Adrenocortical carcinoma (ACC) and colorectal cancer (CRC) are rare tumors that can be seen in pediatric and adolescent patients. Both are associated with underlying cancer predisposition syndromes, most commonly Li-Fraumeni syndrome for ACC and familial adenomatous polyposis and Lynch syndrome for CRC. The mainstay of treatment for these diseases is surgical resection, with chemotherapy and other targeted therapies being used for advanced disease. Pediatric CRC and ACC have distinct molecular characteristics from those of their adult counterparts, and should be managed at specialized centers with a multidisciplinary approach to optimize outcomes.
Wilms tumor (WT) is the most common pediatric renal malignancy, characterized by triphasic histology and strong links to disrupted kidney development. Advances in molecular profiling and international collaboration have refined risk stratification using histology, stage, and biomarkers. Treatment combines surgery, chemotherapy, and radiation, with protocol differences between Children's Oncology Group (upfront nephrectomy) and International Society of Pediatric Oncology (preoperative chemotherapy). Nephron-sparing surgery is prioritized for bilateral disease to preserve renal function. Despite excellent survival for favorable histology WT, diffuse anaplasia and adverse molecular features require intensified therapy. Emerging trials aim to reduce toxicity while maintaining cure rates.
Neuroblastoma (NB) is the most common extracranial pediatric solid tumor. The most common location for development of NB is in the adrenal glands, but primary tumors may develop along the developmental pathway of neural crest progenitor cells. NB may vary in severity and prognosis from low-risk congenital neuroblastic lesions to aggressive high-risk neuroblastoma. Therapy is risk-based with intensification of therapy for those at greater risk and de-escalation for patients with lower risk tumors. Surgical resection has a critical role in local control of NB for the most optimal outcome.
This article aims to summarize the fundamentals of pediatric and adolescent extracranial germ cell tumors (GCTs) and to review current best practices regarding surgical and overall management. An understanding of the principles of surgical management of GCTs is critical to ensure optimal outcomes for patients. Details of surgical management vary based on tumor site of origin, but the role of surgery is central to ensuring adequate treatment and staging for tumors of all locations. For pediatric patients, the role of surgery is to maximize chances for disease cure while also maintaining organ function, including future fertility.
Differentiated thyroid carcinoma (DTC) and melanoma are 2 rare cancers seen in pediatric patients. Both pediatric DTC and melanoma present with more advanced disease than their adult counterparts but also have improved long-term outcomes compared to adults. The primary treatment for DTC is surgery with compartment-based lymph node dissection in patients with clinically involved nodes. Wide local excision is the foundation of melanoma treatment, with sentinel lymph node biopsy and completion lymph node dissection based on disease characteristics. For both pediatric melanoma and thyroid carcinoma, molecular testing is an important component for diagnosis, risk stratification, and targeted therapies.
Substantial recent progress has been made in the management of osteosarcoma and Ewing sarcoma, the most common childhood bone sarcomas. Surgical treatment continues to evolve through navigation, 3D printing, biomaterials, and custom-growing implants. New targeted therapies on the horizon have the potential to achieve a breakthrough improvement in survivorship. Newer radiation modalities, such as proton and carbon-ion radiotherapy, have greater therapeutic potential than conventional photon-beam radiotherapy. This article provides a comprehensive review of common childhood sarcomas and highlights recent advances.
