title: Histologic Spectrum of Giant Cell Tumor (GCT) of Bone in Patients 18 Years of Age and Below: A Study of 63 Patients.
abstract: Although the majority of giant cell tumors (GCTs) of the bone occur in adult patients, occasionally they arise in the pediatric population. In this setting they may be mistaken for tumors more commonly seen in this age group, including osteosarcoma, aneurysmal bone cyst, and chondroblastoma. All cases of primary GCT of the bone arising in patients 18 years and below were retrieved from our institutional archives and examined with emphasis on the evaluation of various morphologic patterns. Clinical/radiologic records were reviewed when available. Analysis for H3F3A/H3F3B mutations was performed in a subset of cases. Sixty-three (of 710) patients treated at our institution for GCT were 18 years of age and below. The following morphologic patterns were identified: fibrosis (31 cases, 49%), reactive-appearing bone (26, 41%), cystic change (7, 11%), foamy histiocytes (6, 10%), secondary aneurysmal bone cyst (3, 5%), and cartilage (2, 3%). Infarct-like necrosis was present in 17 tumors (27%), and the mitotic rate ranged from 0 to 35 mitoses/10 high-power fields (median 5 mitoses/10 high-power field). Follow-up information (n=55; 6 mo to 69.6 y; median, 11.6 y) showed 21 patients with local recurrence (38%) and 2 patients with lung metastasis (4%). Polymerase chain reaction with sequencing showed that 5 of 5 tested cases harbored H3F3A mutations. In summary, GCT arising in the pediatric population is rare, representing 9% of GCTs seen at our institution. The morphologic spectrum of these tumors is broad and similar to that seen in patients above 18 years of age. It is important to recognize that matrix formation may be observed in GCT, including reactive-appearing bone and cartilage, as well as areas of fibrosis mimicking osteoid production, to avoid misclassification as osteosarcoma or other giant cell-rich lesions common in children.
authors.affiliation: Departments of *Laboratory Medicine and Pathology †Radiology ‡Biomedical Statistics and Informatics §Orthopedic Surgery, Mayo Clinic, Rochester, MN.
authors.forename: Alyaa
authors.initials: A
authors.lastname: Al-Ibraheemi
authors.forename: Carrie Y
authors.initials: CY
authors.lastname: Inwards
authors.forename: Riyam T
authors.initials: RT
authors.lastname: Zreik
authors.forename: Doris E
authors.initials: DE
authors.lastname: Wenger
authors.forename: Sarah M
authors.initials: SM
authors.lastname: Jenkins
authors.forename: Jodi M
authors.initials: JM
authors.lastname: Carter
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Boland
authors.forename: Peter S
authors.initials: PS
authors.lastname: Rose
authors.forename: Long
authors.initials: L
authors.lastname: Jin
authors.forename: Andre M
authors.initials: AM
authors.lastname: Oliveira
authors.forename: Karen J
authors.initials: KJ
authors.lastname: Fritchie
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000235:genetics; D001859:Bone Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018212:Giant Cell Tumor of Bone / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D006657:Histones / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D009154:Mutation; D011379:Prognosis; D016016:Proportional Hazards Models
chemical_list: D014408:Biomarkers, Tumor; C000609950:H3-3A protein, human; D006657:Histones