Value of the Sentinel Node Procedure in Pediatric Extremity Rhabdomyosarcoma: A Systematic Review and Retrospective Cohort Study.

BACKGROUND: Our aim is to show whether the sentinel node procedure (SNP) is recommendable for pediatric patients with extremity rhabdomyosarcoma (RMS). Lymph node metastases are an important prognostic factor in pediatric patients with extremity RMS. Accurate nodal staging is necessary to treat the patient accordingly. An alternative to the current recommended lymph node sampling is the sentinel node procedure (SNP). METHODS: A systematic review was performed summarizing all published cases of SNP in addition to 13 cases from our hospital and 8 cases from two other hospitals that have not been published before. RESULTS: For all patients (n = 55), at least one SLN was identified, but the SNP technique used was not uniform. The SNP changed the nodal classification of eight patients (17.0%) and had a false-negative rate of 10.5%. CONCLUSIONS: The SNP is recommendable for pediatric patients with extremity RMS. It can change lymph node status and can be used to sample patients in a more targeted way than nodal sampling alone. Therefore, we recommend use of the SNP in addition to clinical and radiological nodal assessment for pediatric patients with extremity RMS.

The results of concentration of care: Surgical outcomes of neuroblastoma in the Netherlands.

INTRODUCTION: In the Netherlands pediatric oncological care for solid tumours is concentrated in one centre since November 2014. One of the most frequently diagnosed solid non-brain tumours in children is the neuroblastoma. Results of surgical treatment of neuroblastoma since the start of this centralization are presented and compared to a historic cohort. METHODS: The new national cohort of neuroblastoma (n = 111) consists of all consecutive patients treated between January 1st, 2015 and April 1st, 2021. The historic neuroblastoma cohort consists of all operated neuroblastoma patients in the Netherlands between 1998 and 2014 (n = 244). Intra-operative complications and surgical outcome were registered. Post-operative complications were divided in short (<30 days after surgery) and long term (>30 days). The severity of complications was graded using the Clavien Dindo Classification (CDC) system. RESULTS: Intraoperative outcomes showed significant differences in favour of the new cohort with less blood loss (p < 0.001), fewer vascular complications (p < 0.001) and shorter duration of surgery (p < 0.001). Short term complications were comparable in numbers, but significantly more patients had CDC grade 3/4/5 complications in the historic cohort (p = 0.005). Long term complications did not differ. Estimated overall survival showed a better survival in the new cohort (log rank 0.022). CONCLUSION: Centralization of care for neuroblastoma patients has led to a significant improvement of both intraoperative outcomes and short term complications.

Incidence, severity and outcome of central line related complications in pediatric oncology patients; A single center study.

BACKGROUND: Central venous access device (CVAD)-related complications are associated with high morbidity rates. This study was performed to underline the importance of CVAD-complication prevention and treatment. METHODS: An audit of practice of CVAD-related complications in pediatric oncology patients receiving a CVAD between January 2015 and June 2017 was performed. CVADs included were totally implantable venous access ports (TIVAPs), Hickman-Broviac® (HB), nontunneled, and peripherally inserted CVADs. RESULTS: A total of 201 children, with 307 CVADs, were analyzed. The incidence rates per 1000 CVAD-days for the most common complications were 1.66 for malfunctions, and 1.51 for central line-associated bloodstream infections (CLABSIs). Of all CVADs inserted, 37.1% were removed owing to complications, of which 45.6% were owing to CLABSIs. In 42% of the CLABSIs, the CLABSI could be successfully cured with systemic antibiotic treatment only. Of all included patients, 5.0% were admitted to the intensive care unit owing to CLABSI. The HB-CVAD compared to the TIVAP was a risk factor for CVAD-related complications, CLABSIs and dislocations in particular. CONCLUSIONS: The incidence of CVAD-related complications is high. Research on the prevention and treatment of CVAD-related complications in pediatric oncology patients should be a high priority for all health care professionals. TYPE OF STUDY: Prognosis study (retrospective). LEVEL OF EVIDENCE: Level II.

An unusual presentation of Kabuki syndrome: clinical overlap with CHARGE syndrome.

Kabuki syndrome is a rare genetic disorder characterized by intellectual disability and multiple congenital anomalies, including short stature, peculiar facial appearance, skeletal anomalies, a variety of visceral malformations and abnormal dermatoglyphic patterns. We describe a case of Kabuki syndrome presenting with atypical features, consisting of bilateral microphthalmia, coloboma, anal atresia and panhypopituitarism, showing considerable phenotypic overlap with CHARGE syndrome. This report demonstrates that clinical follow-up and molecular genetic testing can be useful for establishing the correct diagnosis.

Diagnosis and treatment of lymph node metastases in pediatric rhabdomyosarcoma in the Netherlands: a retrospective analysis.

BACKGROUND: In pediatric rhabdomyosarcoma (RMS), evaluation of lymph node involvement (N1) is an important staging aspect, but difficult to assess. The aim of our study was to evaluate the assessment of lymph node infiltration and impact on outcome in N1 RMS patients. METHODS: We identified 277 non-metastatic RMS patients diagnosed and treated between 1990 and 2008. Patients with recorded N1 disease were evaluated for their diagnostic procedures and outcome. RESULTS: In 13.7% N1 status was reported. In 19 of 34 N1 patients, lymph node biopsies were performed for histologically confirmation. Different treatment modalities were used to treat lymph node metastases. In total 23 of 31 patients received local treatment of the node (11/23 RT, 4/23 surgery, and 8/23 both). All patients received chemotherapy. Lymph node relapse occurred in 7 of 31 patients who were treated with one or two modalities. Only 1 (14%) of 8 patients treated with three modalities relapsed. In N0 patients 10 (4.2%) of 239 had a regional lymph node relapse, and 9 of 10 died. CONCLUSION: Lymph node metastases are an essential part of staging. Node positivity contributes to relapse of disease. Nodal relapse is also associated with a high mortality rate. These data imply that nodal assessment needs to be optimal and standardized for improved staging.