BACKGROUND: There is a reported increased risk of intra-abdominal tumors in children with both syndromic (SH) and isolated idiopathic hemihyperplasia (IH). Recommendations for tumor surveillance have been made, although there is no consensus for frequency and duration of screening. Our objective was to review the incidence of abdominal neoplasms in our pediatric population with SH and IH. METHODS: We reviewed the diagnostic criteria, imaging findings, and any associated syndrome in all patients diagnosed with hemihypertrophy over a 10-year period. RESULTS: One of 10 patients with SH, a child with Beckwith-Wiedemann syndrome, developed a hepatoblastoma resulting in a 10% tumor incidence in patients with SH. Three of the 250 (1.2%) children with IH developed an abdominal neoplasm. One was diagnosed with adrenal carcinoma and the other 2 with Wilms tumor. CONCLUSIONS: We found an increased incidence of abdominal tumors in both SH and IH, however, our incidence of tumors with IH is lower than earlier reported studies. On the basis of this lower 1.2% incidence, the current literature on IH and available molecular genetic testing, it is reasonable to recommend referral of these patients to a clinical geneticist to identify subgroups with a higher risk for tumor development that are more likely to benefit from routine imaging surveillance. LEVEL OF EVIDENCE: II-Retrospective study.
BACKGROUND: There is a reported increased risk of intra-abdominal tumors in children with both syndromic (SH) and isolated idiopathic hemihyperplasia (IH). Recommendations for tumor surveillance have been made, although there is no consensus for frequency and duration of screening. Our objective was to review the incidence of abdominal neoplasms in our pediatric population with SH and IH. METHODS: We reviewed the diagnostic criteria, imaging findings, and any associated syndrome in all patients diagnosed with hemihypertrophy over a 10-year period. RESULTS: One of 10 patients with SH, a child with Beckwith-Wiedemann syndrome, developed a hepatoblastoma resulting in a 10% tumor incidence in patients with SH. Three of the 250 (1.2%) children with IH developed an abdominal neoplasm. One was diagnosed with adrenal carcinoma and the other 2 with Wilms tumor. CONCLUSIONS: We found an increased incidence of abdominal tumors in both SH and IH, however, our incidence of tumors with IH is lower than earlier reported studies. On the basis of this lower 1.2% incidence, the current literature on IH and available molecular genetic testing, it is reasonable to recommend referral of these patients to a clinical geneticist to identify subgroups with a higher risk for tumor development that are more likely to benefit from routine imaging surveillance. LEVEL OF EVIDENCE: II-Retrospective study.
Authors: Inge van der Velpen; Pamela Schendelaar; Evelyn van Pinxteren-Nagler; Chantal M Mouës-Vink Journal: Plast Reconstr Surg Glob Open Date: 2016-04-21