medical staff closely cooperate with each other.

Many hospitals that treat pediatric cancer have self-help groups for parents of pediatric cancer patients (parents’ groups). Although such groups are independent from medical professionals and are a useful source of reassurance for patients’ parents, some hospitals that treat pediatric cancer do not have parents’ groups. One of the reasons for this is that there are no volunteers at these institutions that want to launch and manage a parents’ group. We successfully launched a parents’ group initiated by doctors. The group leaders were directly chosen by the doctors, and the details of the purpose of the parents’ group were decided in a preparatory meeting. The first meeting of the parents’ group was held 3 months after the initial proposal. Although doctors launched the parents’ group, medical professionals do not participate in it. To ensure that the group’s activities continue, it will be important to keep the same venue, inform relevant families about the group, and encourage medical professionals to support the group. The launch of the parents’ group has led to the development of smooth relationships between the patients’ parents and the medical staff. Therefore, the parents’ group not only functions as a peer-support organization, but also helps in forming bonds between patients’ parents and medical professionals. This approach could be used as a model by hospitals that would like to launch parents’ groups.

[Objective] To elucidate educational targets for cancer practice including pediatric oncology for nurse practitioners (NPs) by comparing Japanese physicians and nurses with NPs in the United States (US).

[Methods:] We conducted a questionnaire-based survey of educational targets for cancer practice in cooperation with oncology physicians and pharmacists.

[Results] Responses were obtained from Japanese physicians (n=230), Japanese nurses (n=246), and US NPs (n=166). The mean educational goals for NPs were high among Japanese nurses (81.3%) and US NPs (78.0%). However, Japanese physicians considered that knowledge of palliative care and adverse effects of anticancer drugs are important (>80%), whereas most other fields are not necessary (mean 57.2%). US NPs, despite their different fields, considered basic oncological knowledge to be a fundamental part of education for NPs.

[Conclusions] The nurses and NPs in both countries consider that comprehensive basic oncological knowledge is indispensable as an educational goal for NPs. Japanese physicians expect NPs to have more knowledge of palliative care and adverse effects of medication than comprehensive basic oncological knowledge.

Herein, we report 2 infantile cases of giant retroperitoneal teratoma successfully resected on the basis of 3D-CT findings. Case 1 was a 3-month-old girl who had developed remarkable abdominal distension. Preoperative 3D-CT angiography revealed that the left renal vein was deviated and disrupted by tumor compression. Collateral vascularity via ovarian vein was expected, and minimal handling during dissection around the renal hilus enabled tumor resection while preserving the left kidney. Case 2 was a 4-month-old girl, and 3D-CT revealed that the inferior vena cava (IVC) and bilateral renal veins were disrupted with the development of collateral vascularity via vertebral venous plexus. Successful tumor resection was performed with the sacrifice of IVC and right renal vein. Both patients showed no renal dysfunction postoperatively. 3D-CT angiography is mandatory for preoperative evaluation, and a surgical procedure based on the evaluation of collateral circulation is crucial for the successful resection of such tumors.

A previously healthy 14-year-old girl complained of headache, appetite loss and depression. She had lost 36% (13.5 kg) of her weight over six months owing to low food intake and frequent vomiting. CT revealed an obstructive hydrocephalus due to a 6 cm mass occupying the third ventricle. She was diagnosed as having pituitary germ cell tumor (germinoma with STGC) on the basis of surgical biopsy results and serum marker levels. Her condition was complicated by panhypopituitarism, diabetes insipidus, anemia (Hb 9.0 g/dL), and thrombocytopenia (Plt count, 58,000/μL). Bone marrow examination revealed marked hypoplasia accompanied by gelatinous degeneration. We performed reduced dose chemotherapy and proton beam therapy targeting the whole ventricle and tumor bed, excluding the craniospinal region. The gelatinous degeneration of the bone marrow was resolved by sufficient nutritional control, hormone replacement therapy, and treatment of the brain tumor. Her blood cell count also normalized subsequently. Most reported cases of gelatinous bone marrow degeneration are found among patients with anorexia nervosa. Not only significant malnutrition but panhypopituitarism may have contributed to her condition.

Purpura fulminans (PF) associated with severe protein C deficiency rapidly progresses and often becomes severe. The human prothrombin complex concentrate (PPSB^®-HT), which contains appreciable amounts of protein C, can be used for home infusion therapy in hemophilia patients. We treated two patients with human-plasma-derived activated protein C concentrate (APC), but this treatment required frequent hospital admission, which put a great strain on them and their respective families. Therefore, we changed APC to PPSB^®-HT for home infusion, which provided an early interventional measure to prevent serious conditions. Pediatric patients with congenital protein C deficiency await the approval of PPSB^®-HT for use in hemophilia treatment.