Takaharu Oue

Long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal reconstruction

PURPOSE The aim of this study was to evaluate the long-term outcomes of four patients with tracheal agenesis who underwent airway and esophageal/alimentary reconstruction. MATERIALS AND METHODS We reviewed the medical records of four long-term survivors of tracheal agenesis and collected the following data: age, sex, type of tracheal agenesis, method of reconstruction, nutritional management, and physical and neurological development. RESULTS The patients consisted of three boys and one girl, who ranged in age from 77 to 109months. The severity of their condition was classified as Floyds type I (n=2), II (n=1), or III (n=1). Mechanical respiratory support was not necessary in any of the cases. Esophageal/alimentary reconstruction was performed using the small intestine (n=2), a gastric tube (n=1), and the esophagus (n=1). The age at esophageal reconstruction ranged from 41 to 55months. All of the cases required enteral nutrition via gastrostomy. Three of the patients were able to swallow a small amount of liquid and one was able to take pureed food orally. The physical development of the subjects was moderately delayed-borderline in childhood. Neurological development was normal in two cases and slightly delayed in two cases. CONCLUSIONS None of the long-term survivors of tracheal agenesis required the use of an artificial respirator, and their development was close to normal. Future studies should aim to elucidate the optimal method for performing esophageal reconstruction to allow tracheal agenesis patients to achieve their full oral intake.

Treatment and ovarian preservation in children with ovarian tumors

BACKGROUND/PURPOSE Ovarian preservation is desirable in children with ovarian tumors. However, the diagnostic and treatment strategies are heterogeneous. The aim of this study was to investigate the management and preservation of ovarian tissue in order to identify the factors associated with ovarian preservation. METHODS Thirty-seven patients (41 ovaries) were surgically treated for ovarian tumors. Four cases were bilateral. The data on the patient symptoms at presentation, imaging, treatment, outcome, pathology, and status of ovarian preservation were retrospectively analyzed for each patient. RESULTS Histological examinations revealed 25 mature teratomas, 5 immature teratomas, 5 cystadenomas, 2 dysgerminomas, and other tumors. Ovarian torsion occurred in 16 ovaries (39%). Ovary-sparing surgery was performed in 22 ovaries (53.7%). Successful ovarian preservation was significantly associated with a smaller tumor size, benign pathology, and a lower degree of torsion (P<0.01). CONCLUSIONS Because the prognosis was favorable in most cases, the preservation of fertility and gonadal function should be a goal in the surgical treatment of ovarian tumors. We recommend ovary-sparing surgery as the first-line treatment for all pediatric ovarian tumors other than those that are preoperatively diagnosed as being malignant and those in which emergent surgical intervention is indicated owing to the suspicion of ovarian torsion.

Primary renal neuroblastoma with metastasis and matrix metalloproteinase-14 expression

We herein report the rare case of a 4‐year–5‐month‐old boy who presented with primary renal neuroblastoma. The patient developed repeated lung and liver metastatic recurrences, but, following a combination of chemotherapy, radiation therapy and aggressive surgical resection, the patient is now in remission. To investigate the pathogenesis of lung metastasis, immunohistochemistry was performed for matrix metalloproteinase‐9 and ‐14 (MMP‐9 and MMP‐14), molecular markers of invasion, metastasis and angiogenesis in neuroblastoma. In the present case, MMP‐9 expression was not observed, but MMP‐14 expression was detected in the primary lesion and was more highly expressed in the metastatic lesion compared with the primary one. Given the MMP‐14 staining in other cases, expression of MMP‐14 may be associated with the aggressiveness of the tumor. This suggests that selected clones with high MMP‐14 expression in the primary tumor might metastasize and form MMP‐14‐rich lesions.

Image-based surgical risk factors for Wilms tumor

PurposeThe standard treatment for Wilms tumor (WT) is primary resection. However, in cases with unresectable tumor or tumor spillage, which are considered to have high surgical risks, more intensive chemotherapy and radiotherapy are required. In the present study, we retrospectively analyzed preoperative image parameters to identify factors associated with surgical risks.MethodsTwenty-nine patients with WT were enrolled in this study. Data on various preoperative image parameters, such as tumor size, tumor volume, displacement of great vessels, and contralateral extension of the tumor were collected, and their relationship with surgical factors, including operative time, hemorrhage, tumor spillage, and unresectability were analyzed.ResultsPatients with unresectable tumor or with tumor spillage (surgical high-risk group) more frequently demonstrated displacement of great vessels and contralateral tumor extension. Operative time and blood loss were also significantly related to tumor size, area, volume, displacement of great vessels and contralateral extension.ConclusionBesides tumor size, displacement of great vessels and contralateral extension were significantly associated with surgical risks. These factors are easily determined using CT images and are, therefore, useful to decide whether preoperative chemotherapy should be started instead of primary tumor resection for large localized WTs.

Outcome of renal tumors registered in Japan Wilms Tumor Study-2 (JWiTS-2): A report from the Japan Children's Cancer Group (JCCG)

Japan Wilms Tumor Study‐2 (JWiTS‐2) mandated central pathology review for all case registrations. The study aimed to compare the outcomes of patients with unilateral Wilms tumor enrolled on the JWiTS‐1 and JWiTS‐2 trials.

Umbilical appearance and patient/parent satisfaction over 5 years of follow-up after umbilical hernia repair in children

BACKGROUND/PURPOSE We evaluated the long-term umbilical appearance and patient/parent satisfaction at follow-up after umbilical hernia repair (UHR). METHODS In a retrospective review of 128 children who underwent UHR and were followed up for over 5 years, satisfaction was assessed using e-mailed questionnaires and attached photographs of the umbilicus. RESULTS The survey response rate was 80.3% by parents, 79.5% by patients, and 72.1% using photographs. The median follow-up period was 7.6 (range, 5.3-10.1) years. The satisfaction rate was 78.9% among parents and 91.5% among patients; however, eight patients (8.5%) reported dissatisfaction with the results. The main reason for dissatisfaction was the shallow depth of the umbilicus (parents, 10/20; patients, 5/8). During the follow-up period, satisfaction increased in 14 cases (14.7%) and decreased in 10 cases (10.5%). Superior hooding was considered an ideal shape for the umbilicus. Concomitant laparoscopic surgery and higher age at surgery were significantly associated with dissatisfaction in parents (P = 0.045) and patients (P = 0.046), respectively. Large defect size was significantly associated with decreased satisfaction during the follow-up period (P = 0.030). CONCLUSION E-mail surveys are useful for long-term follow-up after UHR. Patients with these above risk factors should receive long-term follow-up with careful attention to patient satisfaction. LEVEL OF EVIDENCE Type of study: prognosis study, level II.

Biopsy of Tumor

Various biopsy procedures such as core needle biopsy, laparoscopic/thoracoscopic biopsy, and excisional/incisional open biopsy are selected according to the purpose, tumor site, and condition of the patient. Tumor biopsy is performed not only for pathological diagnosis but for evaluation of the chemotherapy effects, confirmation of the remaining of the viable malignant cells, and analysis of tumor biology. Biopsy specimens should be divided and appropriately stored for each analysis, for example, formalin fixation-paraffin embedding for histological diagnosis, sterile tissue culture medium for chromosomal analysis, and snap frozen deep freezer storage for DNA/RNA analysis. After biopsy, confirmation of hemostasis is most important.