title: Teratoma with a malignant somatic component in pediatric patients: the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience.
abstract: BACKGROUND
  Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse.
  
  
  METHODS
  The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC. Clinical files and pathology specimens were reviewed.
  
  
  RESULTS
  The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1). Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1). Three patients were in stage I, four in stage II, three in stage III, and four in stage IV. All but one patient underwent surgery and only females showed carcinoma components. Nine patients relapsed or progressed and eight died. Six patients are alive and disease-free. Two patients underwent complete resection and four were treated based on transformed histologies. Relapse-free and overall survival rates were 35.7% and 42.8%, respectively (median follow-up, 31 months).
  
  
  CONCLUSIONS
  Prognosis for germ cell tumors (GCTs) containing MSC is worse than that for GCTs. The pediatric disease appears to be more heterogeneous in tumor site distribution and MSC histology than in adults. Our series suggests no effects of age, histology, or gender on outcome. Surgery has an essential role in localized disease, with complete resection highly desirable. Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive. Malignant GCT warrants GCT-directed chemotherapy.
authors.affiliation: Pediatric Oncology Unit, Fondazione IRCCS National Cancer Institute, Milano, Italy. monica.terenziani@istitutotumori.mi.it
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Renata
authors.initials: R
authors.lastname: Boldrini
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Massimo
authors.initials: M
authors.lastname: Conte
authors.forename: Tina
authors.initials: T
authors.lastname: De Laurentis
authors.forename: Ilaria
authors.initials: I
authors.lastname: Ilari
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Inserra
authors.forename: Luigi
authors.initials: L
authors.lastname: Piva
authors.forename: Fortunato
authors.initials: F
authors.lastname: Siracusa
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Paolo
authors.initials: P
authors.lastname: Tamaro
authors.forename: Margherita
authors.initials: M
authors.lastname: Lo Curto
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007558:Italy; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D013724:Teratoma / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome