When germline mutations are identified by a cancer multigene panel test on pediatric hematologic oncology, the guidelines for disclosure of secondary findings in multigene panel tests for solid tumors can be used as a reference. It is desirable to note the difference in clinical actionability of the hematology/oncology panel, which aims to not only identify therapeutic targets but also diagnose and predict prognosis. Information on germline mutations is useful in clinical settings. For instance, germline variants in TP53 that cause Li-Fraumeni syndrome (LFS) are a risk factor for the development of second cancers and are useful information to determine the indication for radiation therapy. Guidelines for the management of LFS have been established, cancer surveillance trials have been initiated, and follow-up methods are being established. Clinical guidance describing surveillance methods has also been published for other pediatric cancer predisposition syndromes. Parents and siblings of the patients who have the germline mutation of the hereditary tumor are at 50% risk of carrying the identical mutation. If the patient is a young child, the parent may develop cancer later in life. The indication for genetic testing of siblings should consider the age of onset of the tumor. To make effective use of germline mutation information in clinical practice, we as healthcare professionals need to be aware of the social, psychological, and ethical circumstances surrounding patients with cancer predisposition.

In pediatric patients with mediastinal tumors (MTs), tissue sampling under general anesthesia (GA) is often considered for diagnosis. However, GA for these patients carries the risk of respiratory circulatory failure due to airway narrowing and other causes. The aim of this study was to examine the risks of these situations and to evaluate the effectiveness of MT needle biopsy as a local anesthetic biopsy option. Patients who were diagnosed with MT and had tissue sampling performed at our hospital between 2012 and 2021 were included in this study. Patients diagnosed on the basis of the results of a peripheral blood test and bone marrow examination were excluded. The medical records were reviewed retrospectively. Twenty-one patients were enrolled, with a median age at biopsy of 6 years. Airway symptoms were present in 13 patients, and orthopnea was observed in two patients. Three patients had superior vena cava syndrome and three had pericardial effusion. The median mediastinal mass ratio was 48, the median % cross-sectional area was 75.7, and the Blank risk classification was high risk in eight patients, intermediate risk in nine patients, and low risk in four patients. The diagnoses were lymphoma, neuroblastoma, germ cell tumor, and others. Tissue sampling under GA was performed on 13 patients, and all patients were diagnosed, with one complication. Local anesthesia (LA) was used for tissue sampling in eight patients, and all patients were diagnosed and none had complications. LA is recommended for MT with high risks of adverse effects of GA. Needle biopsy of MT under LA is safe and effective.

Background: Dexrazoxane is reported to reduce anthracycline-induced myocardial damage. Under institutional review board approval, we introduced dexrazoxane combined with anthracycline chemotherapy to determine the short- and middle-term safety of Japanese childhood cancer patients who require high-cumulative anthracycline exposure. Methods: Dexrazoxane was administered at a 10-fold dose of the planned anthracycline dose by 15 min injection immediately followed anthracycline administration. Other therapies were carried out as usual. Before dexrazoxane treatment, informed consent was obtained from their guardians. Results: Thirty-eight children (20 males and 18 females) were treated with dexrazoxane from February 2015 to January 2022. The diagnoses were brain tumors in 9, solid tumors in 25, and hematological malignancies in 4 patients. Their median age at their first dexrazoxane treatment was 4 years and 2 months. Eleven patients received stem cell transplantation (allogeneic in 3 and autologous in 8), and six patients received living-donor liver transplantation. The median cumulative anthracycline dose at the start of dexrazoxane treatment was 260 mg/m², and at the last treatment, it was 375 mg/m². There was no adverse event due to dexrazoxane during their treatment. Bone marrow suppression periods and organ function seemed to be unaffected. The median observational period of the 38 patients was 657 days. So far, no one has developed heart failure or secondary cancer. Four patients died due to their cancer. The long-term heart-protective and adverse effects of dexrazoxane need to be evaluated with a longer follow-up period. Conclusion: Dexrazoxane can be used safely without any acute adverse effects on anthracycline chemotherapy among Japanese children with cancer.

Background: We established a regional oncofertility network in the Tokai and Hokuriku regions started operating it in August 2020. This network aims to optimize care and improve knowledge about oncofertility. We focused on children and adolescents with hematological cancer in this study.

Methods: This retrospective study was conducted at nine institutions in the Tokai and Hokuriku regions on children and adolescents with hematological cancer. We investigated patients under 18 years of age and diagnosed as having hematological cancer, who visited oncofertility clinics in the nine institutions between January 2013 and June 2021.

Results: This study included 115 patients (71 males and 44 females) with confirmed hematological cancer. The consultation rate was lower in patients younger than 12 years old. Of the 66 patients who attempted sperm cryopreservation, it was unsuccessful in nine patients because of aspermia. Of the 17 patients who attempted ovarian tissue cryopreservation, the cryopreservation before hematopoietic stem cell transplantation was successful in 16 patients. Seven patients also received a perioperative blood transfusion. Among the 11 patients who attempted oocyte cryopreservation, it was achieved in eight patients, including patients under 14 years of age.

Conclusion: We expect to support and improve this oncofertility network to explore the need for fertility-preserving procedures on children and adolescents with hematological cancer.

The purpose of this study was to identify the difficulties that childhood cancer survivors face in their school life and the corresponding late physical and/or psychosocial effects, thus promoting an understanding of the support these children may require. An anonymous questionnaire on learning status, difficulties, and support after returning to school was conducted; 16 children provided valid responses, which were analyzed. The median age at diagnosis was 11.5 (8–17) years. Eleven children were transferred to hospital schools. The median daily study time per hospital stay was three hours. After discharge, the median time of nine children spent at home was two (1.5–3) months. After school reentry, 10 children experienced cognitive difficulties, including poor learning outcomes or deterioration of grades caused by the loss of concentration and/or memory. Seven of those affected began to experience such learning difficulties within one year after returning to school, whereas for three students, the difficulties began one to two years later. Only one child talked to a teacher, and six could not resolve their problems. During gymnastics classes, nine children (56.3%) could not participate in exercises. The nonparticipation problem occurred more than six years after school reentry for three children. To tackle survivors’ problems reasonably, building a support system that ensures the availability of teachers for consultation is imperative so that children can discuss their difficulties as needed. Cooperation and follow-up care from medical, community health, and special needs educational institutions are also necessary.

Children with retinoblastoma (RB) undergoing enucleation start wearing a prosthetic eye and thus need to learn prosthesis care. The purpose of this study was to identify the status quo of artificial eye care in children with RB and the difficulty faced with learning prothesis care during the learning process. Semi-structured interviews were conducted with 18 mothers of children with RB aged 3 to 10 years undergoing enucleation and who are learning or have mastered prosthesis care. Categories and subcategories were extracted using a qualitative descriptive design. The median (min.–max.) age of the mothers was 38 (35–46) years. The median (min.–max.) age of the children at enucleation was 19 (1–59) months. As the status quo, 33 categories were extracted from 91 subcategories, including the following: “Encouraging a child when they voluntarily try to tend to their artificial eye”, “Encouraging children to understand the purpose of care”, “Removing the artificial eye with a suction cup”, “Washing and wiping clean the prosthesis after use during the day”, and “Taking good care of one’s own sense during wearing/removal of the prosthesis”. Prosthesis care included the preparation of the prosthesis, wearing/removal, maintenance of cleanliness, prevention of damage and loss down the drain, and privacy protection. Various ways to care for prosthesis were developed. Mothers faced the fear of losing the eye and had difficulty encouraging their children to understand and partake in prosthetic eye care. This suggests a need for education on eye conditions and methods of care according to patients’ lives during hospitalization after enucleation and for support to facilitate learning of the care process in a manner that respects children’s independence.

We present the case of an 8-year-old girl diagnosed as having B-cell precursor acute lymphoblastic leukemia (BCP-ALL) at the age of 4 years. Her initial steroid response was good. The first remission was achieved after induction therapy and maintained with chemotherapy for 2.5 years. Bone marrow relapse occurred 4.5 years after the initial presentation and 2 years after therapy. A leukemia-related fusion gene test revealed TCF3-ZNF384 positivity. During second remission, bone marrow transplantation was performed from her HLA8/8 allele-matched father using a conditioning regimen (melphalan, 180 mg/m²; total-body irradiation, 12 Gy). Neutrophil recovery was achieved 17 days after transplantation, with no acute complications other than acute graft-versus-host disease of the skin. She remains disease-free one year after transplantation. The prognosis of recurrent TCF3-ZNF384-positive BCP-ALL is poor, and hematopoietic cell transplantation may provide good results. Although this patient achieved good results using a myeloablative conditioning regimen, the appropriate conditioning regimen intensity remains unclear, warranting future investigation.

Ogilvie syndrome, also called acute colonic pseudo-obstruction, is a rare disorder characterized by abrupt and progressive colonic dilatation without mechanical obstruction. We report the case of a 13-year-old girl who complained of severe abdominal pain and constipation two months after the completion of chemotherapy for acute myeloid leukemia. She presented with marked bowel distention, impaired consciousness, and generalized blisters with crust formation. Abdominal computed tomography and brain magnetic resonance imaging showed marked colonic dilatation and a high-intensity area in the occipital lobe, respectively, on T2-weighted images. On the basis of these findings, Ogilvie syndrome and reversible posterior leukoencephalopathy syndrome resulting from generalized herpes zoster were diagnosed. The patient was treated successfully by the administration of acyclovir, neostigmine, dexamethasone, and glycerol and by transanal tube decompression. Immunological recovery after the completion of leukemia treatment takes several months. Therefore, patients should be cautiously monitored keeping in mind the possibility of severe infectious complications even after the completion of chemotherapy.

Four patients with relapsed/refractory medulloblastoma were treated with bevacizumab/irinotecan/temozolomide triple therapy, together with an intrathecal methotrexate injection. Three patients achieved sustained progression-free survival and improved quality of life. One patient managed to live for up to 54 months after relapsing. This treatment regimen was applied as a bridging therapy in an infant with disease refractory to conventional treatment, thus allowing craniospinal irradiation at the age of three and resulting in long-term remission. No severe adverse effects necessitating the termination of treatment were noted, although the development of severe myelosuppression caused by prolonged repeated administration should be considered. In terms of efficacy and safety compared with the current therapy, bevacizumab/irinotecan/temozolomide triple therapy may be an alternative for treating relapsed/refractory medulloblastoma.

Benign or malignant adrenocortical tumors are rare and difficult to diagnose. A 5-year-old girl presented with premature pubic hair; imaging confirmed a right adrenal tumor. She was diagnosed as having a functional adrenal tumor and gonadotropin-independent precocious puberty. She had no metastases and underwent total resection of the tumor. However, this tumor was judged to be malignant on the basis of the Weiss criteria and benign on the basis of the Wieneke criteria. After comprehensive morphological and immunohistochemical evaluations based on the two specific criteria and the presence of Ki-67, respectively, it was decided that postoperative chemotherapy would not be performed. A year after the surgery, no recurrence was observed, and unnecessary postoperative therapy was avoided.

A 12-year-old male was referred to our center for an anterior mediastinal mass with associated symptoms of persistent cough. Computed tomography (CT) revealed a large mediastinal mass compressing the left main bronchus. First, needle biopsy was carried out under local anesthesia owing to the risk of airway obstruction under sedation. The pathological diagnosis was difficult because of the absence of neoplastic features. Open biopsy was performed under general anesthesia after shrinkage of the mass by combination chemotherapy. However, it was still difficult to make a definitive diagnosis. Two months after chemotherapy, the tumor increased in size, and 18F-Fludeoxyglucose-Positron Emission Tomography/Computed Tomography (PET-CT) revealed an abnormal uptake of FDG at the solid part of the mediastinal mass. A third biopsy was performed by thoracotomy, and the site of intense uptake of FDG was resected. Finally, he was diagnosed as having large B-cell lymphoma. It is essential to establish a biopsy protocol that is both safe and reliable, especially for cases of oncologic emergency.

[Case] The patient was a girl aged one year and eight months. [Clinical course] A mass in the left abdomen was noticed several days before hospitalization. Imaging revealed a giant tumor (maximum diameter of approximately 9.5 cm) that extended beyond the midline, originating near the inferior pole of the left kidney, indicative of a nephroblastoma. Owing to its large size and a strong suspicion that the tumor was dorsally adherent to the retroperitoneum, its total removal was deemed difficult. We administered preoperative chemotherapy using the International Society of Pediatric Oncology (SIOP) WT 2001 regimen, which led to a tumor reduction. Tumor resection was subsequently performed. The surgical margins were negative for tumor cells, and the pathological diagnosis was congenital mesoblastic nephroma. [Conclusion] Tumor reduction by preoperative chemotherapy facilitated the complete removal of a congenital mesoblastic nephroma. The administration of anthracyclines and radiation therapy could be avoided by using the SIOP WT 2001 regimen.

In children with PRETEXT III hepatoblastoma, trisegmentectomy is considered for complete anatomic resection, but it may result in posthepatectomy liver failure. Herein, we present three cases of patients on whom hemihepatectomy + subsegmentectomy was performed to obtain radicality and to preserve remnant liver volume. All patients achieved complete microscopic resection and a recurrence-free survival of more than 5 years. There was no significant difference in the surgery duration or blood loss between these three patients and pediatric patients with hepatoblastoma who underwent hemihepatectomy. There was also no significant difference in the duration until postoperative chemotherapy. This procedure may allow both complete anatomical resection of the tumor and preservation of residual liver volume equivalent to hemihepatectomy.