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Precocious pseudo puberty caused by ovarian tumors. Report of three cases
INTERNATIONAL
GENITOURINARY PRECOCIOUS
PSEUDO
OVARIAN
TRACT
PUBERTY
TUMORS.
CAUSED
REPORT
OF
BY
THREE
R. Niswander, N. G. Courey Woodward. Obstet. Gynec. 26:
CASES. K.
and
T.
381,
1965.
105
ABSTRACTS OF PEDIATRIC SURGERY
The authors report three cases of children; ages 3, 41/2, and 10 months, with precocious puberty caused by ovarian tumors, and they review briefly previously reported cases in the literature. Two of these cases were granulosa cell tumors and the third a thecoma. The authors point out that interpretation of urinary estrogen levels must be made with caution, for an ovarian tumor can be present in a child with precocious puberty even when estrogen excretion is not increased. Most evidences of estrogen stimulation regress after removal of the tumor, although breast regression is not always complete. The comas are usually benign, but the authors point out a previous reference to granulosa cell tumors in children where 2 out of 31 died with metastatic disease.W. H. Hen&en
buccal smears are typical for these patients. Various degrees of Mullerian development are always present. One testis, usually intra-abdominal, is well formed; histologically, Leydig cells are usually present, but spermatogonia are not always found. On the opposite side a gonadal streak composed of fibrous stroma is found. All patients were sex chromatin negative. All had a uterus and vagina. Information on development of the tubes on the side of the testis was available for 22 of 23 patients: in 17, tubes were well developed and in 5, no tubes were present. Information on development of the tubes on the side without a testis was available for 21 patients; in all cases a tube was present. Of the 23 patients, 14 had testicular development on the right side and 11 on the left. In all cases the testis was sufficiently active in spite of unilaterality and dysgenesis to masculinize the external genitalia, giving at least ambiguous structures; in 6 patients this masculinization was sufficient that male rearing had been selected.-W. H. Hdren CRYPTORCHID AND SCROTAL
COMPLETE
MASCULIN~ZATION GENITALIA
TERNAL
OF THE
CONGENITAL
IN
ADREN~CORTICAL
HYPERPLASIA.
TATION
CASES. W.
Baker,
OF Two
H.
J. Ural.
McCrystal
94:266,
and
Ex-
E.
PRESEN-
Maxted, R. Fitzgerald.
1965.
I.
CYTOLOGICAL,
QUANTITATIVE
A. A&ado, 927, 1965.
HUMAN
TESTES.
CYTOCHEMICAL,
STUDIES. R. E.
et al. J.
Clin.
Man&i, Endocr.
AND
J. 25:
Quantitative studies concerning the variation in cell population and cytochemical
Two cases, one a 10 month old infant, and one an adult, are described in females with congenital adrenocortical hyperplasia in whom there was complete fusion of the labioscrotal folds and the urethra was located in the phallus. Buccal smears were chromatin positive. The vaginal orifice was seen on the verumontanum in one patient
characteristics of the undescended testis at different ages were carried out in 58 cases of unilateral cryptorchidism. The cryptor-
and entered just distal sphincter in the other.-W.
teins and fibrotic changes were noted in the stromal connective tissue; these changes also reached a maximum in adulthood. No differences were observed at any age in the Leydig cells of the cryptorchid testes as compared to those of the contralateral testicle or to testes of normal subjects. It is assumed that the arrested development of the germinal epithelium and the progressive alterations observed in the stromal connective tissue are responsible for the irreparable damage observed in the cryptorchid testis in adulthood.-G. Sugarman
CLINICAL,
HISTOLOGIC,
to the external H. Hen&en AND
CYT~CENETIC
FINDINGS IN MALE HERMAPHRODITISM. III. MALE
HERMOPHRODITES WITH ASYMMET-
RICAL GONADAL DIFFERENTIATION GONADAL
DYSGENESIS).
H. W. Jones.
Obstet.
(MIXED
P. A. Zourhs
Gynec.
26~48,
and 1965.
The authors report 3 cases with asymmetric gonadal differentiation and 20 cases from the literature. Short stature, ambiguous exwith chromatin negative ternal genitalia,
chid testis showed arrested development of the germinal epithelium and Sertoli cells which started in early infancy and reached a maximum in adulthood. At puberty, decreased extravascular diffusion of serum pro-
GENITOURINARY PRECOCIOUS
PSEUDO
OVARIAN
TRACT
PUBERTY
TUMORS.
CAUSED
REPORT
OF
BY
THREE
R. Niswander, N. G. Courey Woodward. Obstet. Gynec. 26:
CASES. K.
and
T.
381,
1965.
105
ABSTRACTS OF PEDIATRIC SURGERY
The authors report three cases of children; ages 3, 41/2, and 10 months, with precocious puberty caused by ovarian tumors, and they review briefly previously reported cases in the literature. Two of these cases were granulosa cell tumors and the third a thecoma. The authors point out that interpretation of urinary estrogen levels must be made with caution, for an ovarian tumor can be present in a child with precocious puberty even when estrogen excretion is not increased. Most evidences of estrogen stimulation regress after removal of the tumor, although breast regression is not always complete. The comas are usually benign, but the authors point out a previous reference to granulosa cell tumors in children where 2 out of 31 died with metastatic disease.W. H. Hen&en
buccal smears are typical for these patients. Various degrees of Mullerian development are always present. One testis, usually intra-abdominal, is well formed; histologically, Leydig cells are usually present, but spermatogonia are not always found. On the opposite side a gonadal streak composed of fibrous stroma is found. All patients were sex chromatin negative. All had a uterus and vagina. Information on development of the tubes on the side of the testis was available for 22 of 23 patients: in 17, tubes were well developed and in 5, no tubes were present. Information on development of the tubes on the side without a testis was available for 21 patients; in all cases a tube was present. Of the 23 patients, 14 had testicular development on the right side and 11 on the left. In all cases the testis was sufficiently active in spite of unilaterality and dysgenesis to masculinize the external genitalia, giving at least ambiguous structures; in 6 patients this masculinization was sufficient that male rearing had been selected.-W. H. Hdren CRYPTORCHID AND SCROTAL
COMPLETE
MASCULIN~ZATION GENITALIA
TERNAL
OF THE
CONGENITAL
IN
ADREN~CORTICAL
HYPERPLASIA.
TATION
CASES. W.
Baker,
OF Two
H.
J. Ural.
McCrystal
94:266,
and
Ex-
E.
PRESEN-
Maxted, R. Fitzgerald.
1965.
I.
CYTOLOGICAL,
QUANTITATIVE
A. A&ado, 927, 1965.
HUMAN
TESTES.
CYTOCHEMICAL,
STUDIES. R. E.
et al. J.
Clin.
Man&i, Endocr.
AND
J. 25:
Quantitative studies concerning the variation in cell population and cytochemical
Two cases, one a 10 month old infant, and one an adult, are described in females with congenital adrenocortical hyperplasia in whom there was complete fusion of the labioscrotal folds and the urethra was located in the phallus. Buccal smears were chromatin positive. The vaginal orifice was seen on the verumontanum in one patient
characteristics of the undescended testis at different ages were carried out in 58 cases of unilateral cryptorchidism. The cryptor-
and entered just distal sphincter in the other.-W.
teins and fibrotic changes were noted in the stromal connective tissue; these changes also reached a maximum in adulthood. No differences were observed at any age in the Leydig cells of the cryptorchid testes as compared to those of the contralateral testicle or to testes of normal subjects. It is assumed that the arrested development of the germinal epithelium and the progressive alterations observed in the stromal connective tissue are responsible for the irreparable damage observed in the cryptorchid testis in adulthood.-G. Sugarman
CLINICAL,
HISTOLOGIC,
to the external H. Hen&en AND
CYT~CENETIC
FINDINGS IN MALE HERMAPHRODITISM. III. MALE
HERMOPHRODITES WITH ASYMMET-
RICAL GONADAL DIFFERENTIATION GONADAL
DYSGENESIS).
H. W. Jones.
Obstet.
(MIXED
P. A. Zourhs
Gynec.
26~48,
and 1965.
The authors report 3 cases with asymmetric gonadal differentiation and 20 cases from the literature. Short stature, ambiguous exwith chromatin negative ternal genitalia,
chid testis showed arrested development of the germinal epithelium and Sertoli cells which started in early infancy and reached a maximum in adulthood. At puberty, decreased extravascular diffusion of serum pro-