期刊
JOURNAL OF PEDIATRIC SURGERY
卷 57, 期 3, 页码 430-442出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.jpedsurg.2021.08.008
关键词
Cancer predisposition syndromes; Solid tumors; Pediatric surgical oncology; Inherited cancer syndromes; Familial cancer
Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms' tumor, hepatoblastoma, and medullary thyroid cancer. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available.
Background/Purpose: Cancer predisposition syndromes (CPS) are a heterogeneous group of inherited dis-orders that greatly increase the risk of developing malignancies. CPS are particularly relevant to pediatric surgeons since nearly 10% of cancer diagnoses are due to inherited genetic traits, and CPS often contribute to cancer development during childhood. Materials/Methods: The English language literature was searched for manuscripts, practice guidelines, and society statements on cancer predisposition syndromes in children. Following review of these manuscripts and cross-referencing of their bibliographies, tables were created to summarize findings of the most common CPS associated with surgically treated pediatric solid malignancies. Results: Pediatric surgeons should be aware of CPS as the identification of one of these syndromes can completely change the management of certain tumors, such as WT. The most common CPS associated with pediatric solid malignancies are outlined, with an emphasis on those most often encountered by pediatric surgeons: neuroblastoma, Wilms' tumor, hepatoblastoma, and medullary thyroid cancer. Fre-quently associated non-tumor manifestations of these CPS are also included as a guide to increase sur-geon awareness. Screening and management guidelines are outlined, and published genetic testing and counseling guidelines are included where available. Conclusion: Pediatric surgeons play an important role as surgical oncologists and are often the first point of contact for children with solid tumors. In their role of delivering a diagnosis and developing a follow-up and treatment plan as part of a multidisciplinary team, familiarity with common CPS will ensure evidence-based practices are followed, including important principles such as organ preservation and intensified surveillance plans. This review defines and summarizes the CPS associated with common childhood solid tumors encountered by the pediatric surgeon, as well as common non-cancerous disease stigmata that may help guide diagnosis. 'Type of study: Summary paper. Level of evidence: 5.
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