title: Mesenchymal hamartoma or embryonal sarcoma of the liver in childhood: a difficult diagnosis before complete surgical excision.
abstract: BACKGROUND
  Clinical experience shows that the primary diagnosis of mesenchymal hamartoma (MHL) and embryonal sarcoma of the liver (ESL) recurrently is mistaken, leading to inadequate managements. We evaluated the accuracy of the primary diagnosis of those liver tumors, compared with the final histological diagnosis.
  
  
  METHODS
  Records of 25 children (0-16 years, treated 01/1989-01/2013) with final diagnosis of MHL or ESL were analyzed.
  
  
  RESULTS
  Final diagnosis was MHL in 18/25 children (10 solid-cystic, 2 cystic, 6 solid) and ESL in 7/25 (4 solid-cystic, 1 cystic, 2 solid). Only 3/7 ESL patients and 15/18 MHL patients fell into the "typical" age group. In 13/25 children primary diagnosis was based on imaging only. Overall, primary diagnosis was concordant with the final diagnosis in 17/25 patients. Of 99/25 biopsied cases, 4/9 biopsy results exposed the wrong final diagnosis; of cystic-solid masses 4/14 were mistaken, of cystic masses 1/3, of solid masses 3/8.
  
  
  CONCLUSIONS
  Preoperative diagnosis of MHL and ESL is challenging because of atypical clinical presentation, misleading "typical" radiological findings, and difficult interpretation of biopsies. If feasible, complete surgical resection of, in particular, solid-cystic liver masses in the pediatric age group must be aimed for, to get a definitive, final diagnosis, followed by an adequate treatment strategy.
authors.affiliation: Hôpitaux Universitaires de Genève, Department of Pediatrics, Division of Pediatric Surgery, 6 Rue Willy Donzé, 1205 Geneva, Switzerland. Electronic address: barbara.wildhaber@hcuge.ch.
authors.forename: Barbara E
authors.initials: BE
authors.lastname: Wildhaber
authors.affiliation: Hôpitaux Universitaires de Genève, Department of Pediatrics, Division of Pediatric Surgery, 6 Rue Willy Donzé, 1205 Geneva, Switzerland.
authors.forename: Ernesto
authors.initials: E
authors.lastname: Montaruli
authors.affiliation: Hôpitaux Universitaires Paris Sud-Bicêtre, Department of Pediatric Surgery, 78 Rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.
authors.forename: Florent
authors.initials: F
authors.lastname: Guérin
authors.affiliation: Hôpitaux Universitaires Paris Sud-Bicêtre, Department of Pediatric Surgery, 78 Rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Branchereau
authors.affiliation: Hôpitaux Universitaires Paris Sud-Bicêtre, Department of Pediatric Surgery, 78 Rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Martelli
authors.affiliation: Hôpitaux Universitaires Paris Sud-Bicêtre, Department of Pediatric Surgery, 78 Rue du Général Leclerc, 94275 Le Kremlin Bicêtre, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Gauthier
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000097:blood; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D003951:Diagnostic Errors; D005260:Female; D006222:Hamartoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D012509:Sarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor
keywords: Cystic liver tumors; Cystic–solid liver tumors; Diagnosis; Embryonal sarcoma of the liver; Management; Mesenchymal hamartoma of the liver; Solid liver tumors; Surgery