A prepubertal girl with pure gonadal dys genesis and 46, XY karyotype is reported. Ex ploratory laparotomy was performed and bilateral gonadal streaks and a small left gonadal tumor were found at the place of the ovaries. The pathologic examination of this tumor revealed gonadoblastoma. The indication to laparotomy in patients with pure gonadal dysgenesis and 46, XY karyotype is discussed.
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References
1.
Boczkowski, K. and Teter, J.: Clinical, histologic, and cytogenetic observations in intersexuality. Obstet. Gynecol.27: 7, 1966.
2.
Borghi, A., Montali, E., Bigozzi, U. and Giusti, G.: XO/XY mosaicism in a phenotypic female with gonadoblastoma. Attempt of classification of the clinical picture of the XO/XY mosaics. Helv. Paediatr. Acta20: 185, 1965.
3.
Cohen, M.M. and Shaw, M.W.: Two XY siblings with gonadal dysgenesis and a female karyotype. N. Engl. J. Med.272: 1083, 1965.
4.
Ferrier, P.E. , Ferrier, S.A., Schärer, K.O., Genton, N., Hedinger, C. and Klein, D.: Disturbed gonadal differentiation in a child with XO/XY/XYY mosaicism: relationship with gonadoblastoma. Helv. Paediatr. Acta22: 479, 1967.
5.
Fine, G., Mellinger, R.C. and Canton, J.N.: Gonadoblastoma occurring in a patient with familial gonadal dysgenesis. J. Clin. Pathol.38: 615, 1962.
6.
Frasier, S.D. , Bashore, R.A. and Mosier, H.D.: Gonadoblastoma associated with pure gonadal dysgenesis in monozygous twins. J. Pediatr.64: 740, 1964.
7.
Goldberg, H.B. and Scully, A.L.: Gonadal malignancy in gonadal dysgenesis: papillary pseudomucinous cystadenocarcinoma in a patient with Turner's syndrome . J. Clin. Endocrinol.27: 341, 1967.
8.
Josso, N., Nezelof, C., Picon, R., de Grouchy, J., Dray, F. and Rappaport, R.: Gonadoblastoma in gonadal dysgenesis: a report of two cases with 46, XY/45, X mosaicism. J. Pediatr.74: 425, 1969.
9.
McDonough, P.C. , Greenblatt, R.B., Byrd, J.R. and Hastigs, E.V.: Gonadoblastoma (gonocytoma III). Report of a case. Obstet. Gynecol.29: 54, 1967.
10.
Naidu, P.N., Ramaswamy, S. and Rad, K.S.: Gonadoblastoma arising from testicular dysplasia
