Pediatric inflammatory myofibroblastic tumor of the bladder with ALK-FN1 fusion successfully treated by alectinib.

An inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm characterized by the proliferation of myofibroblasts and inflammatory cell infiltration. Although radical resection is the only established treatment strategy for IMT, it can cause functional disorders when vital organs are affected. We describe a case of pediatric IMT of the bladder with FN1-ALK (fibronectin 1-anaplastic lymphoma kinase) fusion. Radical resection might lead to urinary disturbance due to the large tumor size at diagnosis. However, the tumor was successfully treated with alectinib, a second-generation ALK inhibitor, followed by transurethral resection of the bladder tumor without any complications.

A Rare Case of Immature Sacrococcygeal Teratoma With Lymph Node Metastasis in a Neonate.

This is the first report of an immature sacrococcygeal teratoma with inguinal lymph node metastasis, providing the histologic transformation of an immature teratoma in association with chemotherapy. Incomplete tumor resection with coccygectomy was performed, and the histopathologic diagnosis was a grade 3 immature teratoma. Following the initial surgery, the residual tumors enlarged and the tumors metastasized to the inguinal lymph node, demonstrating immature teratoma without yolk sac tumor components. Although the tumor markers normalized after chemotherapy, the residual tumors had enlarged significantly. Therefore, a complete resection of the residual tumors was performed, and they were found to be mature teratomas.

Clostridium tertium Peritonitis and Bacteremia in a Neonate With Congenital Intestinal Atresia: A Case Report.

We report a 1-day-old girl who was affected by peritonitis and bacteremia caused by Clostridium tertium following perforation of congenital intestinal atresia. Splenic infarction was also suspected during C. tertium bacteremia. C. tertium was identified by using mass spectrometry and 16S rRNA sequencing. This patient was successfully treated with emergency laparotomy and broad-spectrum antibiotics.

Long-term functional outcome of sacrococcygeal teratoma after resection in neonates and infants: a single-center experience.

PURPOSE: This study aimed to evaluate the incidence and factors associated with long-term functional outcomes of sacrococcygeal teratoma (SCT) after resection in neonates and infants. METHODS: Twenty-nine patients with a minimum of 3 years of follow-up who underwent resection and were histologically diagnosed with SCTs between 1982 and 2017 at our institution were included. RESULTS: The median age at the time of the study was 10.0 years. Functional disorders occurred after surgery in 6 (20.7%) patients. Anorectal dysfunction, urologic dysfunction, and lower-extremity motor disorders occurred in 6 (20.7%), 4 (13.8%), and 3 (10.3%) patients, respectively. One patient with all three types of functional disorders developed intestinal perforation due to ileus and died of sepsis at 13 years of age. The overall mortality rate after tumor resection was 3.4%. The patients who developed functional disorders presented a low 1-min Apgar score, larger tumors requiring abdominosacral resection, surgical injury to the pelvic organs, and immature or malignant histological findings. CONCLUSION: Although the mortality rate was low, the long-term rate of functional disorders after SCT resection was approximately 20%. SCT patients with large tumors, surgical injury to the pelvic organs, and immature or malignant histological findings require thorough follow-up.

Clinical outcomes and protocol for the management of isolated congenital diaphragmatic hernia based on our prenatal risk stratification system.

BACKGROUND/PURPOSE: The aim of this study was to evaluate our prenatal risk stratification system for risk-adjusted management in fetuses with isolated congenital diaphragmatic hernia (CDH). METHODS: Ninety-four infants prenatally diagnosed with isolated CDH treated between 1998 and 2017 at our institution were included in this retrospective single-center cohort study. RESULTS: The patients were prenatally classified into four risk groups: Group A (n = 54), which consisted of infants with neither liver-up nor a contralateral lung-to-thorax transverse area (L/T) ratio <0.08. The infants in group A were divided into two subgroups: Group A-1 (n = 24) consisted of mild conditions; and Group A-2 (n = 30) consisted of severe conditions; Group B (n = 23), which consisted of infants with either liver-up or L/T ratio <0.08; and Group C (n = 17), which consisted of infants with both liver-up and L/T ratio <0.08. The rates of survival to discharge in Groups A-1, A-2, B, and C were 100.0%, 100.0%, 87.0%, and 58.8%, respectively. The rates of intact discharge were 91.7%, 90.0%, 52.1%, and 23.5%, respectively. CONCLUSIONS: Our prenatal risk stratification system demonstrated a significant difference in the severity of postnatal status and clinical outcomes between the groups. STUDY TYPE: Case Series, Retrospective Review. LEVELS OF EVIDENCE: LEVEL IV.

Effective kissing stent to severe stenosis of the superior mesenteric artery replacing the common hepatic artery.

BACKGROUND: Endovascular therapy (ET) for chronic mesenteric ischemia (CMI) is a effective treatment to relieve the symptoms, such as postprandial abdominal pain, food fear, and progressive weight loss. CMI is not known to be caused by rare anatomical variation of severe stenosis of the superior mesenteric artery (SMA), with replaced the common hepatic artery to the SMA. The treatment of such a rare anatomical variation using ET technique has not been discribed. ET with kissing stent technique can be applied to the CMI accompanied with a rare anatomical variation. CASE PRESENTATION: An 80-year-old woman presented with a history of intermittent, severe epigastric pain. Over the preceding 5 months, she had less severe and self-resolving epigastric pain 15-30 min after every meal. Abdominal computed tomography (CT) showed severe calcification of the SMA origin and bubble-like intramural gas of the small bowel with the contrasted wall pneumoperitoneum. As the patient did not have peritonitis, a conservative approach was used. Angiography performed after symptom resolution showed severe stenosis of the SMA origin with calcification, and the SMA had replaced the common hepatic artery. ET with the kissing stent technique, namely stenting to the SMA and common hepatic artery, was successfully performed and relieved the patient's symptoms. CONCLUSIONS: CMI cause the symptoms of Pneumatosis intestinalis (PI) and pneumoperitoneum. Severe stenosis of the SMA origin replacing the common hepatic artery is a rare anatomic variation, which can cause CMI symptoms. ET with a kissing stent is the effective treatment option for the mesenteric artery stenosis accompanied with such rare anatomical variation.

Needlescopic video-assisted wedge resection combined with the subcostal trans-diaphragmatic approach for undetermined peripheral pulmonary nodules.

BACKGROUND: Reduced mortality from lung cancer by computed tomography (CT) screening facilitates the use of video-assisted thoracic surgery (VATS) lung wedge resection to obtain a definite diagnosis and to treat tiny nodules. The authors evaluated their initial experience using novel needlescopic VATS wedge resection combined with the subcostal trans-diaphragmatic (SCTD) approach for managing undetermined peripheral pulmonary nodules. METHODS: Between 2009 and 2012, 35 patients who had 36 operations underwent needlescopic VATS wedge pulmonary resection with the SCTD approach. Preoperative percutaneous CT-guided marking of the nodule was performed. Two 3-mm miniports were placed in the thorax for the thoracoscopic camera and minigrasper. Just anterior to the 10th rib, a 2-cm subcostal incision was made, and a 12- or 15-mm port was placed trans-diaphragmatically into the chest cavity. Wedge resection of the lung was performed with endostaplers introduced through a subcostal port. RESULTS: The median tumor size was 1.1 cm. Localization of the tumor was widely distributed. The mean operation time was 51 min, and the mean blood loss was 4.2 mL. No patients required conversion to thoracotomy, and one patient required conversion to conventional VATS. Additional thoracic ports were placed in five patients, and the needlescopic incision was extended to 15 mm in one patient. The median duration of chest drainage was 1 day. Additional analgesia was not required for 22 patients and was used for less than 1 day for three patients, less than 2 days for seven patients, and less than 3 days for seven patients. The pathologic diagnosis of the nodules was malignant for 28 patients and benign for 8 patients. On postoperative day 7 or at admission, 34 patients were free of postoperative neuralgia. CONCLUSIONS: Needlescopic VATS wedge pulmonary resection combined with the SCTD approach is both safe and feasible and offers the specific advantages of minimal invasiveness and good cosmetic outcomes.