Surgical challenges of excision of retroperitoneal germ cell tumors in children: a single institutional study with literature review

Background: Retroperitoneal germ cell tumors (GCTs) are rare, commonly large tumors, often diagnosed in infancy. Complete surgical resection may pose a serious challenge as encasement of major vessels and organ displacement can lead to perioperative complications. This study aims to illustrate the surgical challenges of excising retroperitoneal GCTs. Results: Nine patients were included: six patients with a teratoma, two patients with a mixed GCT with a yolk sac tumor component, and one patient with a pure yolk sac tumor. Six were males and seven were younger than 1 year of age at time of presentation. In all cases, perioperative vascular or organ-related complications occurred in one patient; this resulted in short bowel syndrome. There was no recurrence or mortality during the follow-up period. Relevant literature is reviewed and described. Conclusions: The anatomical relation of a retroperitoneal GCT to the major vessels and organs is unpredictable. Preoperative radiological evaluation can help to improve the understanding of the vascular anatomy and to plan accordingly. Anteriorly displaced veins may appear insignificant and arteries, such as the superior mesenteric artery may be encased. Preoperative imaging may prevent perioperative complications, leading to safer surgical procedures. Level IV evidence: Therapeutic stud

The diagnostic value of magnetic resonance imaging in differentiating benign and malignant pediatric ovarian tumors

BACKGROUND: The diagnostic workup of ovarian tumors in children and adolescents is challenging because preserving fertility, in addition to oncological safety, is of particular importance in this population. Therefore, a thorough preoperative assessment of ovarian tumors is required. OBJECTIVE: To investigate the diagnostic value of MR imaging in differentiating benign from malignant ovarian tumors in children and adolescents. MATERIALS AND METHODS: We conducted a retrospective study of all children and adolescents age <18 years who underwent MR imaging of ovarian tumors during 2014-2019 at a pediatric specialty center. Two radiologists reviewed all MR imaging. We used pathology reports to define the histological diagnosis. RESULTS: We included 30 girls who underwent MR imaging for an ovarian tumor. Signs indicative for malignancy were tumors with a diameter ≥8 cm, with areas of contrast enhancement, irregular margins, extracapsular tumor growth, and ascites. All benign and malignant ovarian tumors were correctly identified by the radiologists. CONCLUSION: The diagnostic utility of MR imaging in classifying ovarian tumors in children and adolescents as benign or malignant is promising and might aid in defining the indication for ovarian-sparing versus non-ovarian-sparing surgery. We recommend evaluating these tumors with MR imaging prior to deciding on surgical treatment

Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT):Patient Characteristics, Treatment, and Outcome—A Systematic Review

Background: Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare aggressive ovarian malignancy mainly affecting children, adolescents, and young adults. Since the discovery of mutations in the SMARCA4 gene in 2014, SCCOHT has become the subject of extensive investigation. However, international uniform treatment guidelines for SCCOHT are lacking and the outcome remains poor. The aim of this systematic review is to generate an overview of all reported patients with SCCOHT from 1990 onwards, describing the clinical presentation, genetic characteristics, treatment, and outcome. Methods: A systematic search was performed in the databases Embase, Medline, Web of Science, and Cochrane for studies that focus on SCCOHT. Patient characteristics and treatment data were extracted from the included studies. Survival was estimated using Kaplan–Meier’s methodology. To assess the difference between survival, the log-rank test was used. To quantify the effect of the FIGO stage, the Cox proportional hazard regression model was estimated. The chi-squared test was used to study the association between the FIGO stage and the surgical procedures. Results: Sixty-seven studies describing a total of 306 patients were included. The median patient age was 25 years (range 1–60 years). The patients mostly presented with non-specific symptoms such as abdominal pain and sometimes showed hypercalcemia and elevated CA-125. A great diversity in the diagnostic work-up and therapeutic approaches was reported. The chemotherapy regimens were very diverse, all containing a platinum-based (cisplatin or carboplatin) backbone. Survival was strongly associated with the FIGO stage at diagnosis. Conclusions: SCCOHT is a rare and aggressive ovarian cancer, with a poor prognosis, and information on adequate treatment for this cancer is lacking. The testing of mutations in SMARCA4 is crucial for an accurate diagnosis and may lead to new treatment options. Harmonization and international collaboration to obtain high-quality data on diagnostic investigations, treatment, and outcome are warranted to be able to develop international treatment guidelines to improve the survival chances of young women with SCCOHT.</p

Een jongen met tularemie na een modderrace

Background Tularaemia is a rare disease. In Europe it mostly occurs in Scandinavia. Since 2011 more cases are being reported in the Netherlands. Tularaemia may manifest itself in various ways. It is important to take strict precautions during biopsy, drainage and biopsy processing in order to prevent transmission. Case description A 10yearold boy presented to the paediatrician with a left inguinal lymphadenitis. A week before the onset of symptoms he had participated in a children's mud race. Serology and PCR of pus from the lymph node tested positive for Francisella tularensis. Treatment with ciprofloxacin was insufficiently effective, so surgical drainage of the gland was performed under strict isolation conditions. Water from the mud race location contained genetic material from F. tularensis. Conclusion Given the rising incidence of tularaemia in the Netherlands, it is important to consider 'tularaemia' in the differential diagnosis in patients with lymphadenitis and epidemiological clues in their case history. Since 1 November 2016 it has been mandatory to report tularaemia in the Netherlands.</p

Esophageal Atresia and Upper Airway Pathology

Esophageal atresia is an anomaly with frequently occurring sequelae requiring lifelong management and follow-up. Because of the complex issues that can be encountered, patients with esophageal atresia preferably should be managed in centers of expertise that have the ability to deal with all types of anomalies and sequelae and can perform rigorous lifelong follow-up. Tracheomalacia is an often-occurring concurrent anomaly that may cause acute life-threatening events and may warrant immediate management. In the past, major thoracotomies were necessary to carry out the aortopexy. Nowadays, aortopexy and posterior tracheopexy can both be performed thoracoscopically with quick recovery

Een jongen met tularemie na een modderrace

Background Tularaemia is a rare disease. In Europe it mostly occurs in Scandinavia. Since 2011 more cases are being reported in the Netherlands. Tularaemia may manifest itself in various ways. It is important to take strict precautions during biopsy, drainage and biopsy processing in order to prevent transmission. Case description A 10yearold boy presented to the paediatrician with a left inguinal lymphadenitis. A week before the onset of symptoms he had participated in a children's mud race. Serology and PCR of pus from the lymph node tested positive for Francisella tularensis. Treatment with ciprofloxacin was insufficiently effective, so surgical drainage of the gland was performed under strict isolation conditions. Water from the mud race location contained genetic material from F. tularensis. Conclusion Given the rising incidence of tularaemia in the Netherlands, it is important to consider 'tularaemia' in the differential diagnosis in patients with lymphadenitis and epidemiological clues in their case history. Since 1 November 2016 it has been mandatory to report tularaemia in the Netherlands

Effects of esketamine sedation compared to morphine analgesia on hydrostatic reduction of intussusception : A case-cohort comparison study

Background: Hydrostatic or pneumatic reduction of intussusception is an invasive procedure that is stressful and may be painful for a child. Resistance of the child may increase the duration of the procedure and decrease success rate of reduction. Analgesia can help to reduce pain, but not necessarily resistance. General anesthesia increases success rate of reduction. However, it requires the presence of an anesthesiologist, and may lead to anesthesia-related complications. Procedural sedation with esketamine could be a safe alternative.  Aim: The aim of this study was to compare hydrostatic reduction using morphine analgesia compared to procedural sedation with esketamine in terms of success rate, adverse events, and duration of reduction.  Methods: A retrospective case-cohort comparison study was performed with two groups of patients who had undergone hydrostatic reduction for ileocolic intussusception and received morphine analgesia (n = 37) or esketamine sedation (n = 20). Until July 2013, reduction was performed after intravenously administered morphine. Hereafter, a new protocol for procedural sedation was implemented and reduction was performed after administration of esketamine. Cases were matched for age and duration of symptoms.  Results: No adverse events requiring intervention other than administration of oxygen were reported for either group. Success rate of reduction using esketamine sedation was 90% vs 70% using morphine analgesia, risk ratio (RR) 1.29, 95% CI[0.93-1.77]. Recurrence rate using esketamine sedation was 10% vs 15% using morphine analgesia, RR 0.67, 95% CI[0.12-3.57]. Reduction time was shorter using esketamine sedation (Median 5 minutes, IQR 9 minutes) vs morphine analgesia (Median 8 minutes, IQR 16 minutes, P =.04, Median difference 3, 95% CI[−1.50-8.75]). Median hospital stay in the esketamine group was 1.5 days (IQR 1.8) vs 2 days (IQR 5.3) in the morphine group.  Conclusion: No serious adverse events were recorded. In comparison to morphine analgesia, with esketamine there was weak evidence for a higher success rate, lower recurrence rate, shorter duration, and shorter length of hospital stay

Evaluation of an Algorithm for Testis-Sparing Surgery in Boys with Testicular Tumors: A Retrospective Cohort Study

Aim: This study reports surgical treatment and its outcome for boys with a testicular tumor, in order to analyze the considerations of testis-sparing surgery (TSS) and investigate whether, in retrospect, treatment was according to a recently developed algorithm. Methods: We retrospectively reviewed boys with testicular tumors who underwent surgical treatment between January 2000 and June 2020 at the Wilhelmina’s Children’s Hospital and the Princess Máxima Center for Pediatric Oncology, The Netherlands. Medical records were searched for clinical characteristics and outcome. Results: We identified 31 boys (median age = 5.5 years) with a testicular tumor, 26 germ cell tumors (GCTs), four sex cord-stromal tumors, and one gonadoblastoma. Seventeen boys (median age = 1.5 years) had malignant and 14 (median age = 3.6 years) had benign tumors. Four boys with benign GCTs were treated with TSS, 25 with radical inguinal orchiectomy (RIO), and 2 with scrotal orchiectomy. No recurrence or testicular atrophy was reported. All boys with benign testicular tumors were treated as suggested by the algorithm, except for one boy treated with RIO. Conclusion: Retrospective analysis of surgical treatment of prepubertal boys with benign testicular tumors showed that TSS appears to be safe, and should be considered based on clinicoradiological data, in line with our algorithm

Fluorescence guided surgery using indocyanine green for pulmonary osteosarcoma metastasectomy in pediatric patients: A feasibility study

Background: In osteosarcoma patients, resection of pulmonary metastases is considered necessary for long term survival. Radiologically detected lesions are sometimes difficult to identify intraoperatively, especially when no palpation is possible during thoracoscopy. In adults, fluorescence guided surgery (FGS) using indocyanine green (ICG) has been shown to be a safe method for intra-operative identification of pulmonary metastases of different primary tumors. Our aim is to determine the feasibility of using ICG for identification of pulmonary metastases in pediatric osteosarcoma patients. Methods: Nine consecutive patients with pulmonary metastases received an intravenous dose of ICG 24 h preoperatively. We started with the adult dosage of 0.5 mg/kg and also used 1.0 mg/kg to confirm that maximum fluorescent signal was achieved. Intra-operatively and post-operatively, lesions were visualized with a near-infrared camera system. Fluorescence was quantified by calculating a tumor-to-background ratio (TBR). Results: Two (22%) patients underwent a thoracoscopy and seven (78%) underwent a thoracotomy. Five (56%) patients had a fluorescent metastasis during surgery. In four (44%) patients there were no fluorescent metastases. In two the metastases were necrotic. In the other two, intraoperative fluorescence was most likely hampered by the depth of the metastases. Ex vivo, all vital metastases were fluorescent and necrotic specimens were not. There was no difference between 1.0 mg/kg and 0.5 mg/kg concerning TBR. No adverse events occurred. Conclusions: ICG for fluorescence guided metastasectomy of pulmonary osteosarcoma is a feasible procedure in the pediatric population. However, its additional value in intra-operative guidance still has to be investigated. Levels of evidence: Level III evidence based on a Diagnostic test study: study without a universally applied “gold” standard

Tunneled vascular access surgery in pediatric oncology; Experience from a national pediatric oncology center

Central venous access is essential in the treatment of children diagnosed with cancer. Therefore, as a paediatric oncologist/surgeon, it is important to identify the most optimal central venous catheter (CVC) for each patient, to accurately perform CVC surgery and to be aware of the possible complications that can occur and how to prevent and treat them. The aim of this article is to provide an overview of recommendations for CVC choice, methods for tunneled CVC surgery and strategies for perioperative complication treatment. This is based on the experience of the surgeons from our national paediatric oncology center in the Netherlands, inserting 700 CVCs each year