[Skip to Content]
Sign In
Individual Sign In
Create an Account
Institutional Sign In
OpenAthens Shibboleth
Purchase Options:
[Skip to Content Landing]
Article
August 4, 1962

Gonadal Dysgenesis with Male Chromatin Pattern: Testicular Feminization Syndrome: Clinical and Pathological Findings in Two Cases in Siblings

Author Affiliations

Bethlehem, Pa.
From the departments of obstetrics and gynecology (Dr. Flor), pathology (Dr. Benz), and medicine (Dr. Schadt), of St. Luke's Hospital.

JAMA. 1962;181(5):375-379. doi:10.1001/jama.1962.03050310015003
Abstract

Two cases of testicular feminization syndrome in siblings were studied from their clinical, surgical, and pathologic aspects. When the endocrine, pathological, and gynecologic findings were correlated, these cases were found difficult to classify. Primitive gonadal elements did not correspond to any normal stages of development. The authors believe they represent individual examples of a broad spectrum of related intersex disorders. As such, these 2 cases and other similar cases are best designated "gonadal dysgenesis." Because of the reported association of gonadal neoplasms, surgical extirpation of gonadal tissue followed by maintenance on small doses of synthetic estrogens was the treatment chosen for these patients.

References
1.
Morris, J. M.:  Syndrome of Testicular Feminization in Male Pseudohermaphrodites ,  Amer J Obstet Gynec 65:1192-1211 ( (June) ) 1953.
2.
Zuspan, E. P.:  Testicular Feminization Syndrome in Male Pseudohermaphroditism. A Report of its Occurrence in Successive Siblings;  Amer J Obstet Gynec 80:454-458 ( (Sept.) ) 1960.
3.
Lubs, H. A., Jr; Vilar, O.; and Bergenstal, D. M.:  Familial Male Pseudohermaphrodism with Labial Testes and Partial Feminization. Endocrine Studies and Genetic Aspects ,  J Clin Endocr 19:1100-1120 ( (Sept.) ) 1959.Crossref
4.
Melicow, M. M., and Uson, Aurelio C.:  Dysgenetic Gonadomas and Other Gonadal Neoplasms in Intersexes ,  Cancer 12:552-572 ( (May) -June) 1959.Crossref
5.
Barr, M. L.:  Sex Chromatin and Phenotype in Man ,  Science 130:679-685 ( (Sept. 18) ) 1959.Crossref
6.
Dockerty, M. B.: Personal communication to the authors.
7.
Mostofi, F. K.; Theiss, E. A.; and Ashley, D. J.:  Tumors of Specialized Gonadal Stroma in Human Male Patients. Androblastomas Sertoli Cell Tumor, Granulosa— Theca Cell Tumor of the Testes and Gonadal Stromal Tumor ,  Cancer 12:944-957 ( (Sept.) -Oct.) 1959.Crossref
8.
Newman, S.; Diraimondo V. C.; and Kolb, F. O.:  Current Concepts of Sexual Abnormalities .  Postgrad Med 27:488-499 ( (April) ) 1960.
×