A case of dumbbell-shaped accessory scrotum with concomitant lipoma.

BACKGROUND: Accessory scrotum is a congenital scrotal anomaly that is usually located anterior to the anus and frequently presents with a lipoma in a bead-like shape. Herein, we present an unusual case of an accessory scrotum with a lipoma connected by a narrow stalk and located posterior to the anus. CASE PRESENTATION: A 1-month-old boy was referred to our hospital for a perineal mass present at birth. He was born at 37 weeks and 2 days, with a birth weight of 2962 g. No abnormalities occurred during the perinatal period, and the birth was uneventful. The mass had an unusual shape, comprising two masses connected by a narrow stalk. The base of the mass was posterior to the anus and was connected to the rectal mucosa. The proximal mass was elastic and soft without skinfolds, whereas the distal mass was elastic and soft with a scrotum-like skinfolds. Magnetic resonance imaging showed no spina bifida. High-intensity adipose tissues in both masses and low-intensity vessels or fibrous stroma in cord-like structures between the two masses were found on T2-weighted images. At 3 months of age, the patient underwent resection in the prone jackknife position. No tumorous lesions were connected to the mass on the rectal and coccyx sides, and the mass was completely removed, preserving the anal sphincter. Histologically, the distal mass had characteristics of a scrotum, whereas the proximal mass was exclusively a lipoma. The connecting stalk had normal skin structures and a blood vessel with parallel-running nerve bundles. The postoperative course was uneventful, and the patient was discharged on postoperative day 6. CONCLUSIONS: This case of accessory scrotum was unusual in its location and the presence of a stalk connecting the accessory scrotum and lipoma. The mechanism underlying accessory scrotum development remains unclear, and our report may impact the discourse regarding the embryological development of the accessory scrotum.

Inhibition of cIAP1 in the effective suppression of chemotherapy­resistant hepatoblastoma.

Cellular inhibitor of apoptosis protein­1 (cIAP1) is a key regulator of programmed cell death and is known to be associated with chemotherapeutic resistance. The present study aimed to investigate the antitumor efficacy of birinapant, a novel selective inhibitor of cIAP1, against cisplatin (CDDP)­resistant hepatoblastoma (HB) cells. Western blot analysis was used to investigate the antitumor effect of birinapant on cIAP1 expression in Huh6 cells at the protein level. A WST­8 assay was performed to evaluate the tumor growth inhibitory effect of birinapant on the human HB cell lines, Huh6 and HepG2. Huh6 cells were exposed to CDDP and/or birinapant in order to confirm tumor growth inhibition. The antitumor efficacy of birinapant plus CDDP combination therapy was significantly higher than that of CDDP monotherapy in a dose­dependent manner (P=0.035). The study also investigated the antitumor efficacy of birinapant plus CDDP combination therapy in an established xenograft model of SCID mice. Compared with CDDP monotherapy, birinapant combined with CDDP showed better inhibition of tumor growth (P=0.121). It was observed that the mRNA expression of cIAP1 in tumors was significantly enriched in the CDDP monotherapy group compared with that in the untreated group. Furthermore, immunohistochemical staining was performed to compare cIAP1 expression in pre­ and post­chemotherapy specimens in patients with HB, and a significant increase was observed in the post­chemotherapy specimens (P<0.001). CDDP­resistant Huh6 (Huh6­CDDPR) cells were also established following repeated exposure to CDDP. Birinapant was substantially more effective against the Huh6­CDDPR cells than against the Huh6 wild­type cells. Taken together, these findings suggest that repeated exposure to CDDP enhances cIAP1 expression in HB cells and that birinapant is a promising therapeutic drug for CDDP­resistant HB.

Clinical outcomes of surgical management for rare types of progressive familial intrahepatic cholestasis: a case series.

BACKGROUND: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of genetic autosomal recessive diseases that cause severe cholestasis, which progresses to cirrhosis and liver failure, in infancy or early childhood. We herein report the clinical outcomes of surgical management in patients with four types of PFIC. CASE PRESENTATION: Six patients diagnosed with PFIC who underwent surgical treatment between 1998 and 2020 at our institution were retrospectively assessed. Living-donor liver transplantation (LDLT) was performed in 5 patients with PFIC. The median age at LDLT was 4.8 (range: 1.9-11.4) years. One patient each with familial intrahepatic cholestasis 1 (FIC1) deficiency and bile salt export pump (BSEP) deficiency died after LDLT, and the four remaining patients, one each with deficiency of FIC1, BSEP, multidrug resistance protein 3 (MDR3), and tight junction protein 2 (TJP2), survived. One FIC1 deficiency recipient underwent LDLT secondary to deterioration of liver function, following infectious enteritis. Although he underwent LDLT accompanied by total external biliary diversion, the patient died because of PFIC-related complications. The other patient with FIC1 deficiency had intractable pruritus and underwent partial internal biliary diversion (PIBD) at 9.8 years of age, pruritus largely resolved after PIBD. One BSEP deficiency recipient, who had severe graft damage, experienced recurrence of cholestasis due to the development of antibodies against BSEP after LDLT, and eventually died due to graft failure. The other patient with BSEP deficiency recovered well after LDLT and there was no evidence of posttransplant recurrence of cholestasis. In contrast, recipients with MDR3 or TJP2 deficiency showed good courses and outcomes after LDLT. CONCLUSIONS: Although LDLT was considered an effective treatment for PFIC, the clinical courses and outcomes after LDLT were still inadequate in patients with FIC1 and BSEP deficiency. LDLT accompanied by total biliary diversion may not be as effective for patients with FIC1 deficiency.

Computed tomography-guided marking using a dye-staining method for preoperative localization of tiny pulmonary lesions in children.

PURPOSE: It is important to confirm the existence of pulmonary metastases in pediatric patients with malignancies. Therefore, we aimed to investigate if computed tomography CT-guided marking is a feasible and safe method for the identification and resection of tiny pulmonary lesions in pediatric cancer patients. METHODS: We retrospectively reviewed the medical records of pediatric cancer patients who underwent CT-guided marking procedures in our institutions between Jan 2011 and Apr 2020. After 2015, these procedures were combined with an indocyanine green (ICG) navigation-guided surgery for hepatoblastoma cases. RESULTS: We targeted a total of 22 nodules in 12 patients. Of these, marking was successful in 18 (81.8%) nodules, 10 of which contained viable malignant cells. Complications caused by the marking procedures included mild pneumothorax and mild atelectasis in two patients, respectively. Of the eight resected nodules in patients with hepatoblastoma, four were ICG-positive and contained viable malignant cells. Two additional ICG-positive nodules, which were unidentified before surgery, were observed intraoperatively. CONCLUSION: CT-guided marking is a feasible and safe method that can be used to identify and resect tiny pulmonary lesions in pediatric cancer patients. An ICG navigation-guided surgery is useful when combined with CT-guided marking, particularly in hepatoblastoma cases.

Safety and Feasibility of Laparoscopic Resection of Neuroblastoma Without Image-Defined Risk Factors Performed by Pediatric Surgical Trainees: A Multicenter Comparison Study.

Introduction: Laparoscopic resection has gradually been adopted for neuroblastoma patients; however, some authors reported that, due to its technically demanding procedures, this operation should be performed only by highly experienced surgeons. The aim of this study was to evaluate the safety and feasibility of laparoscopic resection of abdominal neuroblastoma by pediatric surgical trainees. Subjects and Methods: In this multicenter, retrospective study, including 18 children with abdominal neuroblastoma indicated for 19 laparoscopic resections (1 with bilateral neuroblastomas) from 1999 to 2018, the clinical data were retrospectively reviewed and compared between trainee and attending surgeons. Results: None of the cases had image-defined risk factors (IDRFs) at surgery. All patients successfully underwent complete laparoscopic resection without blood transfusion, open conversion, or intraoperative or postoperative complications. Of the 19 cases, 6 were performed by pediatric surgical trainees under the guidance of attending surgeons, and 13 were performed by attending surgeons. With comparable tumor, largest diameter, operative time, and bleeding amount were not significantly different between the two groups. In the trainee surgeon group, one local recurrence occurred at a secondary resection site in the bilateral neuroblastoma patient with Stage L2. Conclusions: Laparoscopic resection of neuroblastoma could be safe and feasible when limited to absent IDRFs at surgery by pediatric surgical trainees under the guidance of experienced attending surgeons, as well as by attending surgeons.

Thoracoscopic Costal Cartilage Excision Combined with the Nuss Procedure for Patients with Asymmetrical Pectus Excavatum.

Background and Aims: We performed thoracoscopic costal cartilage excision (TCCE) combined with the Nuss procedure to correct asymmetrical pectus excavatum (PE). We reviewed the efficacy of combined TCCE and Nuss procedure for asymmetric PE. Patients and Methods: Overall, 8 patients with asymmetrical PE underwent TCCE with the Nuss procedure. The Haller index, asymmetry index, and angle of sternal rotation were calculated using preoperative computed tomography. The procedure was performed using bilateral 2.5-cm incisions at the same level of the deepest chest wall depression. The most depressed three to four costal cartilages were partially resected through a right mini-thoracotomy. Subsequently, one or two titanium bars were implanted and secured with stabilizers. The cosmetic outcome was evaluated on the following four ratings: excellent, good, fair, and failure ( = recurrence). Results: The median age at surgery was 14.5 years (8-20 years). The number of bars was one in 3 cases and two in 5 cases. The preoperative Haller index, asymmetry index, and angle of sternal rotation were 4.3 (3.5-5.9), 1.15 (1.04-1.26), and 21.5° (15°-31°), respectively; 2 patients had scoliosis before the Nuss procedure. Complications included surgical site infection and hemothorax. Median follow-up time was 25.5 months (3-63). Bars were removed in 3 patients, 3 years postoperatively. Cosmetic results were excellent, 4; good, 2; fair, 1; failure, 1. Both patients with scoliosis had poor outcomes (fair, 1; failure, 1). Conclusions: Combined TCCE with Nuss procedure is considered safe and effective for patients with asymmetrical PE. Careful long-term follow-up is required, especially in cases with scoliosis.

Potential role of the PD-L1 expression and tumor-infiltrating lymphocytes on neuroblastoma.

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.

The feasibility and safety of single-incision totally extraperitoneal inguinal hernia repair after previous lower abdominal surgery: 350 procedures at a single center.

PURPOSE: To evaluate the feasibility and safety of single-incision laparoscopic surgery for totally extraperitoneal inguinal hernia repair (SILS-TEP) with previous lower abdominal surgery (PLAS). METHODS: A retrospective analysis of 350 patients undergoing SILS-TEP for a primary inguinal hernia from January 2012 to December 2015 at Osaka Police Hospital was performed, and the outcomes of the patients with and without PLAS were compared. RESULTS: SILS-TEP was performed in 84 patients with PLAS and 266 patients without PLAS. Appendectomy was the most common previous operative procedure. There were more patients with an ASA score of ≥3 in the PLAS group than in the control group (p < 0.05). The mean operative time, and the rates of conversion and postoperative complications were comparable between the two groups. There were no cases of recurrence in either group. CONCLUSIONS: SILS-TEP could be safely performed in patients with PLAS and achieved better cosmetic outcomes than conventional laparoscopic surgery.

Single-incision totally extraperitoneal inguinal hernia repair is safe and feasible in elderly patients: A single-center experience of 365 procedures.

The aim of this study was to evaluate the feasibility and safety of SILS for totally extraperitoneal inguinal hernia repair in elderly patients. A retrospective analysis of 365 patients who underwent of SILS for totally extraperitoneal inguinal hernia repair from January 2012 to November 2015 at Osaka Police Hospital was performed, and the outcomes of patients aged <80 years and those aged ≥80 years were compared. There was a greater proportion of patients with an ASA score ≥3 among those ≥80 years than among those <80 years. The mean operative time for unilateral inguinal hernia was 94 min in patients <80 years and 98 min in patients ≥80 years. The mean operative time for bilateral inguinal hernia was 133 min in patients <80 years and 130 min in patients ≥80. Intraoperative bleeding was minimal in all patients. Conversion to a different operative procedure occurred in 3% (10/322) of patients <80 years and in 5% (2/43) of patients ≥80 years (P = 0.6). The mean postoperative hospital stay was 2.2 days for patients <80 years and 2.2 days for patients ≥80 years. The mean follow-up period 21 ± 14 months (range, 3-50 months) for patients <80 years and 17 ± 14 months (range, 3-50 months) for patients ≥80 years (P = 0.3). Postoperative complications were seen in 12% (38/322) of patients <80 years and in 14% (6/43) of patients ≥80 years (P = 0.7). A seroma was seen in 9% (28/322) of patients <80 years and in 12% (5/43) of patients ≥80 years (P = 0.6). A wound infection occurred in 2% (8/322) of patients <80 years. These seromas and wound infections were managed conservatively. Pulmonary embolism was seen in one patient <80 years (0.3%). There was no mortality or recurrence in either group. SILS for totally extraperitoneal inguinal hernia repair has good cosmesis and can be performed in elderly patients with acceptable morbidity and mortality.

Single-incision laparoscopic surgery as a teaching procedure: a single-center experience of more than 2100 procedures.

PURPOSE: To confirm the safety and feasibility of single-incision laparoscopic surgery (SILS) and to compare the patient outcomes of single-incision laparoscopic surgery for laparoscopic appendectomy (SILS-LA) performed by resident doctors vs. staff surgeons. METHODS: We performed a retrospective analysis of patients who underwent SILS between May, 2009 and May, 2015 at Osaka Police Hospital. RESULTS: We analyzed 2172 patients. The operations performed consisted of cholecystectomy (n = 598), appendectomy (n = 202), inguinal hernia repair (n = 301), colorectal surgery (n = 673), and gastrectomy (n = 398). SILS was performed safely for a wide range of procedures with acceptable conversion and perioperative complication rates. The resident doctors in our department operated safely on 77 % (156/202) of patients undergoing SILS-LA. The staff surgeons operated on more elderly patients and patients with complicated appendicitis than did the resident doctors. The operative outcomes of the resident-performed SILS-LAs were better than those of the staff surgeons, although there was a patient selection bias. CONCLUSIONS: SILS seems safe and feasible for a wide range of procedures. Based on our findings, we believe that SILS-LA could be a useful teaching procedure for resident doctors to perform on selected patients, under the guidance of an experienced staff surgeon.

Single-incision totally extraperitoneal inguinal hernia repair as a teaching procedure: one center's experience of more than 300 procedures.

PURPOSE: To evaluate resident doctors' proficiency in performing single-incision laparoscopic surgery for totally extraperitoneal inguinal hernia repair (SILS-TEP), and assess patient outcomes by comparing procedures performed by resident surgeons vs. those performed by staff surgeons. METHODS: We analyzed retrospectively 301 patients who underwent SILS-TEP between January 2011 and May 2015 at Osaka Police Hospital. RESULTS: The mean operative times for unilateral and bilateral hernia repairs in the resident-surgeon and the staff-surgeon groups were 99 vs. 88 min, respectively (p < 0.05), and 130 vs. 137 min, respectively. There was no significant difference in the incidence of conversion to a different procedure between the groups. The mean postoperative hospital stay was 2.0 days for patients from the resident-surgeon group vs. 2.8 days for those from the staff-surgeon group (p < 0.05). Seromas and wound infections developed in 8 % (12/148) of patients from the resident-surgeon group vs. 12 % (19/153) of those from the staff-surgeon group. No other major complications or hernia recurrence were noted in either group. CONCLUSIONS: SILS-TEP was performed safely, with low morbidity and no recurrence, by the resident surgeons under appropriate guidance by staff surgeons.

[Curative Resection for Chemorefractory Advanced Gastric Cancer with Pancreatic Invasion by Pancreatoduodenectomy - A Case Report].

A 37-year-old woman was diagnosed with advanced gastric cancer. Pancreatic invasion of the tumor and positive cytology from peritoneal washing was found on staging laparoscopy. The patient received chemotherapy consisting of S-1 and CDDP, but the tumor size increased after 5 courses. The patient then received 2 courses of second line chemotherapy consisting of biweekly CPT-11; however, the tumor progressed and caused stenosis of the stomach. After gastrojejunostomy for the stenosis, negative cytology of intraperitoneal lavage was found. The patient underwent pancreatoduodenectomy with right hemicolectomy and wedge resection of the portal vein as curative resection. The patient was alive without recurrence 10 months after the surgery.

[A Case of Pathological Complete Response after SOX Chemotherapy in Advanced Gastric Cancer].

The patient was a 78-year-old woman. She was referred to our hospital and diagnosed with advanced gastric cancer with para-aortic lymph node(#16)metastasis. She received the SOX regimen(L-OHP 100mg/m2)chemotherapy and developed fatigue, anorexia, and neutropenia. After 4 courses of the SOX regimen, the #16 metastasis was reduced remarkably. A curative operation was performed and histological evaluation of the primary and lymphatic lesion after chemotherapy showed Grade 3 findings. The SOX regimen is tolerable in the outpatient clinic and is useful as part of multidisciplinary treatment for advanced gastric cancer.

Laparoscopic treatment of pediatric hydrocele and the evaluation of the internal inguinal ring.

BACKGROUND: There are few reports of the laparoscopic findings of the internal inguinal ring (IIR) in patients with hydrocele. The purpose of this study was to assess the safety and efficacy of laparoscopic percutaneous extraperitoneal closure (LPEC) for hydrocele in comparison with that of open repair (OR) and compare the findings of the IIR between cases of hydrocele and inguinal hernia (IH). MATERIALS AND METHODS: We analyzed 69 consecutive patients with hydrocele who underwent surgery at our institution between April 2009 and February 2014. The patients were divided into two groups (LPEC and OR) according to the procedure. Age, length of operation/anesthesia, and complications were compared. Concerning the findings of the IIR, we classified the features into three categories, as follows: Type 1, flat; Type 2, narrow patent processus vaginalis (PPV) with a peritoneal veil; and Type 3, widely opened PPV. We then compared these findings between the cases of hydrocele and IH treated with LPEC during the study period. RESULTS: Among a total of 69 patients, 40 underwent LPEC, and 29 underwent OR. There were no significant differences in the length of operation/anesthesia and complications. No recurrences were observed in either group. The findings of the IIR were mostly classified as Type 2 (59.1%) among the cases of hydrocele and Type 3 (92%) among the cases of IH. CONCLUSIONS: LPEC is a safe and effective procedure for treating hydrocele. The findings of the IIR differ between cases of hydrocele and IH.