Growing teratoma syndrome in children and adolescents: Prevalence and surgical outcome.

INTRODUCTION: Patients affected by metastatic germ cell tumors may occasionally experience enlargement of masses with concurrent normalization of tumor markers during or after chemotherapy. This phenomenon is described as growing teratoma syndrome (GTS). The aim of the pre sent study is to assess the prevalence of GTS in the pediatric population and its implications in terms of surgical outcome. PATIENTS AND METHODS: The clinical notes of patients diagnosed with stage III and IV malignant germ cell tumors from January 2010 until December 2020 at our Institution were retrospectively reviewed. The prevalence of GTS, treatment strategies, survival, and outcome were analyzed. RESULTS: Thirty-three patients with high-stage malignant germ cell tumors were diagnosed in our institution in the analyzed period. Nine patients (28%) had radiologic evidence of enlargement of persistent masses with normal markers after chemotherapy; these patients were classified as GTS patients. All nine patients underwent resection of metastatic lymph nodes, and six had surgery on visceral metastases. In six patients, radical excision of all metastatic sites was achieved; five patients are alive and in complete remission, while one died because of peri-operative complications. Out of the three patients who could not achieve radical excision of the metastases, two died of progressive disease, and one is alive with progressive disease. CONCLUSIONS: Patients affected by GTS have a risk of progression of chemotherapy-resistant disease and death. Radical surgical excision is essential to achieve disease control and long-term survival.

Percutaneous treatment of chest wall chondroid hamartomas: the experience of a single center.

BACKGROUND: Thoracic mesenchymal hamartomas are rare benign lesions. Rarely symptomatic, they may compress pulmonary parenchyma, leading to respiratory distress. Although spontaneous regression has been documented, the more common outcome is progressive growth. The treatment of choice is en bloc excision of the involved portion of the chest wall, frequently leading to significant deformity. OBJECTIVE: The aim of our study was to describe percutaneous techniques to treat these lesions. MATERIALS AND METHODS: We collected data of children with thoracic mesenchymal hamartomas who were treated at our institution from 2005 to 2020 using various percutaneous techniques. Techniques included radiofrequency thermoablation, microwave thermoablation (microwave thermoablation) and cryoablation. RESULTS: Five children were treated for chest wall hamartomas; one child showed bilateral localization of the mass. Two children underwent microwave thermoablation, one radiofrequency thermoablation and two cryoablation; one child treated with cryoablation also had radiofrequency thermoablation because mass volume increased after the cryoablation procedure. The median reduction of tumor volume was 69.6% (24.0-96.5%). One child treated with microwave thermoablation showed volumetric increase of the mass and underwent surgical removal of the tumor. No major complication was reported. CONCLUSION: Percutaneous ablation is technically feasible for expert radiologists and might represent a valid and less invasive treatment for chest wall chondroid hamartoma, avoiding skeletal deformities.

Femoral hernia in pediatric population: a diagnostic and surgical challenge.

PURPOSE: Femoral hernia (FH) is a rare and often misdiagnosed pathology in pediatric population. The aim of our study was to describe the experience of a Tertiary Center in children with FH, underlying diagnostic and surgical details that could improve its management. METHODS: A retrospective study of pediatric patients who underwent FH repair from January 2010 to June 2023 at our Institution was performed. RESULTS: In the analyzed period, 31 patients underwent surgical procedure for FH at our institution, of whom 16 (51.6%) were female. The mean age at time of surgery was 5.8 years (range 0.5-17.1 years). The rate of pre-operative misdiagnosis was 35.5% and open approach was adopted in all cases (16.1% with mesh application). Only two patients (6.4%) experienced surgical complications: one recurrence (repaired six months later) and one post-operative hematoma (treated successfully with conservative method). CONCLUSION: Due to the high rate of misdiagnosis, the variety of surgical approaches proposed, and the potential for intraoperative complications, FH poses a challenge for pediatric surgeons and urologists, as confirmed by the literature. It is essential to underline the importance of a proper clinical examination in order to correctly diagnose FH, make the best surgical plan for the patient and prevent post-operative complications.

The pediatric DAV-expert algorithm: A GAVeCeLT/GAVePed consensus for the choice of the most appropriate venous access device in children.

In pediatric patients, the choice of the venous access device currently relies upon the operator's experience and preference and on the local availability of specific resources and technologies. Though, considering the limited options for venous access in children if compared to adults, such clinical choice has a great critical relevance and should preferably be based on the best available evidence. Though some algorithms have been published over the last 5 years, none of them seems fully satisfactory and useful in clinical practice. Thus, the GAVePed-which is the pediatric interest group of the most important Italian group on venous access, GAVeCeLT-has developed a national consensus about the choice of the venous access device in children. After a systematic review of the available evidence, the panel of the consensus (which included Italian experts with documented competence in this area) has provided structured recommendations answering 10 key questions regarding the choice of venous access both in emergency and in elective situations, both in the hospitalized and in the non-hospitalized child. Only statements reaching a complete agreement were included in the final recommendations. All recommendations were also structured as a simple visual algorithm, so as to be easily translated into clinical practice.

The Use of Cavitron Ultrasonic Surgical Aspirator for High-Risk Neuroblastoma with Image-Defined Risk Factors in Children.

Aim of the study: The cavitron ultrasonic surgical aspirator (CUSA) has gained popularity in adult surgical oncology, but its application in children is limited to liver surgery and neurosurgical procedures. The complete resection of neuroblastoma with image-defined risk factors (IDRFs) is still considered one of the most difficult procedures to achieve in pediatric surgical oncology, with a high morbidity rate and potential risk of intraoperative mortality. The aim of our study is to describe the application of ultrasonic dissection in neuroblastoma with IDRFs. Methods: A retrospective study was performed, analyzing patients operated on from 2000 to 2018. Patient characteristics, resection completeness, and postoperative surgical and oncology outcomes were analyzed. Main results: Twenty-six patients with high-risk neuroblastoma and IDRFs were operated on in the study period with a CUSA. A complete macroscopic resection was performed in 50% of patients, while the other half was operated on with minimal residual (<5 mL). Six post-operative complications occurred without the need for surgery (Clavien−Dindo < 3). The overall survival was 50%, with a median follow-up of 69.6 months (5.6−140.4). Conclusions: The application of the CUSA in neuroblastoma with IDRFs can be considered an effective and safe alternative technique to achieve a radical resection.

Chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis: Active disease or scarring? A case report and literature review.

The second most common abdominal tumor in children is Wilms' tumor, and the lung is where it most often metastasizes. The typical metastases are multiple, peripherally located, round, and variable-sized nodules. Atypical patterns are also possible and may create diagnostic challenges, especially in patients treated with chemotherapy. Among these, cavitating metastases are an anecdotal type of atypical secondary lung lesions. Here, we report a case of a chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis discovered during the follow-up for an anaplastic nephroblastoma in a 6-year-old girl. Furthermore, we conducted a review of the existing literature on this exceedingly rare radiological pattern to establish its best management.

Case report: A simple and reliable approach for progressive internal distraction of the sternum for Jeune syndrome (asphyxiating thoracic dystrophy): preliminary experience and literature review of surgical techniques.

Background: Described for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS. Material and methods: A retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed. Results: We presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves. Conclusions: We obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings.

Preoperative Spinal Angiography for Thoracic Neuroblastoma: Impact of Identification of the Adamkiewicz Artery on Gross Total Resection and Neurological Sequelae.

BACKGROUND: Patients with thoracic neuroblastoma (TNB) are at high risk of postoperative neurologic complications due to iatrogenic lesions of the artery of Adamkiewicz (AKA). The role of performing a preoperative spinal angiography (POSA) in these patients must be clarified. The present study sought to further understand the relationship between POSA and TNB, as well as the effects of identifying the AKA on surgical excision and neurological consequences. METHODS: Data from patients with TNB who underwent POSA between November 2015 and February 2022 at our tertiary pediatric center were retrospectively analyzed. RESULTS: Six patients were identified, five of whom (83%) were considered eligible for surgical excision. Gross total resection (GTR) was achieved in three patients (60%), which included two patients with an AKA contralateral to the tumor, and one with an homolateral AKAl. After a median follow-up of 4.1 years from diagnosis, no patients developed neurological complications; five (83%) were alive and well, and one died from refractory recurrence. CONCLUSIONS: Among patients with TNB, POSA was useful for identifying the AKA and defining the optimal surgical strategy. POSA should be considered in the preoperative evaluation of TNB to increase the likelihood of GTR and reduce the threats of iatrogenic neurologic sequelae.

Recurrent thoracic air leak syndrome in patients affected by pulmonary graft-versus-host disease: Surgical strategies and outcome.

BACKGROUND AND AIMS: Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) that affects approximately 0.83%-3.08% patients after allogenic hematopoietic stem cell transplant. Such complication is defined as the occurrence of any form of air leak in the thorax, including spontaneous pneumomediastinum or pneumopericardium, subcutaneous emphysema, interstitial emphysema and pneumothorax and has a negative impact on post-transplant survival. The aim of the present study is to describe a single-center experience in the surgical management of recurrent TALS in adolescents and young adults and its outcome. METHODS: We retrospectively reviewed the clinical notes of patients with previous allogenic hematopoietic stem cell transplant who underwent surgical procedures for recurrent TALS from January 2016 until March 2021. We analyzed clinical data, number of episodes of thoracic air leak, surgical procedures and relative outcome. RESULTS: In the examined period, four patients, aged 16-25 years, underwent surgical procedures for TALS, including thoracostomy tube placement, thoracoscopic pleurodesis and thoracotomy. All the patients had been diagnosed with pGvHD before the onset of TALS, with a mean time lapse of 276 days (range 42-513). These patients experienced on average 4.5 air leak episodes (range 3-6). All the patients experienced at least two episodes before surgery. One patient underwent emergency tube thoracostomy only, three patients underwent thoracoscopic pleurodesis and two patients underwent thoracotomy. After surgery, patients were free from air leak symptoms for a mean time of 176 days (range 25-477). Pulmonary function progressively deteriorated, and all the patients eventually died because of respiratory failure after a mean time of 483 days (range 127-1045) after the first episode of air leak. CONCLUSIONS: Surgery provides temporary relief to symptoms related to TALS but has limited effects on the underlying pathophysiologic process. The development of TALS in a sign of progressive pulmonary function worsening and is associated with high risk of respiratory failure and mortality.

Safety and effectiveness of subcutaneously anchored securement for tunneled central catheters in oncological pediatric patients: A retrospective study.

BACKGROUND: Proper securement of central venous catheters plays an important role in onco-hematological pediatric patients. A new subcutaneously anchored securement device has been recently introduced in the clinical practice, and it has been extensively used in children. METHOD: In our study, we have retrospectively investigated the safety and the effectiveness of such device, reviewing the experience of three Italian pediatric oncological units. We have considered only tunneled catheters (cuffed or non-cuffed) inserted in children with malignancy; all types of tunneled central catheters were included in the analysis (both centrally and peripherally inserted) as long as they were secured with a subcutaneously anchored device. We investigated the incidence of dislodgment and of other catheter-related complications, with special attention to local adverse effects potentially related to the securement device. RESULTS: We collected data from 311 tunneled catheters of different caliber: 80.4% were centrally inserted central catheters (CICC), 15.4% were peripherally inserted (PICC), and 4.2% were femorally inserted. Approximately half of the catheters (51%) were non-cuffed. Incidence of dislodgment was very low (2.6%) and the incidence of local pain or inflammation potentially related to the securement device was minimal (1.9%). Catheter related bacteremias were below 1 episode/1000 catheter days. No symptomatic catheter related thrombosis was reported. There was no significant difference in complications comparing cuffed versus non-cuffed catheters, or CICCs versus PICCs, or hematologic tumors versus solid tumors. CONCLUSION: In our retrospective analysis of a vast population of oncological pediatric patients with tunneled central catheters, the subcutaneously anchored securement device was tolerated very well, and it was highly effective in preventing dislodgment, both in cuffed and non-cuffed catheters.

Pigmented median raphe cyst of the scrotum. A rare pediatric case and review of the literature.

AIM: Median raphe cyst are uncommon malformations of male genitalia, in which are rarely described melanin pigments or melanocytes; less than ten cases have been reported in literature. The aim of our study is to describe a rare ormations, case of pigmented median raphe cyst of the scrotum, successfully treated in our hospital. CASE EXPERIENCE: A 6-years-old boy underwent surgical removal of a melanocytic lesion of the ventral surface of the scrotum in Day Surgery regimen. He reported no surgical complication or recurrence. RESULTS: Histology showed multiple cystic nodules, lined by squamous and pluri-stratified columnar epithelium, some of which contained melanic deposits and were anti-MART-positive. DISCUSSION: Even though the first case has been reported in 1985, the etiology of median raphe cysts remains unclear. Infrequently associated with trauma or infections, these lesions seem to origin from an abnormal development of the periurethral glands or atypical closure of the median raphe. Rarely melanin pigments or melanocytes are described in the histological examination, and the cause of the pigmentation is still unknown. CONCLUSION: Median raphe cysts present a non-negligible variety of clinical presentations and histological features. Pigmented ones represent the rarest form: further studies may be necessary to clarify their pathogenesis and describe their clinical evolution. KEY WORDS: Median raphs, Male genitalia, Malformations.

Phytotherapy as ancillary treatment after urinary stone lithotripsy in pediatric age.

AIM: Small stone fragments (NSRF) may be observed after mini-invasive lithotripsy. A 3-components herbal extract drug was tested in young patients presenting NSRF to assess efficacy, safety and tolerability. MATERIAL AND METHODS: Patients aged 6-18 years, treated by endo-urological procedures in 5-year period, were randomly divided in 2 groups. Group A received a 3-components phytotherapic composed of Herniaria hirsuta and Peumus boldus plus water oral intake for 12 days/month in 3 months. Group B had no adjuvant phytotherapy. Group A and B were divided in 2 subgroups, according to persistence of NSRF. Patients were evaluated after the endo-urological procedure (Time 0), at 3-months therapy (Time 1) and after 3-months follow-up (Time 2). Persistence or development of new micro-lithiasis, adverse effects and urological check were registered. RESULTS: Thirty-four patients were enrolled (Group A=15, Group B=19). Two patients were excluded. In Group A, 6 patients were stone free at Time 0 and had no recurrence, while 7 patients (53.8%) had NSRF at Time 0, reduced to 3 (23.0%) and to 2 (15.4%) at Time 1 and 2 respectively. In Group B, 11 patients (57.9%) presented NSRF at Time 0, reduced to 8 (42.1%) and 7 (36.8%) at Time 1 and 2. The difference was significant (Time 1 p=0.006, Time 2 p=0.009). No adverse effects were reported. DISCUSSION AND CONCLUSIONS: The drug was effective in preventing new stones development and reducing significantly stone fragments persisting after endo-urological lithotripsy in children, with optimal tolerability and no adverse effects. KEY WORDS: Arbutin, Boldine, Phytotherapy, Pediatrics, Umbelliferone, Urinary tract stones.

Case report: Primary ovarian Burkitt's lymphoma: A puzzling scenario in pediatric population.

Burkitt's lymphoma (BL) is defined as a highly invasive B-cell lymphoma, usually characterized by an excellent prognosis, more than 90% of children and adolescents being cured with highly dose-intensive multiagent chemotherapy. Primary ovarian localization without involvement of other organs is a rare manifestation of BL, especially in pediatric population. Symptoms at diagnosis are similar to other ovarian lesions and differential diagnosis may be challenging for clinicians. A 12-year-old girl was referred to our institution for abdominal pain and palpable mass observed by the pediatrician. Diagnostic work-up demonstrated a large mass arising from the right ovary, causing compression on abdominal aorta, inferior vena cava, ureters and bowel, with a second smaller lesion on the left ovary. At surgery, a 15 cm-large, ruptured mass arising from the right ovary was found, associated with a second lesion originating from the left ovary (8 cm) and multiple nodules of the greater omentum. Right salpingo-oophorectomy was performed, incisional biopsies were taken from the left ovary and omental nodules and peritoneal fluid samples were collected for cytology. Pathology revealed a Burkitt lymphoma and the patient underwent chemotherapy according to AIEOP LNH-97 Protocol, group R3 with Rituximab. Preoperative diagnosis of primary ovarian lymphoma is extremely difficult. Surgical exploration is often necessary in patients presenting with acute abdominal or pelvic pain; when the suspicion of primary ovarian lymphoma arises intraoperatively, every effort should be made to minimize invasive procedure in order to enhance post-operative recovery.

Paraplegia after thoracotomy: a single center experience with pediatric patients and a review of the literature.

AIM: Paraplegia is an infrequent although fearsome complication of anesthesia and surgical procedures, such as epidural anesthesia and thoracotomy. It may occur in both adults and children and a medullary lesion may be confirmed by magnetic resonance imaging, rather than computed tomography. The aim of this study is to describe the experience of two pediatric tertiary centers, contextualizing it with the other cases reported in literature. MATERIAL AND METHODS: We reported three pediatric cases of post-operative paraplegia in oncological patients, focusing on the potential causes and underling the possible strategies to prevent this complication. RESULTS: From our study, two principal features emerged: 1) Epidural anesthesia may expose children to a greater risk of spinal cord permanent damage due to the execution of the procedure under general anesthesia, which deprives the anesthesiologist of an important feedback about the position of the device; 2) In thoracotomy, the risk of paraplegia tends to increase along with the proximity to the costo-vertebral angle, especially if electrocautery or hemostatic materials are used. CONCLUSIONS: A prompt post-surgical neurological status routine assessment in pediatric patients undergoing epidural anesthesia or thoracotomy should be mandatory and, associated with the correct imaging study, may lead to the most appropriate therapeutic pathway and to a better prognosis. KEY WORDS: Anesthesia, Children, Epidural, Thoracotomy, Paraplegia.

Case report: Bilateral pleural effusion secondary to late migration of a tunneled central venous catheter in a patient affected by high risk neuroblastoma.

The insertion of long-term central venous catheters is a standard of care for children affected by malignancies, although it can be associated with life-threatening complications. The present paper reports an unusual mechanical complication related to the use of a long term tunneled central venous catheter in a pediatric oncologic patient. An 18 months old child, diagnosed with stage M high-risk retroperitoneal neuroblastoma, underwent ultrasound-guided placement of a 6 Fr bilumen long-term tunneled central venous catheter in the right internal jugular vein prior to the beginning of induction chemotherapy. The correct position of the distal tip of the catheter was confirmed by fluoroscopy. After 4 months of regular use of the device, the patient experienced neck swelling during high-dose chemotherapy infusion. A chest x-ray showed a dislocated catheter and bilateral pleural effusion. CT scan demonstrated the tip of the catheter rupturing the medial wall of the right jugular vein and entering the mediastinum; furthermore, pneumomediastinum, subcutaneous neck emphysema and bilateral pleural effusion were noticed and a thrombus was evident in the right jugular vein at the insertion in the brachiocephalic vein. The patient was then transferred to the Intensive Care Unit and bilateral thoracostomy tubes were placed urgently (500 mL of clear fluid were evacuated from pleural spaces). The dislocated catheter was removed electively on the following day under fluoroscopy. Despite ultrasound-guided placement and long-term uneventful use of the catheter, life-threatening central venous catheter-related mechanical complications can occur; the current case report emphasizes the importance of careful monitoring of patients with central venous catheters in order to quickly diagnose and treat potentially lethal complications.

Intraoperative Neuromonitoring for Thyroid Surgery in Children and Adolescents: A Single Center Experience.

Intraoperative neuromonitoring (IONM) of the recurrent laryngeal nerve (RLN) has been shown in adults to minimize nerve palsy after thyroid surgery, but only few studies on its efficacy in a pediatric population have been reported. We conducted a retrospective study on patients operated for thyroid lesions from 2016 to 2022. The analyzed population was divided in two groups: patients treated from 2016 to 2020, when the identification of the RLN was performed without IONM (Group A); and patients treated since 2021, when IONM was implemented in every surgical procedure on the thyroid (Group B). Intraoperative Neurophysiological Monitoring was performed by using corticobulbar motor-evoked potentials and continuous electromyography. Twentyfive children underwent thyroid resection, 19 (76%) of which due to thyroid carcinoma. Each patient's recurrent nerve was identified; IONM was used in 13 patients. In Group A, one temporary nerve palsy was identified postoperatively (8.3%), while in group B one nerve dysfunction occurred (7.7%). No statistically significant difference was found between the two groups in terms of post-operative RLN palsy. No surgical complication due to the use of IONM was reported. In children and teenagers, intraoperative neuromonitoring of the recurrent laryngeal nerve is a safe and accurate method, minimizing the risk of nerve damage.

Intraoperative Neuromonitoring for Pediatric Pelvic Tumors.

Background: Tumors of the pre-sacral and sacral spaces are a rare occurrence in children. Total tumor excision is required due to the significant risk of relapse in the event of partial surgery, but the surgical procedure may lead to postoperative problems such as urinary, sexual, and anorectal dysfunctions. Intraoperative neuromonitoring (IONM) has gained popularity in recent years as a strategy for preventing the onset of neurologic impairments by combining several neurophysiological techniques. The aim of our study is to describe the experience of Bambino Gesù Children's Hospital in the use of IONM in pediatric pelvic surgery. Materials and Methods: The data of patients treated for pelvic malignancies at Bambino Gesù Children's Hospital from 2015 to 2019 were retrospectively collected. All patients were assessed from a neurologic and neuro-urologic point of view at different time-points (before and immediately after surgery, after 6 months, and 1-year follow-up). They were all monitored during a surgical procedure using multimodal IONM including transcranial motor evoked potentials (TcMEP), triggered-EMG (t-EMG), pudendal somatosensory evoked potentials (PSSEP), and bulbocavernosus reflex (BCR). Results: During the study period, ten children underwent pelvic tumor removal at our Institution. In all cases, intraoperative neurophysiological recordings were stable and feasible. The preservation of neurophysiological response at the same intensity during surgical procedures correlated with no new deficits for all neurophysiological techniques. Discussion: Although the impact of the IONM on surgical strategies and clinical follow-up is unknown, this preliminary experience suggests that the appropriate use of several neurophysiological techniques can influence both the radicality of pelvic tumor removal and the neurological and urological outcome at clinical follow-up. Finally, because of the highly complex anatomy and inter-individual variances, this is especially useful in this type of surgery.

Uterine leiomyoma in pediatric population: A case report and review of the literature.

Uterine leiomyomas are rare in the pediatric population with less than 20 cases in adolescences reported in the literature. Furthermore, these masses represent a common presentation of gynecologic tumors with increasing age. We report a case of a 14-year-old female who presented with abdominal pain and increasing abdominal girth. Workup with ultrasound, CT and MRI demonstrated a large pelvic mass. Complete resection by median laparotomy was performed. The mass weighed 5,596 g and was 29.5 cm × 27 cm × 19 cm; the pathological examination confirmed the hypothesis of leiomyoma. The patient remained asymptomatic at 3 months follow up.

Vascular Access in Pediatric Oncology and Hematology: State of the Art.

Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer.