Research

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23 pages, 9820 KiB

Open AccessArticle

Rhabdomyosarcoma and Extraosseous Ewing Sarcoma

by Juan P. Gurria and Roshni Dasgupta

Children 2018, 5(12), 165; https://0-doi-org.brum.beds.ac.uk/10.3390/children5120165 - 10 Dec 2018

Cited by 24 | Viewed by 9826

Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of [...] Read more.

Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of 4.5 cases per 1,000,000 children. The known histological diagnosis of the two major subtypes (embryonal and alveolar) has been recently enhanced by tumor biological markers and molecular differentiation diagnostic tools that have improved not only the updated classification based on risk stratification, but also the treatment approach based on the clinical group. Ewing sarcoma (ES) is a round cell tumor, highly malignant and poorly differentiated that is currently the second most common malignant bone tumor in children. In rare instances, it develops from an extraskeletal origin, classified as extraosseous Ewing sarcoma (EES). We provide an updated, evidence-based and comprehensive review of the molecular diagnosis, clinical and diagnostic approach and a multidisciplinary medical and surgical management according to the latest standard of care for the treatment of pediatric RMS and EES. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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Review

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9 pages, 1012 KiB

Open AccessReview

Current Management of Pleuropulmonary Blastoma: A Surgical Perspective

by Samantha Knight, Tristan Knight, Amir Khan and Andrew J. Murphy

Children 2019, 6(8), 86; https://0-doi-org.brum.beds.ac.uk/10.3390/children6080086 - 25 Jul 2019

Cited by 35 | Viewed by 6104

Abstract

Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1—related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age [...] Read more.

Pleuropulmonary blastomas (PPB) are pediatric, embryonal cancers of the lung parenchyma and pleural surfaces and are among the most common DICER1—related disorders. These tumors undergo evolution through several forms, allowing division into types I, Ir, II, and III, with correlates to the age of diagnosis and prognosis. We sought to provide a comprehensive review of the relevant literature describing the characteristics of these tumors and their multidisciplinary treatment, with an emphasis on surgical management. We describe the complementary roles of chemotherapy and surgery in the successful management of this disease. We discuss the timing of surgery and options for surgical approaches. We address the differentiation of PPB from congenital pulmonary airway malformation and the role of DICER1 testing for children with pulmonary cysts. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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8 pages, 2618 KiB

Open AccessFeature PaperReview

Neuroblastoma: Tumor Biology and Its Implications for Staging and Treatment

by Kyle J. Van Arendonk and Dai H. Chung

Children 2019, 6(1), 12; https://0-doi-org.brum.beds.ac.uk/10.3390/children6010012 - 17 Jan 2019

Cited by 46 | Viewed by 10721

Abstract

Neuroblastoma, the most common extracranial solid tumor of childhood, has widely variable outcomes dependent on the specific biology of the tumor. In this review, current biologic principles that are used to stratify risk and guide treatment algorithms are discussed. The role for surgical [...] Read more.

Neuroblastoma, the most common extracranial solid tumor of childhood, has widely variable outcomes dependent on the specific biology of the tumor. In this review, current biologic principles that are used to stratify risk and guide treatment algorithms are discussed. The role for surgical resection in neuroblastoma is also reviewed, including the indications and timing of surgery within the greater treatment plan. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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12 pages, 254 KiB

Open AccessReview

Pulmonary Metastasectomy in Pediatric Solid Tumors

by Nicole J. Croteau and Todd E. Heaton

Children 2019, 6(1), 6; https://0-doi-org.brum.beds.ac.uk/10.3390/children6010006 - 08 Jan 2019

Cited by 14 | Viewed by 5432

Abstract

Metastatic disease and the complications of treating metastatic disease are the primary causes of mortality in children with solid malignancies. Nearly 25% of children with solid tumors have metastatic disease at initial diagnosis and another 20% develop metastases during or after treatment. The [...] Read more.

Metastatic disease and the complications of treating metastatic disease are the primary causes of mortality in children with solid malignancies. Nearly 25% of children with solid tumors have metastatic disease at initial diagnosis and another 20% develop metastases during or after treatment. The most common location of these metastases is the lung. The role of surgery in metastatic disease depends greatly on the histology of the primary. In general, tumors that are refractory to adjuvant therapies are most appropriate for pulmonary metastasectomy. This article will summarize the indications for metastasectomy in pediatric solid tumors and discuss the ongoing debate over the technique of metastasectomy in osteosarcoma. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

11 pages, 1460 KiB

Open AccessReview

Hepatoblastoma—The Evolution of Biology, Surgery, and Transplantation

by Irene Isabel P. Lim, Alexander J. Bondoc, James I. Geller and Gregory M. Tiao

Children 2019, 6(1), 1; https://0-doi-org.brum.beds.ac.uk/10.3390/children6010001 - 21 Dec 2018

Cited by 62 | Viewed by 10893

Abstract

The most common primary malignant liver tumor of childhood, hepatoblastoma has increased in incidence over the last 30 years, but little is still known about its pathogenesis. Discoveries in molecular biology provide clues but have yet to define targeted therapies. Disease-free survival varies [...] Read more.

The most common primary malignant liver tumor of childhood, hepatoblastoma has increased in incidence over the last 30 years, but little is still known about its pathogenesis. Discoveries in molecular biology provide clues but have yet to define targeted therapies. Disease-free survival varies according to stage, but is greater than 90% in favorable risk populations, in part due to improvements in chemotherapeutic regimens, surgical resection, and earlier referral to liver transplant centers. This article aims to highlight the principles of disease that guide current treatment algorithms. Surgical treatment, especially orthotopic liver transplantation, will also be emphasized in the context of the current Children’s Oncology Group international study of pediatric liver cancer (AHEP-1531). Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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12 pages, 1206 KiB

Open AccessReview

Minimally Invasive Surgery in Pediatric Surgical Oncology

by Hannah M. Phelps and Harold N. Lovvorn, III

Children 2018, 5(12), 158; https://0-doi-org.brum.beds.ac.uk/10.3390/children5120158 - 26 Nov 2018

Cited by 17 | Viewed by 4618

Abstract

The application of minimally invasive surgery (MIS) to resect pediatric solid tumors offers the potential for reduced postoperative morbidity with smaller wounds, less pain, fewer surgical site infections, decreased blood loss, shorter hospital stays, and less disruption to treatment regimens. However, significant controversy [...] Read more.

The application of minimally invasive surgery (MIS) to resect pediatric solid tumors offers the potential for reduced postoperative morbidity with smaller wounds, less pain, fewer surgical site infections, decreased blood loss, shorter hospital stays, and less disruption to treatment regimens. However, significant controversy surrounds the question of whether a high-fidelity oncologic resection of childhood cancers can be achieved through MIS. This review outlines the diverse applications of MIS to treat pediatric malignancies, up to and including definitive resection. This work further summarizes the current evidence supporting the efficacy of MIS to accomplish a definitive, oncologic resection as well as appropriate patient selection criteria for the minimally invasive approach. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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13 pages, 227 KiB

Open AccessReview

Biological Drivers of Wilms Tumor Prognosis and Treatment

by Hannah M. Phelps, Saara Kaviany, Scott C. Borinstein and Harold N. Lovvorn III

Children 2018, 5(11), 145; https://0-doi-org.brum.beds.ac.uk/10.3390/children5110145 - 26 Oct 2018

Cited by 28 | Viewed by 4656

Abstract

Prior to the 1950s, survival from Wilms tumor (WT) was less than 10%. Today, a child diagnosed with WT has a greater than 90% chance of survival. These gains in survival rates from WT are attributed largely to improvements in multimodal therapy: Enhanced [...] Read more.

Prior to the 1950s, survival from Wilms tumor (WT) was less than 10%. Today, a child diagnosed with WT has a greater than 90% chance of survival. These gains in survival rates from WT are attributed largely to improvements in multimodal therapy: Enhanced surgical techniques leading to decreased operative mortality, optimization of more effective chemotherapy regimens (specifically, dactinomycin and vincristine), and inclusion of radiation therapy in treatment protocols. More recent improvements in survival, however, can be attributed to a growing understanding of the molecular landscape of Wilms tumor. Particularly, identification of biologic markers portending poor prognosis has facilitated risk stratification to tailor therapy that achieves the best possible outcome with the least possible toxicity. The aim of this review is to (1) outline the specific biologic markers that have been associated with prognosis in WT and (2) provide an overview of the current use of biologic and other factors to stratify risk and assign treatment accordingly. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

9 pages, 474 KiB

Open AccessReview

Pediatric Solid Tumors in Resource-Constrained Settings: A Review of Available Evidence on Management, Outcomes, and Barriers to Care

by Nicholas H. Carter, Andrew H. Avery, Jaime Libes, Harold N. Lovvorn III and Erik N. Hansen

Children 2018, 5(11), 143; https://0-doi-org.brum.beds.ac.uk/10.3390/children5110143 - 23 Oct 2018

Cited by 9 | Viewed by 4230

Abstract

International disparities in outcomes from pediatric solid tumors remain striking. Herein, we review the current literature regarding management, outcomes, and barriers to care for pediatric solid tumors in low- and middle-income countries (LMICs). In sub-Saharan Africa, Wilms Tumor represents the most commonly encountered [...] Read more.

International disparities in outcomes from pediatric solid tumors remain striking. Herein, we review the current literature regarding management, outcomes, and barriers to care for pediatric solid tumors in low- and middle-income countries (LMICs). In sub-Saharan Africa, Wilms Tumor represents the most commonly encountered solid tumor of childhood and has been the primary target of recent efforts to improve outcomes in low-resource settings. Aggressive and treatment-resistant tumor biology may play a role in poor outcomes within certain populations, but socioeconomic barriers remain the principal drivers of preventable mortality. Management protocols that include measures to address socioeconomic barriers have demonstrated early success in reducing abandonment of therapy. Further work is required to improve infrastructure and general pediatric care to address disparities. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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13 pages, 1378 KiB

Open AccessReview

Bilateral Wilms Tumor: A Surgical Perspective

by Andrew J. Murphy and Andrew M. Davidoff

Children 2018, 5(10), 134; https://0-doi-org.brum.beds.ac.uk/10.3390/children5100134 - 24 Sep 2018

Cited by 25 | Viewed by 9257

Abstract

Historically, the management of bilateral Wilms tumor (BWT) was non-standardized and suffered from instances of prolonged chemotherapy and inconsistent surgical management which resulted in suboptimal renal and oncologic outcomes. Because of the risk of end-stage renal disease associated with the management of BWT, [...] Read more.

Historically, the management of bilateral Wilms tumor (BWT) was non-standardized and suffered from instances of prolonged chemotherapy and inconsistent surgical management which resulted in suboptimal renal and oncologic outcomes. Because of the risk of end-stage renal disease associated with the management of BWT, neoadjuvant chemotherapy and nephron-sparing surgery have been adopted as the guiding management principles. This management strategy balances acceptable oncologic outcomes against the risk of end-stage renal disease. A recent multi-institutional Children’s Oncology Group study (AREN0534) has confirmed the benefits of standardized 3-drug neoadjuvant chemotherapy and the utilization of nephron-sparing surgery in BWT patients; however, less than 50% of patients underwent bilateral nephron-sparing surgery. The coordination of neoadjuvant chemotherapy and the timing and implementation of bilateral nephron-sparing surgery are features of BWT management that require collaboration between oncologists and surgeons. This review discusses the surgical management strategy in the context of BWT disease biology, with an emphasis on timepoints during therapy at which surgical decision making can greatly impact this disease and minimize long-term toxicities. Full article

(This article belongs to the Special Issue Pediatric Surgical Oncology)

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