A. Monaco et al., TESTICULAR FEMINIZATION SYNDROME DIAGNOSED IN AN ELDERLY PATIENT AT SURGERY - A CASE-REPORT, Panminerva Medica, 38(3), 1996, pp. 193-198
A case of Morris' syndrome in which the diagnosis has been realized on
ly in old age is reported, A 69 year-old patient, with female external
genitalia and secondary sexual characteristics, was referred to us wi
th a diagnosis of a mass in the right inguinal region, Her personal hi
story was based on a primary amenorrhoea, which was unsuccessfully inv
estigated since she was adolescent, At the age of 63, during surgery f
or a left inguinal hernia realized in another hospital, a testis-like
mass with the spermatic cord was casually found, During our hospitaliz
ation, a surgical removal of the right inguinal mass was performed, an
d the histologic examination showed the presence of a dominant scleroh
yalin testicular tissue without evidence of seminal epithelium and spa
rse focuses of Leydig cells hyperplasia, Besides, the determination of
gonadotropins and sex hormones yielded an increased production of LH,
FSH, estradiol, testosterone and androstenedione. A cytogenetic analy
sis showed a 46, XY karyotype, The diagnosis realized only in old age
has compelled the patient to live all her life, from sexual maturity,
with indecision and doubt, and without a clinical explanation of found
amental utility even from the psychological point of view, Finally, in
our patient the absence of cytologic aspect of malignant transformati
on in the removed testes in a six Sears period, seem fortuitous, It is
always necessary to consider Morris' syndrome among the possible dise
ases causing primary amenorrhoea in the clinical evaluation of young p
henotypic female patients.