Abstract
The presence of the Y chromosome in the karyotype of patients with disorders of sex differentiation is significantly associated with an increased risk to develop specific types of malignancies, predominantly type II germ cell tumors (GCTs). Gonadoblastoma in the gonads without an obvious testicular differentiation and intratubular germ cell neoplasia of unclassified type in testicular tissue are the precursor lesions of most GCTs. Gonadal dysgenesis, the characteristic feature of Ullrich-Turner syndrome (UTS), further contributes to increase this tumor risk. The reported incidence of Y chromosome material in UTS is 6 to 8% and in these cases an early gonadectomy is strongly recommended to prevent the risk of a malignancy. The aim of this work was to retrospectively analyze the clinical outcome and the histopathological and cytogenetic findings of our UTS patients who underwent gonadectomy to establish strict selection criteria aimed at promoting an organ-sparing surgery.
| Original language | English |
|---|---|
| Pages (from-to) | 273-6 |
| Number of pages | 4 |
| Journal | European Journal of Pediatric Surgery |
| Volume | 26 |
| Issue number | 3 |
| DOIs | |
| Publication status | Published - Jun 2016 |
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Keywords
- Journal Article
Cite this
Ullrich-Turner Syndrome and Tumor Risk : Is There Another Chance to Early Gonadectomy in Positive TSPY and SRY Patients? / Silveri, Massimiliano; Grossi, Armando; Bassani, Francesca; Orazi, Cinzia; Camassei, Francesca Diomedi; Zaccara, Antonio.
In: European Journal of Pediatric Surgery, Vol. 26, No. 3, 06.2016, p. 273-6.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Ullrich-Turner Syndrome and Tumor Risk
T2 - Is There Another Chance to Early Gonadectomy in Positive TSPY and SRY Patients?
AU - Silveri, Massimiliano
AU - Grossi, Armando
AU - Bassani, Francesca
AU - Orazi, Cinzia
AU - Camassei, Francesca Diomedi
AU - Zaccara, Antonio
N1 - Georg Thieme Verlag KG Stuttgart · New York.
PY - 2016/6
Y1 - 2016/6
N2 - The presence of the Y chromosome in the karyotype of patients with disorders of sex differentiation is significantly associated with an increased risk to develop specific types of malignancies, predominantly type II germ cell tumors (GCTs). Gonadoblastoma in the gonads without an obvious testicular differentiation and intratubular germ cell neoplasia of unclassified type in testicular tissue are the precursor lesions of most GCTs. Gonadal dysgenesis, the characteristic feature of Ullrich-Turner syndrome (UTS), further contributes to increase this tumor risk. The reported incidence of Y chromosome material in UTS is 6 to 8% and in these cases an early gonadectomy is strongly recommended to prevent the risk of a malignancy. The aim of this work was to retrospectively analyze the clinical outcome and the histopathological and cytogenetic findings of our UTS patients who underwent gonadectomy to establish strict selection criteria aimed at promoting an organ-sparing surgery.
AB - The presence of the Y chromosome in the karyotype of patients with disorders of sex differentiation is significantly associated with an increased risk to develop specific types of malignancies, predominantly type II germ cell tumors (GCTs). Gonadoblastoma in the gonads without an obvious testicular differentiation and intratubular germ cell neoplasia of unclassified type in testicular tissue are the precursor lesions of most GCTs. Gonadal dysgenesis, the characteristic feature of Ullrich-Turner syndrome (UTS), further contributes to increase this tumor risk. The reported incidence of Y chromosome material in UTS is 6 to 8% and in these cases an early gonadectomy is strongly recommended to prevent the risk of a malignancy. The aim of this work was to retrospectively analyze the clinical outcome and the histopathological and cytogenetic findings of our UTS patients who underwent gonadectomy to establish strict selection criteria aimed at promoting an organ-sparing surgery.
KW - Journal Article
U2 - 10.1055/s-0035-1551568
DO - 10.1055/s-0035-1551568
M3 - Article
C2 - 25978024
VL - 26
SP - 273
EP - 276
JO - European Journal of Pediatric Surgery
JF - European Journal of Pediatric Surgery
SN - 0939-7248
IS - 3
ER -