title: Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm.
abstract: About 80% of children treated for acute lymphoblastic leukemia (ALL) will be long-term survivors. Second malignant neoplasm (SMNs) are a devastating sequelae observed on these children, with an estimated cumulative risk of 2-3.3% fifteen years after diagnosis. Primitive neuroectodermal tumor of bone (PNET) is rarely observed as a SMN following treatment of childhood ALL. The authors described the occurrence of a chest wall PNET of the bone at the site of a central line placement associated with both germ-line and tumor cell p53 mutation in a 8-year-old boy 1 year after completing therapy for standard risk ALL. A review of the literature of 25,051 children treated for ALL discovered 230 SMNs (0.99%), and only one case of PNET of the bone was noted among this group. The occurrence of a SMN in children treated for ALL is a rare event. Such an occurrence, in particular the development of an unusual SMN, should be evaluated for a germline p53 mutation.
authors.affiliation: Department of Pediatrics, Section of Hematology/Oncology, University of Louisville School of Medicine, Louisville, Kentucky, USA. csuarez@psm.edu
authors.forename: Carlos R
authors.initials: CR
authors.lastname: Suarez
authors.forename: Salvatore J
authors.initials: SJ
authors.lastname: Bertolone
authors.forename: Ashok B
authors.initials: AB
authors.lastname: Raj
authors.forename: Susan
authors.initials: S
authors.lastname: Coventry
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D006801:Humans; D008297:Male; D009154:Mutation; D016609:Neoplasms, Second Primary / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000473:pathology; D035441:Thoracic Wall / Q000378:metabolism / Q000473:pathology*; D016896:Treatment Outcome; D016159:Tumor Suppressor Protein p53 / Q000235:genetics*
chemical_list: D016159:Tumor Suppressor Protein p53