PMID- 28375941
OWN - NLM
STAT- MEDLINE
DCOM- 20170922
LR  - 20181110
IS  - 1536-3678 (Electronic)
IS  - 1077-4114 (Linking)
VI  - 39
IP  - 5
DP  - 2017 Jul
TI  - Two Tumors in 1: What Should be the Therapeutic Target? Pediatric Germ Cell Tumor 
      With Somatic Malignant Transformation.
PG  - 388-394
LID - 10.1097/MPH.0000000000000823 [doi]
AB  - BACKGROUND: Germ cell tumors with somatic malignant transformation (GCT with SMT) 
      are rare in children and poorly described. Data are missing to determine if 
      therapies should target the GCT, the SMT compound, or both simultaneously. 
      PATIENTS AND METHODS: A retrospective national study was conducted in the Societe 
      Francaise des cancers de l'Enfant (SFCE) Centers. Medical records from patients 
      aged 0 to 18 years diagnosed with GCT with SMT between 2000 and 2015 were 
      analyzed. Any stages and primary sites were considered as well as synchronous and 
      metachronous cases. RESULTS: Fifteen patients were identified. Thirteen patients 
      had synchronous GCT with SMT. In the latter cases, primaries were ovary (5), 
      mediastinum (3), pineal gland (3), sacrococcyx (1), and parametrium (1). SMT 
      histologies were central primitive neuroectodermal tumor (5), embryonal 
      rhabdomyosarcomas (3) or thyroid papillary adenocarcinoma, leukemia, poorly 
      differentiated carcinoma, mixed sarcomas, and miscellaneous histology (1 case 
      each). Chemotherapy was targeted against the GCT (3), the SMT (6), or both 
      components (3). The last patient received surgery exclusively. Partial or 
      complete response to chemotherapy was observed in 5/10 assessable cases: 2/3 
      patients treated with GCT-dedicated chemotherapy, 3/6 patients treated with 
      SMT-dedicated therapy, and 0/1 treated with combined therapy. In addition, 2 
      patients with mediastinal GCT primary had metachronous SMT, with acute myeloid 
      leukemia and thyroid papillary adenocarcinoma, 8 months and 8 years, 
      respectively, after the diagnoses of GCT. Two patients (1 synchronous and 1 
      metachronous) were cured with surgery exclusively. At the end of follow-up, 6 
      patients died of their disease including all 4 with postsurgical macroscopic 
      residue. CONCLUSIONS: GCT with SMT constitutes a very rare entity in children and 
      adolescents. Surgical removal of the tumor is the cornerstone of the treatment 
      and might be sufficient in selected cases. In the remaining cases, the best 
      management is still unknown and should take into account both components and 
      their respective chemosensitivity. Long-term surveillance is advised for patient 
      with unresected teratoma as late transformation can occur.
FAU - Faure Conter, Cecile
AU  - Faure Conter C
AD  - *Institute of Pediatric Hematology and Oncology  section signDepartment of Pathology, Women 
      and Children's Hospital, Lyon daggerDepartment of Child and Adolescent Cancer, Gustave 
      Roussy Cancer Institute, Villejuif double daggerDepartment of Pediatric Oncology, University 
      Hospital Center of Nantes, Nantes  parallelDepartment of Pediatric Oncology of Timone 
      Children's Hospital, Marseille  paragraph signDepartment of Pediatric Oncology, University 
      Hospital Center of Rouen, Rouen #Department of Pediatric Oncology, Children's 
      Hospital, Toulouse daggerdaggerDepartment of Pediatric, Adolescents, Young Adults, Institut 
      Curie, Paris, France **Department of Pediatric Hematology and Oncology, Lebanese 
      Hospital, University Medical Center, Beirut, Lebanon.
FAU - Fresneau, Brice
AU  - Fresneau B
FAU - Thebaud, Estelle
AU  - Thebaud E
FAU - Bertrand, Amandine
AU  - Bertrand A
FAU - Dijoud, Frederique
AU  - Dijoud F
FAU - Rome, Angelique
AU  - Rome A
FAU - Dumesnil, Cecile
AU  - Dumesnil C
FAU - Castex, Marie Pierre
AU  - Castex MP
FAU - Ghanem, Anguella
AU  - Ghanem A
FAU - Orbach, Daniel
AU  - Orbach D
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Pediatr Hematol Oncol
JT  - Journal of pediatric hematology/oncology
JID - 9505928
SB  - IM
MH  - Adolescent
MH  - *Cell Transformation, Neoplastic/pathology
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - France
MH  - Humans
MH  - Infant
MH  - Male
MH  - *Neoplasms, Germ Cell and Embryonal/mortality/pathology/therapy
MH  - Retrospective Studies
MH  - Surgical Procedures, Operative
MH  - Survival Rate
MH  - Treatment Outcome
EDAT- 2017/04/05 06:00
MHDA- 2017/09/25 06:00
CRDT- 2017/04/05 06:00
PHST- 2017/04/05 06:00 [pubmed]
PHST- 2017/09/25 06:00 [medline]
PHST- 2017/04/05 06:00 [entrez]
AID - 10.1097/MPH.0000000000000823 [doi]
PST - ppublish
SO  - J Pediatr Hematol Oncol. 2017 Jul;39(5):388-394. doi: 
      10.1097/MPH.0000000000000823.