Publications by authors named "Roshni Dasgupta"

110 Publications

Update on pediatric testicular germ cell tumors.

J Pediatr Surg 2021 Apr 15. Epub 2021 Apr 15.

Department of Surgery, Mott Children's Hospital, University of Michigan, Ann Arbor, MI, United States.

Background: Testicular germ cell tumors are uncommon tumors that are encountered by pediatric surgeons and urologists and require a knowledge of appropriate contemporary evaluation and surgical and medical management.

Method: A review of the recommended diagnostic evaluation and current surgical and medical management of children and adolescents with testicular germ cell tumors based upon recently completed clinical trials was performed and summarized in this article.

Results: In this summary of childhood and adolescent testicular germ cell tumors, we review the initial clinical evaluation, surgical and medical management, risk stratification, results from recent prospective cooperative group studies, and clinical outcomes. A summary of recently completed clinical trials by pediatric oncology cooperative groups is provided, and best surgical practices are discussed.

Conclusions: Testicular germ cell tumors in children are rare tumors. International collaborations, data-sharing, and enrollment of patients at all stages and risk classifications into active clinical trials will enhance our knowledge of these rare tumors and most importantly improve outcomes of patients with testicular germ cell tumors.

Level Of Evidence: This is a review article of previously published and referenced level 1 and 2 studies, but also includes expert opinion level 5, represented by the American Pediatric Surgical Association Cancer Committee.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.04.001DOI Listing
April 2021

An Introduction to Pediatric Surgical Oncology.

Authors:
Roshni Dasgupta

Surg Oncol Clin N Am 2021 Apr;30(2):xv-xvi

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Avenue, Cincinnati, OH 45229, USA. Electronic address:

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http://dx.doi.org/10.1016/j.soc.2021.01.001DOI Listing
April 2021

Management of Rhabdomyosarcoma in Pediatric Patients.

Surg Oncol Clin N Am 2021 Apr 27;30(2):339-353. Epub 2021 Jan 27.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Ave, Cincinnati, OH 45229, USA.

Rhabdomyosarcoma is the commonest soft tissue sarcoma in children. Clinicians need vigilance to recognize the different signs and symptoms this tumor can present with because of variable sites of origin. Diagnosis requires a safe biopsy that obtains sufficient tissue for pathologic, genetic, and biological characterization of the tumor. Treatment depends on accurate staging with imaging and surgical sampling of draining lymph nodes. A multidisciplinary team assigns patients to risk-based therapy. Patients require chemotherapy and usually a combination of complex, site-specific surgery and/or radiotherapy. Outcomes for localized rhabdomyosarcoma continue to improve but new treatments are required for metastatic and relapsed disease.
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http://dx.doi.org/10.1016/j.soc.2020.11.003DOI Listing
April 2021

Current surgical management of children with osteosarcoma and pulmonary metastatic disease: A survey of the American Pediatric Surgical Association.

J Pediatr Surg 2021 Feb 6;56(2):282-285. Epub 2020 Oct 6.

Department of Surgery, UT Health Science Center San Antonio, San Antonio, TX.

Background: Rates of long-term survival for children with pulmonary metastatic osteosarcoma are low, and complete surgical resection of all visible pulmonary metastases is necessary for long term survival. Surgical approaches for metastasectomy include thoracotomy and thoracoscopy, with the approach chosen influenced by training and institutional bias. Thoracotomy with manual palpation of lung surfaces can identify nodules not seen on preoperative imaging, but no clear survival benefit has been demonstrated compared to complete thoracoscopic resection of all visible nodules.

Methods: All member of the American Pediatric Surgical Association were surveyed, and 204 members responded.

Results: Thoracoscopy was the preferred approach of 34% of surgeons for patients with 3 unilateral nodules but only 21% for those with 5 unilateral nodules. Hospital volume did not correlate with operative approach. Localization strategies are used by 37% of surgeons who prefer thoracotomy and 64% who prefer thoracoscopy. Importantly, the vast majority of responding surgeons (84%) expressed a willingness to participate in a randomized controlled trial of thoracotomy versus thoracoscopy.

Conclusion: Findings of this survey of North American pediatric surgeons confirm both the need for, and interest in, a prospective trial to define optimal surgical management of children with osteosarcoma with limited pulmonary metastasis.

Level Of Evidence: V.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.09.060DOI Listing
February 2021

Surgical management of paratesticular rhabdomyosarcoma: A consensus opinion from the Children's Oncology Group, European paediatric Soft tissue sarcoma Study Group, and the Cooperative Weichteilsarkom Studiengruppe.

Pediatr Blood Cancer 2021 Apr 1;68(4):e28938. Epub 2021 Feb 1.

Division Pediatric Surgery, East Carolina University, Greenville, North Carolina, USA.

The treatment of paratesticular rhabdomyosarcoma (PT-RMS) has varied over time and by cooperative group. The International Soft Tissue Sarcoma Database Consortium (INSTRuCT) is a collaboration of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee, European pediatric Soft tissue sarcoma Study Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). The INSTRuCT surgical committee has been given charge of the development of internationally applicable consensus guidelines for the surgical treatment of rhabdomyosarcoma. This clinical consensus opinion document addresses accepted principles and areas of controversy, such as scrotal violation and retroperitoneal nodal evaluation, providing an evidence-based guideline for the surgical treatment for PT-RMS.
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http://dx.doi.org/10.1002/pbc.28938DOI Listing
April 2021

The effects of early anesthesia on neurodevelopment: A systematic review.

J Pediatr Surg 2021 May 19;56(5):851-861. Epub 2021 Jan 19.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States.

Background: There is growing concern regarding the impact of general anesthesia on neurodevelopment in children. Pre-clinical animal studies have linked anesthetic exposure to abnormal central nervous system development, but it is unclear whether these results translate into humans. The purpose of this systematic review from the American Pediatric Surgical Association (APSA) Outcomes and Evidence-Based Practice (OEBP) Committee was to review, summarize, and evaluate the evidence regarding the neurodevelopmental impact of general anesthesia on children and identify factors that may affect the risk of neurotoxicity.

Methods: Medline, Cochrane, Embase, Web of Science, and Scopus databases were queried for articles published up to and including December 2017 using the search terms "general anesthesia and neurodevelopment" as well as specific anesthetic agents. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to screen manuscripts for inclusion in the review. A consensus statement of recommendations in response to each study question was synthesized based upon the best available evidence.

Results: In total, 493 titles were initially identified, with 56 articles selected for full analysis and 44 included for review. Based on currently available developmental assessment tools, a single exposure to general anesthesia does not appear to have a significant effect on general neurodevelopment, although prolonged or multiple anesthetic exposures may have some adverse effects. Exposure to general anesthesia may affect different domains of development at different ages. Regional anesthetic techniques with the addition of dexmedetomidine and/or some intravenous agents may mitigate the risks of neurotoxicity. This approach may be performed safely in some patients and can be considered as an option in selected short procedures.

Conclusion: There is no conclusive evidence that a single short anesthetic in infancy has a detectable neurodevelopmental effect. Data do not support waiting until later in childhood to perform general anesthesia for single short procedures. With the complexities and nuances of different anesthetic methods, patients and procedures, the planning and execution of anesthesia for the pediatric patient is generally best accomplished by an anesthesiologist, ideally a pediatric anesthesiologist.

Type Of Study: Systematic review of level 1-4 studies.

Level Of Evidence: Level 1-4 (mainly level 3-4).
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http://dx.doi.org/10.1016/j.jpedsurg.2021.01.002DOI Listing
May 2021

Appendectomy Versus Observation for Appendicitis in Neutropenic Children With Cancer.

Pediatrics 2021 Feb;147(2)

Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, Seattle, Washington.

Background: Optimal management of neutropenic appendicitis (NA) in children undergoing cancer therapy remains undefined. Management strategies include upfront appendectomy or initial nonoperative management. We aimed to characterize the effect of management strategy on complications and length of stay (LOS) and describe implications for chemotherapy delay or alteration.

Methods: Sites from the Pediatric Surgery Oncology Research Collaborative performed a retrospective review of children with NA over a 6-year period.

Results: Sixty-six children, with a median age of 11 years (range 1-17), were identified with NA while undergoing cancer treatment. The most common cancer diagnoses were leukemia (62%) and brain tumor (12%). Upfront appendectomy was performed in 41% of patients; the remainder had initial nonoperative management. Rates of abscess or perforation at diagnosis were equivalent in the groups (30% vs 24%; = .23). Of patients who had initial nonoperative management, 46% (17 of 37) underwent delayed appendectomy during the same hospitalization. Delayed appendectomy was due to failure of initial nonoperative management in 65% ( = 11) and count recovery in 35% ( = 6). Cancer therapy was delayed in 35% ( = 23). Initial nonoperative management was associated with a delay in cancer treatment (46% vs. 22%, = .05) and longer LOS (29 vs 12 days; = .01). Patients who had initial nonoperative management and delayed appendectomy had a higher rate of postoperative complications ( < .01).

Conclusions: In pediatric patients with NA from oncologic treatment, upfront appendectomy resulted in lower complication rates, reduced LOS, and fewer alterations in chemotherapy regimens compared to initial nonoperative management.
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http://dx.doi.org/10.1542/peds.2020-027797DOI Listing
February 2021

Defining the role of advanced care practitioners in pediatric surgery practice.

J Pediatr Surg 2020 Dec 5. Epub 2020 Dec 5.

Division of Pediatric Surgery, Department of Surgery, Zucker School of Medicine at Hofstra/Northwell, Cohen Children's Medical Center, 1111 Marcus Avenue, New Hyde Park, NY, United States.

Introduction: The role of advanced care practitioners (ACPs) in pediatric surgery is increasingly important and not well described.

Methods: Electronic surveys were sent to pediatric surgery division chiefs within the Children's Hospital Association.

Results: We received 77/163 survey responses (47%). The median number of ACPs per service was 3.0 (range 0-35). ACP number correlated with inpatient census, surgeon number, case volume, trauma centers, intensive care unit status, and fellowship programs but not with presence of residents/hospitalists, hospital setting, or practice type. Nearly all programs incorporated nurse practitioners while almost half utilized physician assistants. Approximately one-third of ACPs were designated for subspecialties (35%) such as trauma and colorectal. Only 9% of centers had surgeon-specific ACPs. ACP responsibilities included both inpatient and outpatient tasks. Nearly all ACPs participated in procedures (89%), mostly bedside (80%). All ACPs worked daytime shifts, with less nights and weekends. Most ACPs billed for services (80%). Satisfaction with ACP coverage was widespread and did not correlate with ACP number. Most respondents felt that ACPs enhance, and not hinder, resident/fellow training (85%).

Conclusion: ACPs are useful adjuncts in pediatric surgery. A better understanding of practice patterns may help optimize utilization to enhance patient care and can be used to advocate for appropriate resources.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.11.030DOI Listing
December 2020

Survival outcomes of patients with localized FOXO1 fusion-positive rhabdomyosarcoma treated on recent clinical trials: A report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.

Cancer 2021 Mar 20;127(6):946-956. Epub 2020 Nov 20.

Cancer and Blood Disease Institute, Children's Hospital Los Angeles, Division of Hematology/Oncology, Department of Pediatrics, Keck School of Medicine, University of Southern California, Los Angeles, California.

Background: The objective of this analysis was to evaluate the clinical factors influencing survival outcomes in patients with localized (clinical group I-III), FOXO1 fusion-positive rhabdomyosarcoma (RMS).

Methods: Patients with confirmed FOXO1 fusion-positive RMS who were enrolled on 3 completed clinical trials for localized RMS were included in the analytic cohort. Outcomes were analyzed using the Kaplan-Meier method to estimate event-free survival (EFS) and overall survival (OS), and the curves were compared using the log-rank test. A Cox proportional hazards regression model was used to perform multivariate analysis of prognostic factors that were significant in the univariate analysis.

Results: The estimated 4-year EFS and OS of 269 patients with localized, FOXO1 fusion-positive RMS was 53% (95% CI, 47%-59%) and 69% (95% CI, 63%-74%), respectively. Univariate analysis revealed that several known favorable clinical characteristics, including age at diagnosis between 1 and 9 years, complete surgical resection, tumor size ≤5 cm, favorable tumor site, absence of lymph node involvement, confinement to the anatomic site of origin, and PAX7-FOXO1 fusion, were associated with improved outcomes. Multivariate analysis identified older age (≥10 years) and large tumor size (>5 cm) as independent, adverse prognostic factors for EFS within this population, and patients who had both adverse features experienced substantially inferior outcomes.

Conclusions: Patients with localized, FOXO1 fusion-positive RMS can be further risk stratified based on clinical features at diagnosis, and older patients with large primary tumors have the poorest prognosis.
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http://dx.doi.org/10.1002/cncr.33334DOI Listing
March 2021

Evolving applications of fluorescence guided surgery in pediatric surgical oncology: A practical guide for surgeons.

J Pediatr Surg 2021 Feb 19;56(2):215-223. Epub 2020 Oct 19.

Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Northwestern University Feinberg School of Medicine, Chicago, IL. Electronic address:

Fluorescence-guided surgery (FGS) is an increasingly available and popular method of visual field augmentation. The basic premise of FGS entails injection of fluorescent indocyanine green (ICG) and subsequent detection with a near-infrared (NIR) camera. For pediatric surgical oncologists, FGS remains experimental but is a promising modality for identifying tumor margins, locating metastases, performing sentinel lymph node biopsies, protecting peritumoral structures of interest, and facilitating reconstruction. Familiarity with basic ICG pharmacokinetics and NIR detection optics is critical for surgeons wishing to judiciously use FGS, as its success is firmly grounded in a thorough understanding of its capabilities and limitations. In this practical guide, we outline several well-described and innovative FGS applications by disease type, including their methods of administration, modes of detection, and typical ICG dosing paradigms. LEVEL OF EVIDENCE: V.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.10.013DOI Listing
February 2021

Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children with Chest Wall Sarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

Ann Surg 2020 Nov 4. Epub 2020 Nov 4.

Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

Objective: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development and (3) patient-reported quality of life in children with sarcoma of the chest wall.

Summary Background Data: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence.

Methods: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008-2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys.

Results: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (IQR = 1-3), and number of ribs resected did not correlate with margin status (p = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, p = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posterior rib resection (HR 8.43; p = 0.003) and increased number of ribs resected (HR 1.78; p = 0.02). Overall, patient-reported quality of life is not impaired following chest wall tumor resection.

Conclusions: Local recurrence occurs in one-quarter of children with chest wall sarcoma and is independent of tumor type. Scoliosis occurs in 13% of patients, but patient-reported quality of life is excellent.
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http://dx.doi.org/10.1097/SLA.0000000000004579DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8093319PMC
November 2020

Lymphadenopathy in children: A streamlined approach for the surgeon - A report from the APSA Cancer Committee.

J Pediatr Surg 2021 Feb 6;56(2):274-281. Epub 2020 Oct 6.

Division of Pediatric Surgery, East Carolina Medical Center, Greenville, NC.

Background/purpose: Lymphadenopathy is a common complaint in children. Pediatric surgeons are often called upon to evaluate, treat, and/or biopsy enlarged lymph nodes. With many nonsurgical causes in the differential diagnosis, the surgeon plays the important role of providing reassurance and timely diagnosis while minimizing the pain and morbidity associated with surgical interventions in children. The purpose of this summary paper is to provide a management guide for surgeons working up children with lymphadenopathy.

Materials/methods: The English language literature was searched for "lymphadenopathy in children". All manuscript types were considered for review, regardless of medical specialty, with emphasis placed on published guidelines, algorithms, and reviews. After thorough review of these manuscripts and cross-referencing of their bibliographies, the attached algorithm was developed, with emphasis on the role and timing of surgical intervention.

Results: The APSA Cancer Committee developed the attached algorithm to fill a gap in the surgical literature. It outlines lymphadenopathy workup and treatment with emphasis on the role and timing of surgical intervention.

Conclusion: This review defines and summarizes the common etiologies and presentations of lymphadenopathy in children, and offers a straightforward algorithm for evaluation of and treatment with an emphasis on malignancy risk and surgical management.

Type Of Study: Summary paper.

Level Of Evidence: Level V.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.09.058DOI Listing
February 2021

Vascular Anomalies - A Preface.

Authors:
Roshni Dasgupta

Semin Pediatr Surg 2020 Oct 16;29(5):150964. Epub 2020 Sep 16.

Division of Pediatric General and Thoracic Surgery Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, United States Phone-513-636-4371, Fax-513-636-7657. Electronic address:

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http://dx.doi.org/10.1016/j.sempedsurg.2020.150964DOI Listing
October 2020

Venous malformations.

Semin Pediatr Surg 2020 Oct 16;29(5):150976. Epub 2020 Sep 16.

Department of Pediatrics, Stanford University School of Medicine, 1000 Welch Rd., Palo Alto, CA 94304, United States. Electronic address:

Venous malformations include a spectrum of slow-flow malformations that together are the most common forms of vascular anomalies. Care of these patients requires a multi-disciplinary approach. Goals of care are to ameliorate symptoms and to preserve function. Use of therapeutic compression garments remains the mainstay of therapy. There are new and promising therapies over the last few years that will be invaluable tools for optimal care of this complex patient population. Advances in medical therapy through inhibition of the mTOR/PI3K/AKT pathway with Sirolimus and more proximal targeted drugs along with advances in sclerotherapy techniques are promising for the long-term improvement and amelioration of symptoms in patients with venous malformations.
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http://dx.doi.org/10.1016/j.sempedsurg.2020.150976DOI Listing
October 2020

Management of visceral vascular anomalies.

Semin Pediatr Surg 2020 Oct 16;29(5):150977. Epub 2020 Sep 16.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Ave, Cincinnati, OH 45229, United States. Electronic address:

Visceral vascular anomalies are common in patients with vascular malformations in other parts of the body and can include lymphatic, venous, and arteriovenous malformations. Depending on the organ or organs involved they may present differently and pose different treatment challenges. Defining the malformation and understanding its extent is paramount in devising management regimens. Medical, interventional, and surgical therapies are often required in combination to treat these complex lesions. There are new and promising advances in the development of therapeutic agents targeting the PI3K/AKT/mTOR pathway. Due to the complex nature of these lesions a coordinated, multi-disciplinary approach is necessary to manage and mitigate symptoms and complications of this diverse group of vascular malformations.
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http://dx.doi.org/10.1016/j.sempedsurg.2020.150977DOI Listing
October 2020

Thoracoscopy vs thoracotomy for the management of metastatic osteosarcoma: A Pediatric Surgical Oncology Research Collaborative Study.

Int J Cancer 2021 Mar 31;148(5):1164-1171. Epub 2020 Aug 31.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital, Cincinnati, Ohio, USA.

Complete surgical resection of pulmonary metastatic disease in patients with osteosarcoma is crucial to long-term survival. Open thoracotomy allows palpation of nodules not identified on imaging but the impact on survival is unknown. The objective of this study was to compare overall survival (OS) and pulmonary disease-free survival (DFS) in children who underwent thoracotomy vs thoracoscopic surgery for pulmonary metastasectomy. A multi-institutional collaborative group retrospectively reviewed 202 pediatric patients with osteosarcoma who underwent pulmonary metastasectomy by thoracotomy (n = 154) or thoracoscopy (n = 48). Results were analyzed by Kaplan-Meier survival estimates and multivariate Cox proportional hazard regression models. With median follow-up of 45 months, 135 (67.5%) patients had a pulmonary relapse and 95 (47%) patients were deceased. Kaplan-Meier analysis showed no significant difference in 5-year pulmonary DFS (25% vs 38%; P = .18) or OS (49% vs 42%, P = .37) between the surgical approaches of thoracotomy and thoracoscopy. In Cox regression analysis controlling for other factors impacting outcome, there was a significantly increased risk of mortality (HR 2.11; P = .027; 95% CI 1.09-4.09) but not pulmonary recurrence (HR 0.96; P = .90; 95% CI 0.52-1.79) with a thoracoscopic approach. However, in the subset analysis limited to patients with oligometastatic disease, thoracoscopy had no increased risk of mortality (HR 1.16; P = .62; 0.64-2.11). In conclusion, patients with metastatic osteosarcoma and limited pulmonary disease burden demonstrate comparable outcomes after thoracotomy and thoracoscopy for metastasectomy. While significant selection bias in these surgical cohorts limits the generalizability of the conclusions, clinical equipoise for a randomized clinical trial in patients with oligometastatic disease is supported.
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http://dx.doi.org/10.1002/ijc.33264DOI Listing
March 2021

Retrospective study of hematologic complications in patients with slow-flow vascular malformations undergoing sclerotherapy.

Pediatr Blood Cancer 2020 10 11;67(10):e28277. Epub 2020 Aug 11.

Hemangioma and Vascular Malformation Center, Division of Interventional Radiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio.

Background: Slow-flow vascular malformations (SFVM) are associated with localized intravascular coagulopathy (LIC), which is characterized by elevated D-dimer and, when severe, hypofibrinogenemia. LIC results in intralesional clotting and hemorrhage and increases risk for significant thrombotic and bleeding complications. Sclerotherapy has been a suggested potential trigger for LIC worsening to disseminated intravascular coagulopathy. Hematologic complications of sclerotherapy in SFVM, along with low-molecular-weight heparin (LMWH) used to prevent worsening LIC, are largely unstudied.

Procedure: Medical records of patients with SFVM and LIC who underwent sclerotherapy at Cincinnati Children's Hospital Medical Center from July 2008 to December 2016 were reviewed for periprocedural hematologic complications. LMWH dose, frequency, and course length were evaluated.

Results: Fifty-nine patients with SFVM and LIC underwent 281 sclerotherapy procedures, of which 86% were in children. Eighty-five percent of patients received periprocedural LMWH, although at various doses and course lengths. No thrombotic complications occurred in children. One adult on LMWH developed pulmonary emboli after sclerotherapy. No major bleeding complications occurred postoperatively. In four patients, fibrinogen dropped below 100 mg/dL post-sclerotherapy, requiring cryoprecipitate. One patient required packed red blood cell (RBC) transfusion for sclerotherapy-induced hemolysis. No intraoperative bleeding or thrombotic events occurred.

Conclusion: LMWH use, at subtherapeutic dosing, was common in this patient population and did not appear to increase risk of significant bleeding before, during, or after sclerotherapy. In children with SFVM, bleeding and thrombotic complications after sclerotherapy appear rare. Although safe, prospective studies are needed to evaluate the efficacy of LMWH to prevent worsening coagulopathy with procedures.
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http://dx.doi.org/10.1002/pbc.28277DOI Listing
October 2020

Can a children's hospital still cut it? Comparing outcomes of pediatric, adolescent and young adult patients undergoing thoracic surgery for lung metastases.

Pediatr Blood Cancer 2020 10 29;67(10):e28434. Epub 2020 Jul 29.

Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Background: Adolescents and young adults (AYAs) with cancer have unique medical challenges compared with younger children and older adults. Dedicated centers have been established to deliver cancer therapy to the AYA population; many of these programs are located in pediatric hospitals. Outcomes of AYA patients on pediatric protocols are generally superior to those on adult protocols. Little is understood about the impact of care within a pediatric environment for surgical care of young adults.

Methods: A retrospective institutional review was performed of patients undergoing thoracic metastectomy between 2012 and 2017. Demographics, procedural factors, cost, and outcomes were analyzed. Patients were divided into two groups: > 18 and <18 years.

Results: Ninety-one procedures were performed: 61.5% (n = 56) were in patients <18 years old and 38.5% (n = 35) were > 18 years old. The median age was 6.5 years for <18 years old and 28 years for > 18 years old. Older patients had a significantly longer operative time on thoracoscopic cases; 91 versus 63 minutes. Fifty percent of the > 18 group had > 1 lesion resected compared with one lesion resected in 80.8% in <18 years old. No significant differences were found between the two groups in the duration of chest tube or length of stay. The AYA group demonstrated more "adult type" comorbidities.

Conclusion: AYA patients have unique developmental and emotional challenges. Surgical intervention in this special population of patients cared for within a pediatric environment shows no significant difference in outcome compared with pediatric patients undergoing the same procedure. AYA patients with "adult type" comorbidities can safely undergo multidisciplinary care including surgery within a pediatric environment without the need to fragment care.
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http://dx.doi.org/10.1002/pbc.28434DOI Listing
October 2020

Reply to Letter to the Editor.

J Pediatr Surg 2020 11 25;55(11):2537-2538. Epub 2020 Jun 25.

Department of Surgery, Division of Urology, University of Colorado School of Medicine and the Children's Hospital of Colorado, Aurora, CO. Electronic address:

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http://dx.doi.org/10.1016/j.jpedsurg.2020.06.026DOI Listing
November 2020

Update on pediatric rhabdomyosarcoma: A report from the APSA Cancer Committee.

J Pediatr Surg 2020 Oct 18;55(10):1987-1995. Epub 2020 Jun 18.

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Background/purpose: Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma.

Methods: A comprehensive review of the current literature on pediatric rhabdomyosarcoma, including the most recent Children's Oncology Group studies and several international collaboratives, was performed by the authors and key findings were summarized in the manuscript.

Results: FOXO1 fusion status is a stronger prognostic factor than histology and is now used for risk stratification in treatment protocols. For assessment of regional nodal involvement, FDG-PET-CT shows poor sensitivity and specificity to detect histologically confirmed nodal metastasis. Thus, surgical assessment of regional lymph nodes is required for rhabdomyosarcoma of the extremities or trunk as well as paratesticular rhabdomyosarcoma in patients ≥10 years of age, although adherence to surgical guidelines remains poor. Hemiscrotectomy performed for scrotal violation in paratesticular rhabdomyosarcoma has not shown an improvement in event free survival and is not recommended.

Conclusions: Surgical and medical treatment strategies for rhabdomyosarcoma in children continue to evolve. This review provides current evidence-based treatment standards with an emphasis on surgical care.

Type Of Study: Review.

Level Of Evidence: Level IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.06.015DOI Listing
October 2020

Pancreaticoduodenectomy for the treatment of pancreatic neoplasms in children: A Pediatric Surgical Oncology Research Collaborative study.

Pediatr Blood Cancer 2020 09 13;67(9):e28425. Epub 2020 Jul 13.

Division of Pediatric Surgery, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada.

Background: To better characterize short-term and long-term outcomes in children with pancreatic tumors treated with pancreaticoduodenectomy (PD).

Methods: Patients 21 years of age or younger who underwent PD at Pediatric Surgical Oncology Collaborative (PSORC) hospitals between 1990 and 2017 were identified. Demographic, clinical information, and outcomes (operative complications, long-term pancreatic function, recurrence, and survival) were collected.

Results: Sixty-five patients from 18 institutions with a median age of 13 years (4 months-22 years) and a median (IQR) follow-up of 2.8 (4.3) years were analyzed. Solid pseudopapillary tumor of the pancreas (SPN) was the most common histology. Postoperative complications included pancreatic leak in 14% (n = 9), delayed gastric emptying in 9% (n = 6), marginal ulcer in one patient, and perioperative (30-day) death due to hepatic failure in one patient. Pancreatic insufficiency was observed in 32% (n = 21) of patients, with 23%, 3%, and 6% with exocrine, or endocrine insufficiencies, or both, respectively. Children with SPN and benign neoplasms all survived. Overall, there were 14 (22%) recurrences and 11 deaths (17%). Univariate analysis revealed non-SPN malignant tumor diagnosis, preoperative vascular involvement, intraoperative transfusion requirement, pathologic vascular invasion, positive margins, and need for neoadjuvant chemotherapy as risk factors for recurrence and poor survival. Multivariate analysis only revealed pathologic vascular invasion as a risk factor for recurrence and poor survival.

Conclusion: This is the largest series of pediatric PD patients. PD is curative for SPN and benign neoplasms. Pancreatic insufficiency is the most common postoperative complication. Outcome is primarily associated with histology.
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http://dx.doi.org/10.1002/pbc.28425DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674210PMC
September 2020

Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): Comprehensive management of a newly diagnosed young adult.

Gynecol Oncol 2020 09 10;158(3):538-546. Epub 2020 Jul 10.

Department of Pathology, University of Cincinnati College of Medicine, United States of America; Division of Pathology, Cincinnati Children's Hospital Medical Center, United States of America.

SCCOHT is an aggressive malignancy linked to alterations of SMARCA4. We describe the diagnosis and therapy of a 32 year old who received multi-agent chemotherapy and underwent a second look operation with HIPEC followed by high-dose chemotherapy with stem cell transplant. Supportive care, oncofertility, and genetic counseling are described.
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http://dx.doi.org/10.1016/j.ygyno.2020.06.160DOI Listing
September 2020

Solitary myofibroma preceding the development of multicentric myofibromatosis: A report of two cases with surveillance recommendations.

Pediatr Blood Cancer 2020 10 3;67(10):e28266. Epub 2020 Jul 3.

Division of Oncology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.

Infantile myofibroma is the most common fibrous tumor of infancy. Despite the frequency of these tumors, the natural history is incompletely understood. We present two cases with a unique pattern of disease: solitary myofibromas with subsequent progression to diffuse myofibromatosis. Given the variable spectrum of disease and the corresponding difference in morbidity and potential mortality based on the extent of disease, we propose surveillance recommendations.
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http://dx.doi.org/10.1002/pbc.28266DOI Listing
October 2020

Pediatric differentiated thyroid carcinoma: An update from the APSA Cancer Committee.

J Pediatr Surg 2020 Nov 11;55(11):2273-2283. Epub 2020 May 11.

Department of Surgery, Boys Town National Research Hospital, Omaha, NE.

Background: Differentiated thyroid carcinomas (DTCs) are rare in young children but represent almost 10% of all malignancies diagnosed in older adolescents.

Methods: This article reviews the recent literature describing surgical therapeutic approaches to pediatric DTC, associated complications, and long-term recurrence and survival outcomes.

Results: Similar to adult thyroid cancers, pediatric DTCs are more common in females and are associated with thyroid nodules, family history of thyroid cancer, radiation exposure, iodine deficiency, autoimmune thyroid disease, and genetic syndromes. Management of thyroid cancers in children involves ultrasound imaging, fine needle aspiration, and surgical resection with treatment decisions based on clinical and radiological features, cytology and risk assessment.

Conclusions: Total thyroidectomy and compartment based resection of clinically involved lymph node basins form the cornerstone of treatment of DTC. There is an evolving literature regarding the use of molecular genetics to inform treatment strategies and the use of targeted therapies to treat iodine refractory and surgically unresectable progressive disease.

Type Of Study: Summary review.

Level Of Evidence: This is a review article of previously published Level 1-5 articles that includes expert opinion (Level 5).
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http://dx.doi.org/10.1016/j.jpedsurg.2020.05.003DOI Listing
November 2020

Impact of local control and surgical lymph node evaluation in localized paratesticular rhabdomyosarcoma: A report from the Children's Oncology Group Soft Tissue Sarcoma Committee.

Int J Cancer 2020 12 11;147(11):3168-3176. Epub 2020 Jul 11.

Department of Surgery, East Carolina University, Greenville, North Carolina, USA.

Paratesticular rhabdomyosarcoma (PT-RMS) carries a favorable prognosis, but questions persist regarding optimal management. Our goal was to determine the importance of primary tumor resection and surgical assessment of retroperitoneal lymph nodes during staging in patients with PT-RMS. We analyzed patients with localized PT-RMS enrolled onto one of four Children's Oncology Group studies (D9602, ARST0331, D9803 or ARST0531). Surgical resection of the primary tumor prior to chemotherapy and radiotherapy was encouraged when possible with retroperitoneal lymph node dissection (RPLND) recommended for patients ≥10 years of age. Among 279 patients (median 8.1 years old), most tumors were resected with negative margins (78.5%) and most patients did not have radiographic enlargement of regional lymph nodes (90.3%). In patients older than 10 years, imaging alone will miss over 51.5% of nodal disease. Five-year event-free survival (EFS) was 92.0% (95% CI 88.4%-95.6%). Sampling ≥7 to 12 retroperitoneal lymph nodes appeared optimal for detecting positive nodes; while there was a trend toward improved EFS among those undergoing template RPLND, this was not statistically significant (P = .068). Age (P = .28), N-stage (P = .39), T-stage (P = .11) and pathologic node involvement (P = .53) were not associated with overall survival. However, older age and larger tumor size had an additive impact on EFS (P = .027) though not overall survival (P = .13). In conclusion, outcomes for patients with PT-RMS are excellent. Reliance on imaging to detect nodal involvement will miss pathologic node involvement and may result in undertreatment. Surgical nodal staging requires at least 7 to 12 nodes to accurately identify patients with regional nodal disease.
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http://dx.doi.org/10.1002/ijc.33143DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7751831PMC
December 2020

Minimally invasive surgery for pediatric renal tumors: A systematic review by the APSA Cancer Committee.

J Pediatr Surg 2020 Nov 31;55(11):2251-2259. Epub 2020 Mar 31.

Department of Pediatric Surgery, University of Michigan, C.S. Mott Children's Hospital, Ann Arbor, MI.

Minimally invasive nephrectomy is performed routinely for adult renal tumors and for many benign pediatric conditions. Although open radical nephroureterectomy remains the standard of care for Wilms tumor and most pediatric renal malignancies, there are an increasing number of reports of minimally invasive surgery (MIS) for those operations as well. The APSA Cancer Committee performed a systematic review to better understand the risks and benefits of MIS in pediatric patients with renal tumors.

Methods: The search focused on MIS for renal tumors in children and followed the Preferred Reporting Items for Systematic Review and Meta-Analysis (PRISMA) checklist. The initial database search identified 491 published articles, and after progressive review of abstracts and full-length articles, 19 were included in this review.

Results: There were two direct comparison studies where open surgery and MIS were compared. The remaining studies reported only on minimally invasive nephrectomy. Across all studies, there were a total of 151 patients, 126 of which had Wilms tumor and 10 patients had RCC. 104 patients had MIS, with 47 patients having open surgery. In the two studies in which open surgery and MIS were directly compared, more lymph nodes were harvested during open surgery (median = 2 (MIS) vs 5 (open); mean = 2.47 (MIS) vs 3.8 (open)). Many noncomparison studies reported the harvest of 2 of fewer lymph nodes for Wilms tumor. Several MIS patients were also noted to have intraoperative spill or positive margins. Survival between groups was similar.

Conclusions: There is a lack of evidence to support MIS for pediatric renal tumors. This review demonstrates that lymph node harvest has been inadequate for MIS pediatric nephrectomy and there appears to be an increased risk for intraoperative spill. Survival data are similar between groups, but follow-up times were inconsistent and patient selection was clearly biased, with only small tumors being selected for MIS.

Type Of Study: Review article.

Level Of Evidence: III.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.03.019DOI Listing
November 2020

Pneumonectomy for Pediatric Tumors-a Pediatric Surgical Oncology Research Collaborative Study.

Ann Surg 2020 Mar 20. Epub 2020 Mar 20.

Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

Objective: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer.

Summary Background Data: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors.

Methods: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival.

Results: Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045) CONCLUSION:: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma.
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http://dx.doi.org/10.1097/SLA.0000000000003795DOI Listing
March 2020

Minimally invasive surgery for abdominal and thoracic neuroblastic tumors: A systematic review by the APSA Cancer committee.

J Pediatr Surg 2020 Nov 20;55(11):2260-2272. Epub 2020 Feb 20.

Department of Surgery, Division of Pediatric Surgery, The Ohio State University College of Medicine, Nationwide Children's Hospital, Columbus, OH. Electronic address:

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.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.02.019DOI Listing
November 2020

Do children and adolescents with completely resected alveolar rhabdomyosarcoma require adjuvant radiation? A report from the Children's Oncology Group.

Pediatr Blood Cancer 2020 05 2;67(5):e28243. Epub 2020 Mar 2.

Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada.

Background: The role of adjuvant radiotherapy (RT) remains unclear in patients with localized, completely resected (group I) alveolar rhabdomyosarcoma (ARMS).

Procedure: Patients with group I ARMS enrolled on any one of three prior Children's Oncology Group (COG) clinical trials (D9602, D9803, or ARST0531) were analyzed. All patients received systemic chemotherapy and 36 Gy adjuvant RT (if given) to the primary site at week 12 or week 4 for D9602/D9803 and ARST0531, respectively.

Results: Thirty-six patients with group I ARMS were treated on D9602 (n = 6), D9803 (n = 17), or ARST0531 (n = 13), of whom 24 (67%) were male. The median age was 4.1 years (range, 0.8-45.8). Twenty (56%) patients had an unfavorable primary site, and 10 (28%) had tumors > 5 cm. FOXO1-fusion status was negative, positive, and unknown in 10 (28%), 15 (42%), and 11 (30%) tumors, respectively. Twenty-two (61%) patients received RT. Overall, the four-year event-free survival (EFS) and overall survival (OS) were 70.8% and 88.3%, respectively. Patients with FOXO1 positivity who received RT had superior EFS compared with those who did not (77.8% vs 16.7%; P = 0.03). Among 10 patients who were FOXO1 negative, the outcome was similar with or without RT.

Conclusions: Although limited by a small sample size, data from this study support the routine use of adjuvant RT in patients with FOXO1-positive disease even after complete resection. Additionally, omitting adjuvant RT is rational for patients with FOXO1-negative ARMS and will be prospectively investigated in the current COG trial ARST1431.
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http://dx.doi.org/10.1002/pbc.28243DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7325864PMC
May 2020

Subspecialization in pediatric surgery: Results of a survey to the American Pediatric Surgical Association.

J Pediatr Surg 2020 Oct 15;55(10):2058-2063. Epub 2020 Feb 15.

Division of Pediatric Surgery, John R. Oishei Children's Hospital, Department of Surgery, University of Buffalo, Buffalo, NY.

Background: Current practice patterns and opinions regarding subspecialization within pediatric surgery are not well known. We aimed to characterize the prevalence of and attitudes surrounding subspecialization within pediatric surgery.

Methods: An anonymous survey regarding subspecialization was distributed to all nonresident members of the American Pediatric Surgical Association.

Results: Of 1118 surveys, we received 458 responses (41%). A majority of respondents labeled themselves 'general pediatric surgeons' (63%), while 34% considered themselves general surgeons with a specific clinical focus, and 3% reported practicing solely within a specific niche. Subspecialists commonly serve as consultants for relevant cases (52%). Common niches included oncology (10%) and anorectal malformations (9%). Subspecialists felt to be necessary included transplant (79%) and fetal (78%) surgeons. Opinions about subspecialization were variable: 41% felt subspecialization improves patient care while 39% believe it is detrimental to surgeon well-roundedness. Only 10% felt subspecialists should practice solely within their subspecialty. Practicing at an academic hospital or fellowship program correlated with subspecialization, while length of time in practice did not.

Conclusion: While pediatric surgeons report that subspecialization may benefit patient care, concerns exist regarding the unfavorable effect it may have on the individual surgeon. A better understanding of how subspecialization affects quality and outcomes would help clarify its utility.

Type Of Study: Review article.

Level Of Evidence: Level V.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.02.006DOI Listing
October 2020