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Background: Scoliosis is one of the long-term consequences of surgical resection of pediatric chest wall tumors. This study aimed to identify the risk factors associated with scoliosis development following the resection of chest wall tumors., Methods: Retrospective cohort study of 64 children who underwent resection of malignant chest wall tumors from 2009 to 2022. Univariate and multivariate analyses were used to investigate factors associated with scoliosis development., Results: The median age at the time of surgery was 7 years (range, 3-21), with 33 (51.6%) patients undergoing surgery before the age of 10 years. The most common histology was Ewing sarcoma (n = 57). A median of 3 (range, 1-5) contiguous ribs were resected. A total of 34 (53.1%) patients had anterior CWTs and 30 (46.9%) had posterior CWTs. Concomitant partial lung and diaphragmatic resection were performed in 12 patients (lung, n = 7; diaphragm, n = 5). Scoliosis convex towards the resection side developed in 21 (32.8%). The primary risk factors for scoliosis were resecting 3 or more ribs (OR 6.44) and resection of the posterior rib segment (OR 5.49). Patients with a tumor resection below 10 years old were not associated with a higher risk of scoliosis., Conclusions: Scoliosis following resection of a primary malignant pediatric chest wall tumor is associated with resection involving three or more ribs and resection of the posterior rib sector., Type of Study: Retrospective observational., Level of Evidence: IV., Competing Interests: Conflict of interest The authors declare there are no competing interests related to this work., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background and Aims: We aimed to review current literature on the impact of Preoperative Identification (POI) of the Adamkiewicz Artery (AKA) in solid pediatric Posterior Thoracic Tumors (PTT), comprising a spectrum of neuroblastic tumors and neuroblastoma, with particular focus on Complete Macroscopic Excision (CME) and Neurologic Complications/Sequelae (NCS)., Methods: A systematic review in accordance with PRISMA guidelines was undertaken. The study included reports on pediatric patients providing available data on POI of the AKA in PTT., Results: Among 838 records screened, 33 full-texts underwent evaluation, and 8 papers were included. Among 49 tumors (N = 49 patients), 30 were neuroectodermic tumors. Spinal angiography (SA) was undertaken in four studies for 32 (65%) patients. No SA-related morbidity was recorded. Otherwise, a combination of CT and/or MRI imaging was described. The lack of detailed reporting on CT/MRI imaging, hindered a feasible detailed comparative analysis among non-SA imaging modality techniques. The overall success rate of AKA POI was 65%, 71% with SA and 33% with non-SA studies. CME was achieved in 73% of SA and 80% non-SA groups. Overall, 5 children experienced NCS, 60% of those who had dumbbell tumors. All NCS occurred in patients without successful POI of the AKA. Furthermore, no SA patient had NCS, while 33% of the non-SA sustained NCS., Conclusions: Successful POI of the AKA may play a key role in prevention of NCS. SA showed the utility of deploying POI, without added morbidity. Furthermore, SA appeared to have a preventive impact on NCS. SA may have a key role on CME which may also be operator dependent., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction: Robotic-assisted minimally invasive surgery (RA-MIS) for tumor resection is an emerging technology in the pediatric population with significant promise but unproven safety and feasibility., Methods: A multi-center retrospective review of patients ≤18 years undergoing RA-MIS tumor resection from December 2015-March 2023 was performed. Patient demographics, perioperative variables, and complication rates were analyzed., Results: Thirty-nine procedures were performed on 38 patients (17 thoracic, 22 abdominal); 37% female and 68% non-Hispanic White. Median age at surgery was 8.3 years (IQR 5.7, 15.7); the youngest was 1.7 years-old. Thoracic operations included resections of neuroblastic tumors (n = 16) and a single paraganglioma. The most common abdominal operations included resections of neuroblastic tumors (n = 5), pheochromocytomas (n = 3), and angiomyolipomas (n = 3). Six patients underwent retroperitoneal lymph node dissection (RPLND) for paratesticular tumors. Median operating time for the cohort was 2:52 h (IQR 2:04, 4:31). Two thoracic cases required open conversion due to poor visualization and lack of working domain. All patients underwent complete tumor resection; one had tumor spillage from a positive margin (Wilms tumor). Median LOS was 1.5 days (IQR 1.1, 3.0). Postoperatively, one patient developed a chyle leak requiring interventional radiology drainage, but none required a return to the operating room., Conclusions: Robotic-assisted surgery is safe and feasible for tumor resection in carefully selected pediatric patients, achieving complete resection with minimal morbidity and short LOS. Resection should be performed by those with robotic expertise for optimal outcomes., Level of Evidence: IV., Type of Study: Original Clinical Research., Competing Interests: Conflicts of interest Dr. Marc Michalsky is a stockholder and educational honorarium with Intuitive Surgical. He also is on the Childhood Obesity Advisory Board for Lilly USA, LLC. No other authors have any disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Surgical treatment of pediatric chest wall tumors requires accurate surgical planning and tumor localization to achieve radical resections while sparing as much healthy tissue as possible. Augmented Reality (AR) could facilitate surgical decision making by improving anatomical understanding and intraoperative tumor localization. We present our clinical experience with the use of an AR system for intraoperative tumor localization during chest wall resections. Furthermore, we present the pre-clinical results of a new registration method to improve our conventional AR system., Methods: From January 2021, we used the HoloLens 2 for pre-incisional tumor localization during all chest wall resections inside our center. A patient-specific 3D model was projected onto the patient by use of a five-point registration method based on anatomical landmarks. Furthermore, we developed and pre-clinically tested a surface matching method to allow post-incisional AR guidance by performing registration on the exposed surface of the ribs., Results: Successful registration and holographic overlay were achieved in eight patients. The projection seemed most accurate when landmarks were positioned in a non-symmetric configuration in proximity to the tumor. Disagreements between the overlay and expected tumor location were mainly due to user-dependent registration errors. The pre-clinical tests of the surface matching method proved the feasibility of registration on the exposed ribs., Conclusions: Our results prove the applicability of AR guidance for the pre- and post-incisional localization of pediatric chest wall tumors during surgery. The system has the potential to enable intraoperative 3D visualization, hereby facilitating surgical planning and management of chest wall resections., Level of Evidence: IV TYPE OF STUDY: Treatment Study., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Resection of pediatric chest wall tumors can result in large defects requiring reconstruction for function and cosmesis. Multiple reconstructive methods have been described. We performed a systematic review of the literature to describe commonly used approaches and outcomes., Methods: A systematic literature search was performed for English-language publications describing chest wall tumor resection and reconstruction using implantable materials in patients ≤21 years, excluding soft tissue resection only, sternal resection, and reconstruction by primary repair or muscle flaps alone. Data were collected on diagnoses, reconstructive method, and outcomes. Rigid chest wall reconstruction was compared to mesh reconstruction., Results: There were 55 articles with 188 patients included. The median age was 12 years. Most tumors were malignant (n = 172, 91.5%), most commonly Ewing's sarcoma (n = 65, 34.6%), followed by unspecified sarcomas (n = 34, 18.1%), Askin's tumor (n = 16, 8.5%; a subset of Ewing's sarcoma) and osteosarcoma (n = 16, 8.5%). A median of 3 ribs were resected (range 1-12). Non-rigid meshes were most common (n = 138, 73.4%), followed by rigid prostheses (n = 50, 26.6%). There were 19 post-operative complications (16.8%) and 22.2% of patients developed scoliosis. There were no significant differences in complications (20.5% rigid vs. 10.6% non-rigid, p = 0.18) or scoliosis (22.7% vs. 14.0%, p = 0.23) by reconstruction method, but complications after rigid reconstruction were more likely to require surgery (90.0% vs. 53.9%, p = 0.09). The median follow-up duration was 24 months., Conclusions: In this review of the literature, there were no significant differences in overall post-operative complications or scoliosis development by reconstruction method, yet complications after rigid reconstruction were more likely to require surgical intervention., Level of Evidence: Level IV., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Background: Minimally invasive surgery has broad applicability to pediatric diseases, including pediatric cancer resection. Neuroblastic tumors of childhood are highly variable in presentation, and so careful selection of appropriate candidates for minimally invasive resection is paramount to achieving safe and durable surgical and oncological outcomes., Methods: The American Pediatric Surgical Association Cancer Committee developed questions seeking to better define the role of minimally invasive surgery for neuroblastic tumors. A search using PubMed, Medline, Embase, Web of Science, ProQuest Dissertations, and Clinical Trials was performed for articles published from 1998 to 2018 in accordance with the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols (PRISMA-P) guidelines., Results: The evidence identified is all retrospective in nature. Minimally invasive surgical resection of neuroblastic tumors is safe for carefully selected smaller (4-6 cm) image defined risk factor (IDRF)-negative abdominal tumors when oncologic principles are followed. Size is a less-well defined criterion for thoracic neuroblastic tumors. Open approaches for both abdominal and thoracic tumors may be preferable in the presence of IDRF's., Conclusion: Small tumors without IDRF's are reasonable candidates for minimally invasive resection. Surgical oncologic guidelines should be closely followed. The quality of data supporting this systematic review is poor and highlights the need for refinement in the study of such surgical techniques to improve knowledge and outcomes for patients with neuroblastic tumors., Type of Study: Systematic Review., Level of Evidence: Level III and Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Chest wall sarcoma is a rare and challenging pathology best managed by a multidisciplinary team experienced in the management of a multiple different pathologies. Knowledge of the management sequence is important for each sarcoma type in order to provide optimal treatment. Surgical resection of chest wall resections remains the primary treatment of disease isolated to the chest wall. Optimal margins of resection and reconstruction techniques have yet to be determined., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Modern surgical practice places increased emphasis on treatment outcomes. There has been a paradigm shift from paternalistic ways of practicing medicine to patients having a major involvement in decision making and treatment planning. The combination of these two factors undoubtedly leaves the surgeon open to greater scrutiny in respect of results and outcomes. In dealing with this it is important that the surgeon, wider multidisciplinary team, and patient appreciate the idea of surgical risk. This article reviews the latest evidence relating to risk assessment in thoracic surgery and suggests how this should be incorporated into clinical practice., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Aim: Childhood malignant chest wall tumors may require extensive surgical resection and reconstruction with musculoskeletal flaps or non-resorbable prosthetic materials. Implant-related complications and scoliosis often occur. This study analyzes the outcomes of chest wall reconstruction using resorbable plates as an alternative approach., Methods: Retrospective review (2007-2015) of patients who underwent resection of malignant primary chest wall tumors in 2 tertiary pediatric centers. Reconstruction was performed using copolymer (l-lactic and glycolic acid) plates, fixed to the ribs and surrounding structures with copolymer screws and/or polyglactin sutures., Results: Eight patients aged 10.6+2.6years were treated. There were no operative complications, and implant removal was not required in any case. Six patients received postoperative radiotherapy. Over follow-up (39.6months, range 9.4-78), chest wall shape was maintained in all, and there were no radiological artifacts. Three patients developed scoliosis (Cobb 17°-33°), but treatment was needed only in one, who had undergone hemivertebrectomy. There were no cases of local tumor relapse. One patient died because of metastatic spread., Conclusions: Implantation of bioabsorbable l-lactic and glycolic acid copolymer plates with a relatively simple technique provided a rigid, stable reconstruction with only mild mid-/long-term complications. Resorbable plates may be a good alternative for pediatric chest wall reconstruction., Level of Evidence: IV., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Purpose: Chest wall tumors in pediatric patients are rare. This study evaluates outcomes in pediatric patients who have undergone chest wall resections secondary to sarcomas., Methods: A retrospective review was performed for patients <19years old who underwent chest wall resections for sarcoma 1999-2014 at the University of Texas MD Anderson Cancer Center., Results: Of 44 patients, Ewing's sarcoma (n=18) and osteosarcoma (n=16) were most common. Other sarcomas included synovial sarcoma, chondrosarcoma, and rhabdomyosarcoma. Gore-Tex® or a Marlex™ mesh and methyl methacrylate sandwich was used in 22 patients, and 9 children did not require reconstruction. Twenty-four (54.5%) patients had normal activity, 3 (6.8%) had occasional discomfort, 2 (4.5%) had pain impairing function, 7 (15.9%) required medication or physical therapy for impairment, and 8 (18.2%) needed additional surgery. Five children (11.4%) developed scoliosis, and all of these patients had posterior rib tumors. Median overall survival for the entire cohort was 41.9±11.82months. Histology (p=0.003), location of tumor on the ribs (p=0.007), and surgical margins (p=0.011) were significantly associated with overall survival. Tumors on the middle and posterior (p=0.003) portions of the ribs had a lower chance of death., Conclusion: Scoliosis is more common in posterior rib resections. Histology, location of the tumor, and surgical margins impact survival, but, type of reconstruction does not., Level of Evidence: III., Type of Study: Treatment Study., (Copyright © 2017. Published by Elsevier Inc.)

Background/purpose: For pediatric tumors of the cervicothoracic junction, an isolated cervical or thoracic surgical approach provides insufficient exposure for achieving complete resection. We retrospectively examined "trap-door" and "clamshell" pediatric thoracotomies as a surgical approach to these tumors., Methods: We searched our database for pediatric patients with cervicothoracic tumors who underwent clamshell or trap-door thoracotomy between 1991 and 2013, reviewing tumor characteristics, surgical technique, completeness of resection, morbidity, and outcome., Results: Trap-door (n=13) and clamshell (n=4) thoracotomies were performed for neuroblastoma (n=9), non-rhabdomyosarcoma soft tissue sarcoma (n=4), germ cell tumor (n=2), rhabdomyosarcoma (n=1), and neuroendocrine small cell carcinoma (n=1). Fourteen of these cervicothoracic tumors were primary, and three were metastatic. Gross total resection was achieved in 15 patients (94%). Operative complications included vocal cord paralysis (n=2), mild upper-extremity neuropraxia (n=2), and hemidiaphragm paralysis (n=1), All but one involved encased nerves. Overall survival was 61% for the series and 80% for patients with primary tumors. Eleven (73%) of 15 patients who underwent gross total resection had no evidence of recurrence. Three patients with metastatic disease died of distant progression within 1.3years., Conclusions: Gross total resection of primary cervicothoracic tumors can be accomplished with specialized exposure in pediatric patients with minimal morbidity., (© 2014.)

Lesions at the thoracic inlet are difficult to access via a thoracic or cervical approach. The use of the anterior cervico-thoracic trapdoor incision has been reported to give good exposure to the anterior superior mediastinum in adults. We report our experience of four cases where a trapdoor incision was used to gain excellent access and exposure to thoracic inlet pathology in children., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: The surgical approach to masses located in the cervicothoracic juncton represents a challenge for surgeons. Many techniques have been described with good results., Methods: We analyzed and compared the results obtained in 2 Italian pediatric surgery centers using 2 different techniques in patients with tumors of the thoracic inlet: center 1, using anterior cervical transsternal approach on 7 patients, and center 2, applying "trap-door" technique on 5 patients., Results: Excision was incomplete in 5 patients and complete in 7 patients. Histologic examination revealed 5 patients with neuroblastoma; 3, ganglioneuroblastoma; 1, mixoid liposarcoma; 1, desmoid fibromatosis; 1, Castleman disease; and 1, Schwann cell tumor. The median duration of the procedure was 345 minutes in center 1 and 245 minutes in center 2. The median blood loss was 200 mL in both centers. The median hospital stay was 11 days in center 1 and 9 days in center 2. Globally, 5 patients developed postoperative complications. No significant differences were encountered comparing the main surgical outcome parameters between the 2 approaches., Conclusions: Both techniques resulted in valid options to achieve a safe excision of thoracic inlet masses with a manageable complication rate and acceptable hospital stay. Surgical risk factors should be carefully investigated preoperatively. Postoperative pain control is important to guarantee early recovery., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Chest wall reconstruction in children is typically accomplished with either primary tissue repair or synthetic mesh prostheses. Primary tissue repair has been associated with high rates of scoliosis, whereas synthetic prostheses necessitate the placement of a permanent foreign body in growing children. This report describes the use of biologic mesh (Permacol; Covidien, Mansfield, MA) as an alternative to both tissue repair and synthetic prostheses in pediatric chest wall reconstruction., Methods: A retrospective chart review was performed identifying patients undergoing chest wall reconstruction with biologic mesh at our tertiary referral children's hospital between 2007 and 2011. Data collection included patient demographics, indication for chest wall resection, number of ribs resected, the administration of postoperative radiation, length of follow-up, postoperative complications, and the degree of spinal angulation (preoperatively and at most recent follow-up)., Results: Five patients (age, 9.0-21.7 years; mean, 15.4 years) underwent resection for primary chest wall malignancy followed by reconstruction with biologic mesh (Permacol) during the study period. There were no postoperative mesh-related complications, and none of the patients developed clinically significant scoliosis (follow-up, 1.1-2.6 years; mean 1.9 years)., Conclusion: Biologic mesh offers a safe and dependable alternative to both primary tissue repair and synthetic mesh in pediatric patients undergoing chest wall reconstruction., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: The management of lymphangiomas in children is a complex problem with frequent recurrence and infection. Vacuum-assisted closure (VAC) devices have been shown to accelerate the healing of open wounds. We hypothesized that VAC therapy might decrease complications after resection of lymphangiomas., Methods: A retrospective review was performed on 13 children (August 2005 to April 2010) who were patients undergoing lymphangioma resection with postoperative VAC therapy. Patient demographics, size and location of the lymphangioma, VAC duration and number of changes, hospital stay, complications, need for further surgery, and length of follow-up were recorded., Results: Thirteen children (mean age, 8 years; mean weight, 34 kg) underwent 15 operations for lymphangiomas followed by postoperative VAC therapy. Locations included the head and neck, thorax and abdomen, and lower extremity. The mean VAC duration was 19 days, and they underwent a mean of 2.6 VAC changes. Six children had operative closure of the wound at a mean of 15 days postoperative. The remaining patients underwent closure by secondary intention. There were no recurrences. Complications included VAC device malfunctions requiring intervention and wound infections. Mean follow-up was 289 days., Conclusion: Postoperative VAC therapy for the treatment of lymphangiomas can be an effective adjunct to surgical treatment by decreasing risks of recurrence and infection., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: We have previously reported the use of the vertical expandable prosthetic titanium rib (VEPTR) for treatment of thoracic dystrophy. This report describes our experience with this device and other novel titanium constructs for chest wall reconstruction., Methods: This is a retrospective chart review of all children and adolescents undergoing chest wall reconstruction with titanium constructs between December 2005 and May 2010., Results: Six patients have undergone chest wall reconstruction with VEPTR or other titanium constructs. Four had chest wall resection for primary malignancy, 1 had metastatic chest wall tumor resection, and 1 had congenital chest wall deformity. There were no immediate complications, and all patients have exhibited excellent respiratory function with no scoliosis., Conclusions: Chest wall reconstruction after tumor resection or for primary chest wall deformities can be effectively accomplished with VEPTR and other customized titanium constructs. Goals should be durable protection of intrathoracic organs and preservation of thoracic volume and function throughout growth. Careful preoperative evaluation and patient-specific planning are important aspects of successful reconstruction., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Teratomas are rare tumors that present most commonly in the anterior mediastinum and retroperitoneum. To date, the retropleural primary site has not been reported., Case Presentation: A 9-month-old boy presented with a large, painless, right-sided abdominal mass. Laboratory values, including tumor markers α-fetoprotein and β-human chorionic gonadotropin, were within normal limits. Diagnostic imaging, including abdominal ultrasound, computed tomographic scan, and magnetic resonance imaging, suggested a large retroperitoneal mass consistent with a teratoma. Because the mass was thought to be arising from the retroperitoneum, an abdominal approach was chosen. After adequate exposure, it became clear that the tumor was arising from above the diaphragm. The diaphragm was incised, and the pleural cavity was entered. The tumor was found in the retropleural space where it was dissected away from the inferior vena cava, aorta, and chest wall. The patient recovered without complications., Results: Histologic examination revealed a mature cystic teratoma with no malignant features. The patient has been disease-free at 7-year follow-up, based upon both clinical examination and diagnostic imaging., Conclusion: This is the first case report of a teratoma arising from the retropleural space. Preoperative imaging may be inaccurate for guiding surgical planning because the diaphragm may not be clearly visualized with current diagnostic techniques. Surgeons should be cautious regarding the location of tumors that arise near the diaphragm and should plan surgical resection carefully., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Background/purpose: Although the use of minimally invasive surgical (MIS) techniques for children with cancer is being practiced by some, its role remains unclearly defined. The purpose of this review was to describe the current literature on MIS for thoracic and mediastinal lesions in children., Methods: We performed a literature search for English studies that evaluated MIS techniques for biopsy or resection in children with suspected or established cancer. Only studies with greater than 20 patients were included in the review., Results: Ten studies were included for review. Each represented institutional retrospective reviews of experience. Seven were single-institution studies, and 3 were multi-institutional. There were no prospective nor randomized identified., Conclusions: Based on primarily retrospective and observational data, the use of MIS for children with cancer who have pulmonary and mediastinal lesions seems to be effective and safe. Ideally, prospective studies are needed to evaluate this further., (Copyright © 2010 Elsevier Inc. All rights reserved.)

High retropharyngeal neuroblastic tumors in children have been excised and debulked transorally or cervically, often with a covering tracheostomy. Although we and others have approached high thoracic lesions thoracoscopically, the trapdoor incision (or modification thereof) is generally reserved for cervicothoracic tumors with significant vessel encasement around the thoracic inlet. We report a case of symptomatic ganglioneuroma extending from the nasopharynx, at the level of the skull base, down to the aortic arch: macroscopic clearance was achieved via an extended trapdoor incision and without recourse to tracheostomy, transoral surgery, or transfusion., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Purpose: Neuroblastoma is the most common extracranial solid tumor in children. Twenty percent of all neuroblastomas arise in the thorax. This study evaluates the open vs thoracoscopic resection of thoracic neuroblastoma., Methods: A retrospective chart review was conducted from the medical records of all children undergoing resection of a thoracic neuroblastoma from 1990 to 2007 at our institution. We evaluated patients who underwent open vs thoracoscopic resection and compared demographics, pathologic condition, stage, operative details, complications, and outcomes between the 2 groups., Results: A total of 149 cases of neuroblastoma were identified during the study period, 36 (24%) of which had tumor located in the thorax. Thirty-six of these patients underwent 37 operations for primary thoracic neuroblastoma. Open thoracotomy was used in 26 cases with the thoracoscopic approach to resection used in the remaining 11. We observed no differences in patient demographics including mean age, sex, or ethnicity. Tumors in both groups were of similar histologic condition, location, surgical margin, lymph node status, and stage. The length of operation was similar between the 2 groups, but length of stay was shorter in the thoracoscopic group (2.0 days; range, 1-7 days vs 3.5 days; range, 2-8 for the open group; P = .01). Estimated blood loss was also less in the minimally invasive group (median, 10 mL; range, 0-75 mL vs 25 mL; 5-650 mL in the open group; P = .02). Review of outcomes showed no significant difference in complications, recurrence, survival, or disease-free survival between these 2 groups., Conclusions: This retrospective review of thoracic neuroblastoma for an 18-year period shows that thoracoscopic resection is an effective approach to this tumor and offers shorter length of stay and decreased blood loss when compared to open thoracotomy., (Copyright 2010 Elsevier Inc. All rights reserved.)

Aims: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood. Patient age, size, histologic finding, and site of the tumor are primary determinants of prognosis in RMS. Chest wall RMS is a site in which the limitations of surgical excision are realized. We aim to determine the impact of surgical excision in chest wall RMS., Methods: A retrospective chart review was conducted of all 130 pediatric patients enrolled in the Intergroup Rhabdomyosarcoma Study (IRS) with chest wall rhabdomyosarcoma from the first (I) through fourth (IV) IRS with follow-up to June 2005. Median follow-up was 12.1 years (4.6-27.2 years)., Results: There was a significant improvement in failure-free survival (FFS) and overall survival (OS) between the first IRS study, I, and IRS-IV. The estimated FFS and OS at 5 years in IRS I was 30% and 40%, respectively, compared to 68% and 78%, respectively, in IRS-IV (P = .03 and P = .05, respectively). There was no association between histologic finding or size and FFS or OS. However, all patients who presented without metastasis had an FFS and OS of 49% and 61%, respectively, compared with metastatic patients, 7% and 7%, respectively (P < .001). Five-year FFS of group I, II, and III patients was 52%, 52%, and 45%, respectively, and OS was 65%, 60%, and 59%, respectively. There was no significant difference in 5-year FFS or OS in patients who had a complete resection (group I), complete resection with positive microscopic margins (group II), or biopsy or partial resection only (group III). In groups I to III patients, the local and regional failure rate at 5 years is 25% and 6%, respectively., Conclusions: The most significant impact on outcome in chest wall RMS patients is metastatic disease at diagnosis. The locoregional failure rate is high but does not appear to impact survival. Alternative treatment strategies are needed for chest wall RMS, but aggressive surgical excision may not be necessary.

A rare case of transmural dumbbell intrathoracic lipoma with intraspinal extension is reported. Preoperative diagnosis was possible with a computed tomographic scan and fine-needle aspiration cytology. Complete excision was curative. The chest wall defect was repaired with Prolene mesh. Only one similar case has been previously reported in the English literature.

Malignant rhabdoid tumor (MRT) is a highly aggressive tumor that occurs in infancy or childhood. The prognosis, especially in infants, is very poor. Here we report the long-term survival of a 5-month-old boy with MRT that arose from the chest wall. After total resection of the tumor, the patient was given 4 cycles of doxorubicin, vincristine, and cyclophosphamide, alternating with ifosfamide and etoposide. After 18 months off therapy, he had a local recurrence at the same site. After a second total resection, he was given additional chemotherapy with 30.6-Gy local irradiation. No further recurrence has been observed for 5 years since the second complete remission. Currently, he is alive and well at 7.5 years post-onset. Our experience in this case suggests a fundamental strategy of successful treatment of this highly malignant pediatric tumor: (1) complete resection of the localized tumor, (2) intensive multiagent chemotherapy for the minimal disseminated disease, and (3) radiotherapy for local control of the disease.

Purpose: The aim of this study was to evaluate the feasibility of thoracoscopy in neurogenic tumors in infants and children., Materials and Methods: From January 2000 to October 2005, 21 patients aged 7 months to 14 years (mean, 6 years) underwent thoracoscopy for tumor resection in 5 French institutions. One 10-mm optical port and 2 operative 5-mm ports were needed. Selective intubation was required for 3 patients aged about 12 years. Tumor was removed with an endoscopic bag in all cases., Results: All procedures were completed successfully without any incomplete resection or recurrence. One conversion was necessary because of a huge mass. A chest tube was left for a mean of 2 days for 17 children. Two children had not had any drainage. Two postoperative chylothorax required chest drainage for 12 days. Only 5 of the 6 older patients (mean age, 12 years) needed a patient-controlled analgesia. The mean operative time was about 100 minutes. Hospital stay ranged from 4 to 12 days. Tumors were neuroblastoma or ganglioneuroblastoma in 16 cases and ganglioneuroma in the 5 other cases., Conclusion: Thoracoscopy for resection of thoracic neurogenic tumors in children is a feasible, safe, and efficient procedure. The surgeon has a better visualization of the tumor and its anatomic connections. Resection can be as complete as an open procedure without having to complicate the operative technique in the same operating time. It avoids cosmetic and functional disorders because of thoracotomy. It allows a good cosmetic resection without spillage.

Purpose: The aim of this study was to determine predictors of outcome in childhood truncal rhabdomyosarcoma., Methods: Retrospective chart review evaluating the impact of demographic features, disease characteristics, and the extent and timing of surgical intervention on outcome was performed., Results: Thirty-three patients with a median age of 8 years were identified. Most had advanced Intergroup Rhabdomyosarcoma Study group III (n = 13) or group IV (n = 9) disease. Primary site included 20 (61%) chest wall, 6 (21%) paraspinal, 5 (15%) periscapular, and 1 (3%) abdominal wall. Most tumors were embryonal (n = 21), larger than 5 cm (n = 27), and locally invasive (n = 13); 7 had positive nodes. Tumor size, nodal status, and gross total tumor resection (upfront or delayed) were significant predictors of event-free and overall survival. Tumors 5 cm or smaller were amenable to upfront surgical resection (P = .007). In patients with tumors larger than 5 cm, resection at any time was associated with a 10-year overall survival 57% +/- 13% compared with 8% +/- 5% in those who had no surgery (P = .003). Tumor recurrence was local in 44% of cases, and survival after local recurrence was rare (1 of 8)., Conclusion: Tumor size, nodal status, and gross total resection at any time are significant predictors of outcome in truncal rhabdomyosarcoma. Gross tumor excision should be the goal of surgical intervention in this disease.

Purpose: Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses. We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT., Methods: Institutional Review Board permission was obtained. Sixty-six patients were diagnosed by histology, immunohistochemistry, and or cytogenetics as having DSRCT at our institution from July 1, 1972, to July 1, 2003. Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival., Results: A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis. The most common presenting complaint was an intraabdominal mass (64%). In 63 patients (96%), the primary tumor was located in the abdomen or pelvis. Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis. Overall, 3- and 5-year survivals were 44% and 15%, respectively. Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy. Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P < .02). Gross tumor resection was highly significant in prolonging overall survival; 3-year survival was 58% in patients treated with gross tumor resection compared to no survivors past 3 years in the nonresection cohort (P < .00001). Ten patients (15%) have no evidence of disease with a median follow-up of 2.4 years (range, 0.4-11.2 years)., Conclusions: Multimodal therapy results in improved survival in patients with DSRCT. Aggressive surgical resection of these extensive intraabdominal neoplasms correlates with improved patient outcome.

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infanthood and early childhood. It is typically located in the extremities and the trunk and less frequently in the head-neck region, mediastinum, and retroperitoneum. However, cases of pleural, thoracic wall, and pulmonary lipoblastoma have also been reported in the literature. Lipoblastoma is a tumor with good prognosis despite its potential for local invasion and rapid growth. Our patient is a 7-month-old girl who was brought to our clinic for a rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the mass was removed by thoracic wall resection and the resultant defect covered with an absorbable polyglactine mesh. The excised mass was diagnosed pathologically as a benign lipoblastoma. In the postoperative period, the thorax wall was stable, and after 24 months of follow-up no recurrence was observed. In the literature, 4 types of thoracic wall lipoblastomas have been reported, but thoracic wall resection has not been applied in any benign lipoblastoma case. To the best of our knowledge, the only benign lipoblastoma case in which a thoracic wall resection was carried out, with its clinical and histopathologic specifications alongside that in the literature, is presented here for the first time.

Primary and metastatic thoracic malignancies are often incurable. Surgeons caring for these patients must be familiar with the options,indications, techniques, and limitations of interventions for palliative treatments in these patients. This article is an overview of the current practices for palliation of a broad spectrum of complaints relating to patients with carcinomas of the lung, esophagus,and mesothelium. The information can be used for treatment of patients with complaints secondary to less common malignancies and metastatic disease of the thorax.

Reconstruction of the chest wall in a child is an uncommon problem for pediatric surgeons. The available material for chest wall reconstruction typically has been borrowed from the adult experience. The authors describe their recent experience with a commercially available bioabsorbable plate in a variety of conditions that require chest wall reconstruction.

Background: Chest wall tumors of primitive neuroectodermal origin (PNET, Ewing's sarcoma [ES]) are rare and have a poor prognosis. Multimodality therapy has improved survival results, and long-term survival is possible. Whether adjuvant radiation therapy is uniformly beneficial remains unclear., Methods: A retrospective analysis of the authors' institutional experience between 1979 and 1998 was performed., Results: Twenty consecutive patients with PNET-ES of the chest wall were identified. The median age was 12 years (range, 2.5 to 21 years). Median follow-up was 3 years (range, 7 months to 19.4 years). Seven patients presented with a mass, 12 with pain, 1 with respiratory distress, and 1 with a neuropathy. Initial therapy consisted of biopsy and neoadjuvant chemotherapy followed by chest wall resection in 12 patients. Of the remaining 8 patients, 6 underwent biopsy, followed by chest wall resection and adjuvant chemotherapy, 1 underwent biopsy, chemotherapy, and resection of a lung nodule, and 1 underwent biopsy, chemotherapy, and a laminectomy and decompression procedure. All 20 patients were included in institutional-based trials using multiagent chemotherapy. Fifteen patients received radiation therapy with a median dose of 3,000 cGy. At last follow-up, 11 patients are alive and disease free, with a median survival of 7.5 years (range, 7 months to 19.4 years). Seven of 11 (64%) survivors had neoadjuvant therapy followed by chest wall resection. Seven of 11 (64%) survivors had radiation therapy. There was no surgical mortality. Twelve patients had treatment-related complications, 3 of which were related to surgical resection. There were no survivors among patients with recurrent disease. Three of the patients who died of disease had both local and distant recurrences, 4 patients had distant recurrence only, and one patient had local recurrence only. Only 4 of 9 (44%) patients who died were treated initially with chemotherapy followed by chest wall resection. All but 1 of those that died (89%) received initial radiation therapy. All 9 patients who did not survive received additional salvage radiotherapy as well., Conclusions: Long-term survival is possible with ES-PNET after complete chest wall resection. This may be facilitated by neoadjuvant chemotherapy. Long-term survival without radiation therapy is possible, and consideration of radiation therapy should be made on a case-by-case basis.

Purpose: Primary chest wall malignancies, which occur infrequently in children, can pose complex technical challenges to the surgeon. This study was undertaken to evaluate the pathology, treatment approaches, role of surgical resection and reconstruction, and outcomes of patients with these tumors., Methods: This is a retrospective review of all patients with malignant primary chest wall tumors treated at our institution between February 1983 and July 1998., Results: Nineteen cases were identified: malignant small round cell type (MSRCT, also called Ewing's sarcoma, primitive neuroectodermal tumor [PNET], and Askin's tumor; n = 8), rhabdomyosarcoma (RMS; n = 6), and other tumors (n = 5). Three patients underwent "upfront" complete resections. Sixteen patients underwent initial biopsy, followed by chemo- or radiotherapy. Nine of these 16 survived to undergo delayed chest wall resections. Six of the 12 "resected" patients required en bloc resection of adjacent muscles or organs; 7 required complex chest wall reconstruction. Eight of 19 patients (42%) have survived (median follow-up of survivors, 4 years), all with no evidence of disease; the remaining 11 patients died of progressive disease. Local invasion did not alter chance of survival. Two of the 10 patients with metastases at diagnosis (20%) survived. Six of the 9 patients (67%) with localized disease survived. All five patients with tumor types other than MSRCT or RMS, metastatic or not at diagnosis, are alive with no evidence of disease. There were no local recurrences., Conclusion: Surgical resection, with en bloc removal of involved structures and chest wall reconstruction, provides excellent local control of malignant chest wall tumors.

Background/purpose: Primitive neuroectodermal tumours (PNET) are rare tumours sharing similar histology, immunohistochemistry, and cytogenetics to Ewing's sarcomas. The aim of the study was to document the effect of site of origin and the completeness of surgical excision on the outcome in infants and children with PNET., Methods: All patients (n = 26) with PNET treated in our hospital during the last 6 years were included in the study. The diagnosis was based on the histopathologic findings and a positive MIC2 antibody test. The tumours were classified according to the Intergroup Rhabdomyosarcoma Study III, and were treated according to the UKEESG protocol for Ewing's Sarcoma., Results: Complete remission (CR) was achieved in 52% of the patients completing their chemotherapy. Overall, survival was 42% (11 of 26). Tumours from the paraspinal and scapula areas responded relatively well (CR, 83%), whereas abdominal PNET did not respond to treatment. Tumours arising from the head, neck, or chest had an intermediate prognosis (37% survival). Three of the 10 patients who had a complete excision of the tumour died of progressive disease., Conclusions: Complete surgical excision reduced the risk of local recurrence but did not prevent metastatic spread. These data indicate that PNET have a worse prognosis and different favourable sites than other sarcomas. New treatment strategies are necessary for these rare tumours.

Many aspects of surgical decision making related to the surgical evaluation and management of children with cancer depend on information gained from radiographic imaging and radiologic consultation. A brief review of the questions that surgeons ask the radiologist may aid the radiologist in focusing the consultation.

The authors present a case of lipoma in infancy. A 12-year-old boy was referred for an asymptomatic fast-growing thoracic tumor of 1-month evolution. A simple chest x-ray showed an intra- and extrathoracic mass of extrapleural localization. Ultrasound scan and computed tomography studies were performed, and the diagnosis of an hourglass lipoma was confirmed by a fine-needle aspiration biopsy. Surgical excision of the tumor was performed and the pathological study reassessed the initial diagnosis of lipoma. The thoracic lipoma is a benign disease and has a good evolution after surgery.

Cystic hygromas are relatively uncommon benign tumors of the lymphatic system. The lesions frequently are apparent at birth, and more than 90% are detected before the end of the second year of life. Most commonly, cystic hygroma presents as a soft tissue mass in the posterior triangle of the neck, and only rarely does it extend into the mediastinum. Isolated intrathoracic hygromas are exceedingly rare and have been reported infrequently among children. Herein the authors review three cases of intrathoracic cystic hygroma, spanning a period of 30 years at their institution.

Mesenchymoma of the chest wall is rare in infancy and can be easily mistaken for a malignant tumor. It is a distinct pathological entity that presents at birth or during infancy as self-limiting expansile intraosseous lesions involving the body of one or more ribs. Histologically, it is a benign focal overgrowth of normal skeletal elements. Two such cases are presented.

Introduction: A multi-institutional study was conducted by the Children's Cancer Group (CCG) to evaluate all soft tissue sarcomas diagnosed within the first month of life., Methods: A retrospective study by 11 CCG institutions of patient records from 1971 to 1991 were reviewed for demographic data, pathology, therapy, and outcome., Results: 32 neonates with soft tissue sarcomas were identified. There were 21 boys and 11 girls. Pathology was equally divided into three groups: Congenital fibrosarcoma (CFS) (12), rhabdomyosarcoma (RMS) (11), and non-RMS soft tissue sarcomas (NRSTS) (9). Anatomic sites consisted of head/neck (11), extremity (9), trunk (8), pelvis (3), and unknown (2). Overall survival rate was 59% (19/32)., Conclusion: Soft tissue sarcomas in the neonate comprise three general groups with survival rates dependent on pathology and extent of disease.

Neuroblastomas located in the apex of the hemithorax or in the lower cervical region may make complete resection via a cervical or a thoracic approach difficult. The authors recently managed two patients with cervicothoracic neuroblastomas through an approach using the trap-door incision often applied in the setting of vascular trauma. This approach allowed a successful, complete excision of these tumors, which may have otherwise been difficult.

The most difficult aspect of the surgical treatment of chest wall tumors is reconstruction of the large residual defect. Materials that have been used include Marlex, Goretex, Vicryl, bone, metal, and fascia. The authors' successful experience with dehydrated human dura (Tutoplast) for moderate-size defects is described. A large Askin's tumor in a 13-year-old boy required resection of the right posterior aspect of the 9th to 11th ribs and the transverse process of T-10, the 12- x 12-cm thoracic defect was closed with dura. Partial soft-tissue coverage was obtained with the latissimus dorsi muscle. Although a scoliosis secondary to paraspinal muscle resection has developed, the chest wall is stable, without evidence of a flail chest, at 18 months of follow-up. A 6-year-old girl underwent left anterior chest wall resection of three ribs for an epithelioid sarcoma. Human dura and a myocutaneous flap were used for reconstruction, with good functional and cosmetic results at 16 months of follow-up. Dura is simple to use, has low antigenicity, and in experimental studies appears to be incorporated into the tissues, acting as a collagen matrix. For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis.

Lumbar hernia is a rare occurrence in infants and children. Congenital variants have been described, frequently associated with musculofascial and skeletal abnormalities, specifically "lumbocostovertebral syndrome" (LCV) and/or meningomyelocele. In LCV syndrome, lumbar herniation results from a single somatic defect occurring during the third to fifth week of embryonal development. Meningomyelocele may predispose to lumbar herniation secondary to abnormalities in muscular innervation related to nerve entrapment in the spinal dysraphism. Acquired lumbar hernia generally can be attributed to surgery, infection, or trauma. Localized neuropraxis, temporary or permanent, may be the underlying factor common to all these defects. The author presents two pediatric cases of lumbar hernia associated with intrathoracic neuroblastoma. The first patient, a 15 month old, had a lumbar hernia after excision of a thoracic ganglioneuroblastoma. This resolved within 1 year, without specific therapy. The second patient, a 4 month old with a lumbar hernia and a large intrathoracic neuroblastoma, had "resolution" of the hernia within 4 months of excision of the paravertebral tumor. In these cases it appears that the lumbar hernia resulted from neuropraxis secondary to intrathoracic paravertebral tumor and its management. In both cases, this deficiency was temporary and resolved without specific therapy. The association of lumbar hernia and intrathoracic neuroblastoma has not been reported previously. These cases suggest the advisability of a screening chest x-ray in children presenting with this diagnosis. Similarly, the cases suggest a role for conservative treatment for the hernia itself, when the neural impairment resulting in the defect is of a temporary nature.

Fetal tumors are being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. High-output cardiac failure and hydrops indicate fetal distress. Management may be limited by the gestational age of the fetus. Our experience with three fetal tumors demonstrates the dilemma with respect to timing of delivery and prognosis. Following the diagnosis of a large sacrococcygeal tumor, a 22-week-gestation fetus became hydropic and died. Another fetus with a rapidly growing posterolateral chest wall mass required cesarean section delivery at 29 weeks gestation. Postnatal course was complicated by pulmonary hypoplasia, intratumoral hemorrhage, and death. The third fetus had an enlarging tumor in the right lobe of the liver. Poor biophysical profile and mild hydrops necessitated cesarean section delivery at 34 weeks. Right hepatic lobectomy was performed and the infant was subsequently discharged home at one month of age. The deleterious effects of the fetal tumor and the need for its removal have to be carefully weighed against the ability of the fetus to survive postnatally.

The Executive Committee of the B.A.P.S. instituted a collaborative study of patients with thoracic neuroblastoma treated by members in the British Isles. The study covers patients diagnosed in the decade 1970-1979. There is clear evidence of thoracic disease in all patients reported but there is a problem in defining whether the thoracic disease is primary or part of disseminated disease. In 9 of the deaths the thoracic component at presentations seems likely to be part of a generalised disease without definition of a primary site. Overall mortality in this series is 35% indicating the better prognosis of the patients presenting with thoracic neuroblastoma compared to other primary sites. If the 9 patients considered to have generalised disease are excluded then the mortality in this collected series is 22% (10 of 45). The investigations and management are outlined and the complications of therapy are considered.

The CO2 laser has found its place in surgery and is now an accepted modality in many fields. Its application in pediatric surgery is well based and it is our opinion that its use in this field should be more widespread.

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.

A biochemical analysis was performed on tumor tissues from 20 patients who presented with thoracic neuroblastomas. Nine patients were under 1 year of age at the time of diagnosis, and 12 patients had stage A disease. Eighteen of the 20 patients are disease free with a mean follow-up of 5 1/2 years. The ganglioside composition of the tumor tissue was investigated, since these cell membrane components have been proposed to play a role in cell to cell interaction and may be altered on cell transformation. In addition, the ganglioside composition of the central nervous system changes with maturation. Previous studies in children with neuroblastoma have shown that tumor tissue containing more complex gangliosides is associated with a better prognosis. Neuroblastomas from patients with thoracic primaries were found to contain more complex gangliosides of the b series (GD1b, GT1b) and fewer monosialogangliosides, suggesting a more differentiated cellular composition. Tissue from one of the thoracic patients who died lacked GT1b. The absence of this ganglioside has proven to be an indicator of a poor prognosis. Four specimens contained no detectable GD2, which is thought to be a specific marker for neuroblastomas. These data suggest that the improved prognosis seen with thoracic neuroblastomas is due to a basic biologic difference within these tumors, and this finding should be taken into consideration when planning therapy.

Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.