Riunione Gruppo Italiano Chirurgia Oncologica Pediatrica Genova 18 Settembre 2009 IL “TUMOR RISK” NEI PAZIENTI CON DIFETTI DELLA DIFFERENZIAZIONE SESSUALE (DSD): INTRATUBULAR GERM CELL NEOPLASIA UNCLASSIFIED (IGCNU) IN UN CASO DI DEFICIT DEL MIF (Mϋllerian Inhibiting Factor). Massimiliano Silveri Dipartimento Chirurgia Pediatrica Ospedale Pediatrico “Bambino Gesù” ‐ Palidoro Francesca Diomedi‐Camassei Dipartimento Laboratori ‐ Servizio Anatomia‐Istologia Patologica
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Riunione Gruppo Italiano Chirurgia Oncologica PediatricaGenova 18 Settembre 2009
IL “TUMOR RISK” NEI PAZIENTI CON DIFETTI DELLA DIFFERENZIAZIONE SESSUALE (DSD):INTRATUBULAR GERM CELL NEOPLASIA UNCLASSIFIED (IGCNU) IN UN CASO DI DEFICIT DEL MIF (Mϋllerian Inhibiting Factor).
Skakkebaek NE. “Possible carcinoma in situ of the undescended testis”, Lancet 1992
TIN. Testicular Intraepithelial Neoplasia. It is defined as the presence of malignant germcells within the seminiferous tubules
At 5 years about 50 % of patients with a testicular biopsy positive for IGCNU havedeveloped invasive germ cell tumors and only a small fraction remain free of invasivetumors by 7 years
Orchiectomy is the treatment of choice in patients with unilateral IGCNU, and low‐dose radiation is efficacious in patients with bilateral IGCNU (although sterility is certain).
INTERVENTO 2
ORCHIFUNICOLECTOMIA DX
ISTOLOGIA DEFINITIVA
“neoplasia intratubulare a cellule germinali, non classificata (IGCNU) con focale estensione extratubulare”
Rx torace: NLTC LFN: NL
“Specific subgroups of DSD patients are prone to the developmentOf a germ cell tumor (GCT).
Their individual risk profile can be estimated by combining knowledge on the specific gonadal differentiation patterns present in patients,and detailed histological and genetic diagnostic investigations”.
Cools M, Loojienga HJ. “Disorders of sex development: update on genetic background, terminology and risk for the development of germ cell Tumors”. World J Pediatr 2009; 5(2): 93‐102
RISK CONDITION RISK (%) PROPOSED MANAGEMENT NUMBER OF STUDIES
HIGH GD (Y+), intra‐abdominal 15‐35 Gonadectomy 12
GD (Y+), scrotal ? Biopsy; in case of CIS: irradiation
0
PAIS, scrotal gonads ? Biopsy; in case of CIS: irradiation
0
From: Cools M, Leendert HJ. “Disorders of sex development: update on the genetic background, terminology and riskfor the development of germ cell tumors”. World J Pediatr Vol 5 No 2; May 15, 2009