title: Peripheral primitive neuroectodermal tumor of the stomach in a 14-year-old boy: a case report.
abstract: Although PNETs are rare malignancies, they should be considered in the differential diagnosis of submucosal gastric tumours in adolescents with clinical alarm symptoms.NET was confirmed by detection of the characteristic EWS/FLI-1 fusion gene, resulting in a reciprocal translocation t(11;22)(q24;q12). Three distinct liver metastases were detected by CT, MRI, and PET. The tumour failed to respond to neoadjuvant polychemotherapy with vincristine, etoposide, doxorubicin, and ifosfamide. Subtotal gastrectomy was performed and, surprisingly, we found diffuse metastatic infiltration of the liver that had not been detected by preoperative staging. Due to the diffuse metastatic disease the young patient's prognosis has to be considered very poor. Because of the tumour's intense expression of CD117 (c-kit), the patient is now treated with the tyrosine kinase inhibitor imatinib (STI571).
authors.affiliation: Second Department of Medicine, Bogenhausen Academic Teaching Hospital, Munich, Germany.
authors.forename: Ralph
authors.initials: R
authors.lastname: Czekalla
authors.forename: Martin
authors.initials: M
authors.lastname: Fuchs
authors.forename: Angela
authors.initials: A
authors.lastname: Stölzle
authors.forename: Andreas
authors.initials: A
authors.lastname: Nerlich
authors.forename: Christopher
authors.initials: C
authors.lastname: Poremba
authors.forename: Karl-Ludwig
authors.initials: KL
authors.lastname: Schaefer
authors.forename: Gregor
authors.initials: G
authors.lastname: Weirich
authors.forename: Heinz
authors.initials: H
authors.lastname: Höfler
authors.forename: Folker
authors.initials: F
authors.lastname: Schneller
authors.forename: Christian
authors.initials: C
authors.lastname: Peschel
authors.forename: Jörg Rüdiger
authors.initials: JR
authors.lastname: Siewert
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schepp
mesh_terms: D000293:Adolescent; D016099:Endoscopy, Gastrointestinal / Q000379:methods; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009367:Neoplasm Staging; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000473:pathology / Q000556:secondary / Q000628:therapy; D049268:Positron-Emission Tomography / Q000379:methods; D013274:Stomach Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome