Incomplete testicular feminization syndrome.
نویسندگان
چکیده
A three and half year old girl presented to us with bilateral symmetrical swellings in the upper part of each labium majus. These were noted by her mother at birth. There was no history of any case of abnormal genitalia or inguinal hernia in her maternal aunts. Neither was there any record of intake of drugs like alcohol, hydantoins or cimetidine by the mother during pregnan On physical examination the girl was of normal height and weight according to her age. Abdominal examination was normal. Examination of the genitalia revealed a normal clitoris and labium minora except for two symmetrically placed, mobile, nontender and irreducible swellings 2x2 cm in the upper part of each labium majus. The depth of normally placed vagina was 2.5 cm. A separate urethral opening was present. Rectal examination was done and uterus was not palpable. The age-adjusted testosterone level was raised as compared to that for a normal girl, i.e., 20 ng/dl (normal prepubertal: 1-11 ng/dl). The barr body test was negative and the karyo-type was XY. Ultrasonography failed to outline the uterus, cervix, fallopian tubes or ovaries. Intravenous urography showed normal appearance of the kidneys, ureters and bladder. The . swellings were explored surgically and on gross examination they appeared to be testes: They had epididymes and vas deferens. Wedge biopsies were taken from both sides. Histopathology confirmed the presence of testicular tissue. It consisted of variable sized juvenile seminiferous tubules lined by measurable number of sertoli cells which showed no functional activity. The intervening stroma was thick and fibrous and contained scattered leydig cells. Considering all these facts a diagnosis of incomplete testicular feminization syndrome was made and bilateral orchidectomy was performed at a later date.
منابع مشابه
Seminoma arising in androgen insensitivity syndrome (testicular feminization syndrome): A case report
Androgen insensitivity (testicular feminization) syndrome is a rare inherited form of male pseudohermaphroditism that occurs in phenotypically normal woman with male karyotype (XY). The undescended testis may go into malignant transformation. The androgen insensitivity syndrome with malignant testicular disorder is very rare. A thirty-one year old female was admitted to the hospital with the co...
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We report a case of intra-abdominal testicular tumor in a 36-year-old married lady presenting with chief complaints of primary amenorrhea. The patient was later diagnosed with testicular feminization syndrome, a form of male pseudohermaphroditism. This testicular tumor was histologically proven as seminoma. Due to rarity, imaging findings in patients with testicular feminization syndrome and in...
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INTRODUCTION AND OBJECTIVES Testicular feminization is the syndrome when a male, genetically XY, because of various abnormalities of the X chromosome, is resistant to the actions of the androgen hormones, which in turn stops the forming of the male genitalia and gives a female phenotype. The androgen insensitivity syndrome occurs in one out of 20,000 births and can be incomplete (various sexual...
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ورودعنوان ژورنال:
- JPMA. The Journal of the Pakistan Medical Association
دوره 42 7 شماره
صفحات -
تاریخ انتشار 1992