Yuichi Shinkoda

Rhabdomyosarcoma masquerading as acute leukemia

Rhabdomyosarcoma (RMS) masquerading as acute leukemia (AL) is very rare. We describe three cases with RMS without any symptoms of solid tumors. Bone marrow (BM) showed approximately 95% of blast‐like abnormal cells, lacking almost all of the surface antigens of myeloid‐ and lymphoid‐lineage. The immunohistochemistry revealed positive of the cells for actin, desmin and myoglobin. It is important to examine BM samples by immunohistochemistry, when a flow cytometric analysis reveals an unusual presentation. Pediatr Blood Cancer 2009;52:286–287.

Response of heavily treated and relapsed hepatoblastoma in the transplanted liver to single-agent therapy with irinotecan.

Abstract:  We describe here a patient with relapsed hepatoblastoma after LDLT who developed heart failure, which was treated with irinotecan hydrochloride (CPT‐11). His native liver was replaced by a liver graft from his mother at 26 months from the onset. However, LDLT failed to induce complete remission and he was diagnosed as relapsed hepatoblastoma six months after LDLT. We again administered cisplatin and doxorubicin. After six courses of chemotherapy, he developed congestive heart failure because of anthracycline toxicity. The chemotherapy regimen was therefore switched to irinotecan at 35 mg/m2 daily for three days/wk for two consecutive weeks, and repeated every 28 days. After four courses of irinotecan, metastatic lesions were remarkably reduced in size, and the serum level of AFP decreased from 0.7 million to 927 ng/mL. No severe side effects were documented and congestive heart failure improved. These results suggest that irinotecan may be safely given to a patient with relapsed hepatoblastoma after LDLT without serious side effects and may contribute to prolonging the survival.

Serum derivative of reactive oxygen metabolites (d‐ROMs) in pediatric hemato‐oncological patients with neutropenic fever

Early markers for predicting the severity of neutropenic fever (NF) in patients with hemato‐oncological patients have not yet been established. Reactive oxygen species are known to play an important role in the antimicrobial function of neutrophils. The aim of this study was to determine the serum levels of derivatives of reactive oxygen metabolites (d‐ROMs) and the biological antioxidant potential (BAP) levels in these patients, and to investigate the associations between these levels and the severity of NF.

Calcineurin-inhibitor-induced pain syndrome after a second allogeneic bone marrow transplantation for a child with aplastic anemia

Abstract:  We report a 10‐yr‐old boy who developed CIPS after a second allogeneic BMT for severe aplastic anemia. He received the second BMT from the same HLA‐matched sibling donor 16 months after the first BMT due to secondary graft failure. The preparative regimen for the second BMT consisted of fludarabine, cyclophosphamide, and anti‐thymocyte globulin. Prophylaxis for acute GVHD was tacrolimus and oral PSL. Engraftment was achieved on day 15. On day 19, he suddenly complained of intermittent pain in the bilateral lower limbs. Electric shock‐like pain continued for a few minutes in succession. This intractable pain was not ameliorated by various analgesic drugs including pentazocine. MRI demonstrated bone marrow edema with high T2 signal intensity in the femur. He was diagnosed as CIPS based on his symptoms and MRI findings. The trough concentration of tacrolimus (10.1 ng/mL) at the onset of CIPS was within the therapeutic range. Bouts of severe pain naturally resolved after day 43 without the discontinuation of tacrolimus. CIPS is a rare complication in HSCT. This is the first non‐malignant, and the first pediatric, case who developed CIPS after HSCT.

SUCCESSFUL TREATMENT OF AN ENDODERMAL SINUS TUMOR OF THE VAGINA BY CHEMOTHERAPY ALONE: A Rare Case of an Infant Diagnosed by Pathological Examination of Discharged Tumor Fragment

A 7-month-old infant was noted to have vaginal bleeding that was accompanied by a discharged tumor fragment. The histological diagnosis was endodermal sinus tumor. Her serum α-fetoprotein (AFP) was increased to 358.7 ng/mL, and magnetic resonance imaging showed a 1.8 × 1.0 cm tumor in the vagina. She received combination chemotherapy with cyclophosphamide, pirarubicin, carboplatin, and etoposide. The tumor in the images disappeared and the serum level of AFP returned to the normal range after 2 cycles. Treatment was complete without surgical or radiological therapy. More than 45 months after the completion of chemotherapy, she is alive without signs of recurrence.

Fetal Ultrasonography to Prevent Irreversible Neurological Sequelae of Neonatal Neuroblastoma

An intra-abdominal mass was observed by fetal ultrasonography at 32 weeks of gestation. The baby was diagnosed as having neuroblastoma at the time of delivery at 39 weeks and its lower extremities were completely paralyzed. The chemotherapy after birth was quite effective to reduce the mass volume but neurological sequelae failed to improve. By carefully monitoring the movement of extremities, it may have been possible to prevent irreversible paralysis by inducing delivery before that state was reached.

Identical reconstitution after bone marrow transplantation in twins who received fresh and cryopreserved grafts harvested at the same time from their older brother.

Abstract:  We report here the reconstitution after bone marrow transplantation (BMT) in identical infant twins with acute myelogenous leukemia (AML). They were diagnosed at 8 and 9 months of age. Complete remission was induced after two courses of chemotherapy. After four and five courses of chemotherapy, respectively, they received BMT at 2‐month interval from the same HLA‐identical older brother. The total dose of marrow nucleated cells (NC) harvested was 77.7 × 108. The first patient was transplanted with half of the total dose of NC. The remaining cells were cryopreserved without the use of a programmed freezer and transplanted into the second patient 2 months later. The number of days for neutrophil (>0.5 × 109/L), platelet (>50 × 109/L), and reticulocyte (>1%) recovery were, respectively, 15, 21, and 14 in the first case and 12, 21, and 15 in the second case. The clinical courses after BMT were uneventful in both cases, except for mild acute GVHD, and complete remission has been maintained >4 yr with full recovery of immune and marrow function. Based on the results in these cases, we confirmed that marrow cells that have been cryopreserved without the use of a programmed freezer could reconstitute immune and marrow function as well as non‐cryopreserved cells.

Continued complete remission without systemic therapy for isolated testicular relapse after bone marrow transplantation in a boy with acute lymphoblastic leukemia

Abstract:  ITR after BMT in cases of acute lymphoblastic leukemia is relatively rare. Treatment for ITR after BMT generally consists of a combination of local irradiation, orchiectomy, and systemic chemotherapy. However, the effectiveness of these modalities has not been established. Both irradiation and systemic chemotherapy including a second transplantation would result in additional toxicity. In this report we describe a boy with ITR 91 months after BMT who has remained in complete remission more than two yr after a unilateral orchiectomy. We did not treat this patient with systemic chemotherapy, as his ITR developed very late. Our experience suggests that orchiectomy alone is a reasonable option for very late ITR after BMT.

Bone marrow transplantation in children with severe aplastic anemia using a conditioning regimen containing 3 Gy of total body irradiation, cyclophosphamide with or without antithymocyte globulin

Abstract:  We have employed the 3 Gy toal body irradiation (TBI) containing conditioning regimen to bone marrow transplantation (BMT) for severe aplastic anemia (SAA) in pediatric patients irrespective of donor type since March 1986. The outcome of BMT for 17 SAA patients is favorable. Eight patients received BMT from human leukocyte antigen matched‐related donors (MRD) and nine received BMT from alternative donors. The conditioning regimen consisted of 3‐Gy TBI and cyclophosphamide of 200 mg/kg in the BMT from MRD. In the case of BMT from alternative donor, antithymocyte globulin 10 mg/kg was added to the regimen. Fifteen of 17 patients (88%) engrafted on median of day 18 (range, 11–26) and all 13 evaluable patients showed complete donor chimerism by median 30 (range, 13–47) days after BMT. Fourteen patients have survived with a median follow‐up of 67 (range, 2–228) months and the probability of survival was 81.9% (95% CI, 63.3–100%). No late complications including second malignancies caused by TBI have been observed and all three female patients have regular menstruation. In conclusion, TBI of 3 Gy appears to be an appropriate dose regarding to ensure engraftment and avoid the risk of late adverse event for SAA patients.

Successful bone marrow transplantation for children with aplastic anemia based on a best‐available evidence strategy

Okamoto Y, Kodama Y, Nishikawa T, Yamaki Y, Mougi H, Masamoto I, Tanabe T, Shinkoda Y, Kawano Y. Successful bone marrow transplantation for children with aplastic anemia based on a best‐available evidence strategy.
Pediatr Transplantation 2010: 14:980–985.