SUCCESSFULLY COMPLETE EXCISION OF A HUGE SACROCOCCYGEAL TERATOMA: A CASE REPORT

A female infant was born with a huge sacrococcygeal teratoma (SCT) at 36 weeks of gestation with a weight of 4546g (combined weight of the infant and tumor). The patient's mother developed polyhydramnios and cesarean section was performed at 36 weeks of gestation. MRI showed Altman classification type Ⅱ SCT. We performed abdominoperineal resection at 3 days after birth. The mass was completely excised, and the size and the weight of the mass were 18×12×15cm and 1100g (Weight of SCT against body weight; 393 g/kg), respectively. The pathological findings revealed a grade Ⅱ immature teratoma with no malignant elements such as yolk sac tumor. The patient was discharged at 45 days after birth with fecal continence, but neurogenic bladder dysfunction. At 9 months after surgery, the patient was doing well with no evidence of tumor recurrence, and AFP levels had returned to normal values

RETROPERITONEAL SCHWANNOMA : A CASE REPORT AND REVIEW OF THE LITERATURE

Reported herein is a rare case of retroperitoneal schwannoma in a patient without von Recklinghausen's disease. This patient was free of symptoms, and the tumor was found by chance during a periodical physical examination. Complete excision of the tumor was performed, and as of this writing, 36 months after the operation, the patient is free of the disease. In addition, a review was carried out on the 121 cases of retroperitoneal schwannoma reported in the Japanese literature from 1981 to 1992. In this series, malignant tumors were seen in 26.4% of all cases, and when this tumor was found in association with von Recklinghausen's disease, the malignancy rate increased to 52.9%. Thirty seven percent of the patients with malignant tumors had died at the time of writing of their case report, and their mean survival period was 18 months after the first surgical treatment. All the patients with benign tumors underwent complete resection and had a good prognosis

神経芽腫におけるPD-L1発現と腫瘍組織浸潤リンパ球の潜在的役割について

Purpose: The programmed death 1 (PD-1)/programmed death ligand 1 (PD-L1) pathway has garnered much attention for its roles in clinical oncology. The aim of this study was to examine the clinical impact of the PD-L1 expression and tumor-infiltrating lymphocytes (TILs) on neuroblastoma. Methods: We evaluated the PD-L1 expression and TIL status in 31 patients with neuroblastoma who underwent a biopsy or resection by an immunohistochemical analysis. Furthermore, we performed the serial analysis of the PD-L1 status before and after chemotherapy in 15 patients. Results: Among the 31 cases, 11 (35%) showed a positive PD-L1 expression. The survival analysis showed a trend toward an association between PD-L1 positivity and a decreased overall survival. PD-L1 positivity tended to be associated with higher levels of tumor markers. In the serial analysis of the PD-L1 status, positivity was noted in 8 of 15 patients before chemotherapy and 6 after chemotherapy. Notably, all four patients with a positive PD-L1 status both before and after chemotherapy had recurrence, and 3 of them died during the follow-up period. Conclusion: Our findings suggest that the PD-L1 tumor expression might be a good biomarker for the treatment of neuroblastoma patients, especially for advanced neuroblastoma.博士（医学）・乙第1458号・令和2年6月30日© Springer-Verlag GmbH Germany, part of Springer Nature 2020This is a post-peer-review, pre-copyedit version of an article published in Pediatric surgery international. The final authenticated version is available online at: http://doi.org/10.1007/s00383-019-04616-9

INDUCTION OF SMALL BOWEL TRANSPLANTATION TOLERANCE BY DONOR-SPECIFIC BONE MARROW TRANSPLANTATION

Immune response after allogeneic small bowel transplantation is more vigorous compared to other organ transplantations. It is often difficult to control small bowel allograft rejection with conventional immunosuppressive therapy. While many experimental studies showed organ transplantation tolerance, there have been few reports of tolerance in allogeneic small bowel transplantation. Induction of bone marrow chimerism is a potent strategy of tolerance induction. We tried to induce tolerance in SBT using donor-specific bone marrow transplantation (BMT) with cyclophosphamide (CYP) and tacrolimus. BN and LEW rats were used as donors and recipients. LEW recipients received BMT from BN donors after injection of CYP. The recipients were further treated with 0.3 mg/kg/day tacrolimus on days 0-6 (n=5). Establishment of bone marrow chimerism in BMT recipients was evaluated by flowcytometry of peripheral blood mononuclear cells. These recipients received small bowel transplantation from BN donors on day 100 after BMT. All of these recipients accepted BN small bowel allografts indefinitely (＞100 days), while untreated LEW controls rejected BN grafts within 8 days (n=6). Histologic signs of chronic rejection were not observed in small bowel allografts in these recipients. In conclusion, donor-specific BMT with a single dose of CYP and a short course of tacrolimus in the early phase successfully induced small bowel transplant tolerance across MHC-barriers. This strategy may lead to technical innovation of immuno- suppressive treatment in clinical small bowel transplantation

PROGNOSTIC SIGNIFICANCE OF p21 AND p53 EXPRESSION IN HEPATOCELLULAR CARCINOMA

The cyclin-dependent kinase inhibitor p21Waf1/Cip1(p21), which can be transcriptionally activated by p53-dependent and -independent manners, functions to block cell cycle progression. In this study, we analyzed the expression of p21, p53 and proliferating cell nuclear antigen (PCNA) in 85 patients with hepatocellular carcinoma (HCC) by inmiunohistochemistry, and examined whether expression of these proteins was related to prognosis in patients with HCC. In HCC, p21 positive tumors significantly showed high PCNA LI and small size, compared with p21 negative tumors. No relationship between p21 and p53 expression was detected. A multivariate Cox model analysis revealed p21, p53 expression and PCNA LI as independent prognostic factors (p=0.0059, p=0.0004, and p=0.0165, respectively). Furthermore, p21 expression significantly correlated with low recurrence rate in the p53 negative cases (p=0.020) and related to a good outcome in the high PCNA LI cases (p=0.056). Accordingly, the analysis of p21, p53 and PCNA could play an important role in early detection of intrahepatic recurrence and might contribute to improvement of the prognostic characterization

Huge hepatocellular carcinoma with multiple intrahepatic metastases: An aggressive multimodal treatment

Introduction: Huge hepatocellular carcinoma (HCC) possesses a potential risk for spontaneous rupture, which leads to a life-threatening complication with a high mortality rate. In addition, a large HCC is frequently accompanied by intrahepatic metastases. Presentation of case: We describe, the case of a 74-year-old woman with a huge extrahepatically expanding HCC with multiple intrahepatic metastases who was treated by liver resection with repeated transcatheter arterial chemoembolization (TACE). To prevent tumor rupture or bleeding, we performed right hepatectomy. After the operation, TACE was applied for multiple intrahepatic metastases in the remnant liver. Furthermore, the elevated protein induced vitamin K absence (PIVKA II) level had decreased to limits within the normal range. Three months after the first TACE, computed tomography revealed several recurrences in the liver. TACE was applied for the second and third time and the tumors were well controlled. Discussion: Although, liver resection is occasionally performed for patients with huge HCC to avoid spontaneous tumor rupture, only surgical approach might not be sufficient for such advanced HCC. To achieve long-term survival, it is necessary to control the residual intrahepatic tumors. We could control multiple intrahepatic metastases with repeated TACEs after hepatectomy. Conclusion: Multimodal treatment involving hepatectomy and TACE might be a good treatment strategy for patients with huge HCC with multiple intrahepatic metastases if the tumors are localized in the liver without distant or peritoneal metastasis

Hepatic artery injury during left hepatic trisectionectomy for colorectal liver metastasis treated by portal vein arterialization

Portal vein arterialization (PVA) has been applied as a salvage procedure in hepatopancreatobiliary surgeries, including transplantation and liver resection, with revascularization for malignancies. Here we describe the use PVA as a salvage procedure following accidental injury of the hepatic artery to the remnant liver occurred during left hepatic trisectionectomy for colorectal liver metastases (CRLM). A 60-year-old man with cancer of the sigmoid colon and initially unresectable CRLM received 11 cycles of hepatic arterial infusion chemotherapy with 5-fluorouracil (1500 mg/week), after which CRLM was downstaged to resectable. One month after laparoscopic sigmoidectomy, a left trisectionectomy and wedge resection of segment 6 were performed. The posterior branch of the right hepatic artery, the only feeding artery to the remnant liver, was injured and totally dissected. Because microsurgical reconstruction of the artery was impossible, PVA was used; PVA is the sole known procedure available when hepatic artery reconstruction is impossible. The patient then suffered portal hypertension, and closure of arterio-portal anastomosis using an interventional technique with angiography was eventually performed on postoperative day 73. Therefore, it is considered that because PVA is associated with severe postoperative portal hypertension, closure of the arterio-portal shunt should be performed as soon as possible on diagnosing portal hypertension

ANESTHESIA FOR RADICAL SURGERY OF NEONATAL CONGENITAL DIAPHRAGMATIC HERNIA USING CONTINUOUS ADMINISTRATION OF FENTANYL AND HIGH-FREQUENCY OSCILLATORY VENTILATION

Patients with congenital diaphragmatic hernia were managed using continu- ous administration of fentanyl and high-frequency oscillation during the perioperative period. Fentanyl was continuously administered at 0.076 μg/kg/min and initial settings for high-frequency oscillation were : fraction of inspired oxygen=0.75, frequency=15 Hz, stroke volume=6.7 ml/kg, mean airway pressure=19 cmH₂O. All the patients were administered dopamine and dobutamine for hemodynamic stability. Their operations were completed with no problem