THE RISK FOR DEVELOPING TUMOR IN PATIENTS WITH GONADAL DYSGENESIS 46,XY
https://doi.org/10.20538/1682-0363-2015-5-41-46
Abstract
Disorders of sex development with Y chromosome material has been associated with a high risk for developing germ cell tumors such as gonadoblastoma or dysgerminoma. The aim of the study was to investigate the risk of germ cell tumors in patients with XY gonadal dysgenesis. Material and methods. In the study included 11 patients with XY gonadal dysgenesis: 4 with complete gonadal dysgenesis and 4 with partial gonadal dysgenesis. Results. Pathologic examination revealed gonadoblastoma and dysgerminoma with gonadoblastoma in 2 of 9 (22%) patients (who had gonadectomy), including 2 of 3 patients (66 %) with complete gonadal dysgenesis. In our series of patients with gonadal dysgenesis 46,XY the risk of germ cell tumors was high, especially in patients with complete gonadal dysgenesis. Considering this results, early gonadectomy is strongly recommended in females patients. The patients with partial dysgenesis with scrotal gonads being reared as males need monitoring.
About the Authors
O. Yu. LatyshevRussian Federation
Latyshev Oleg Yu.
L. N. Samsonova
Russian Federation
Samsonova Lyubov N.
E. P. Kasatkina
Russian Federation
Kasatkina Elvira P.
G. F. Okminyan
Russian Federation
Okminyan Goar F.
Ye. V. Kiselyova
Russian Federation
Kiselyova Yelena V.
Ye. S. Timofeyeva
Russian Federation
Timofeyeva Yekaterina S.
References
1. Pasterski V., Prentice P., Hughes I.A. Consequences of the Chicago consensus on disorders of sex development (DSD): current practices in Europe. Arch. Dis. Child., 2010, vol. 95 (8). pp. 618–623.
2. Lee P.A., Ahmed F.S., Houk C.P., Hughes I.A. Consensus Statement on Management of Intersex Disorders. Pediatrics, 2006, vol. 118, no. 2, pp. 753–758.
3. Douglas G., Aelrad M.E., Brandt M.L., Grabtree E., Di-etrich J.E., French S. Consensus in guildelines for evaluation of DSD by Texas Children Hospital Multidisciplinary Gender Medicine Team. Int. Pediatr Endocrinol., 2010, 2010:919700.
4. Wunsch L., Holrerhus P.M., Wessel L. Patients with disorders of sex development (DSD) at risk of gonadal tumor de-velopment: management based on laparoscopic biopsy and molecular diagnosis. BJU International, 2012, vol. 110, pp. 958–965.
5. Johansen L.M., Hagen C.P., Rajpert-De M.E. 45,X/46,XY mosaicism: phenotypic characteristics, growth, and repro-ductive function – a retrospective longitudinal study. J. Clin. Endocrinol. Metab., 2012, vol. 97, pp. 1540–1549.
6. Matsumoto F., Shimada K., Ida S. Tumors of bilateral streak gonads in patients with disorders of sex development con-taining Y chromosome material. Clin. Pediatr. Endocrinol., 2014, vol. 23 (3), pp. 93–97.
Review
For citations:
Latyshev O.Yu., Samsonova L.N., Kasatkina E.P., Okminyan G.F., Kiselyova Ye.V., Timofeyeva Ye.S. THE RISK FOR DEVELOPING TUMOR IN PATIENTS WITH GONADAL DYSGENESIS 46,XY. Bulletin of Siberian Medicine. 2015;14(5):41-46. (In Russ.) https://doi.org/10.20538/1682-0363-2015-5-41-46