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Estrinism in a girl 2½ years of age
Estrinism in a girl 2% years of age With
minimal
ovarian
w. w.
COPPEDGE,
LEWIS
B.
Atlanta,
changes
suggestive
of thecosis
M.D.
HASTY,
M.D.
Georgia
T H I s is a case report of a white baby whom we delivered on July 12, 1956. The mother’s prenatal course was essentially normal and labor began spontaneously 3 days before the expected date of confinement. It was an uneventful 3 hour labor with a normal spontaneous delivery, this being the third term pregnancy. The birth weight of the infant was 6 pounds, 13 ounces. On March 6, 1959, when the patient was about 21/z years of age, we saw her because of a second episode of vaginal bleeding, the first bleeding having occurred 28 days previously. The mother reported the first bleeding had lasted for 7 days and resembled normal menstrual flow. The mother also reported that the patient had been developing normally except that some slight breast enlargement had been noted for the past 6 months and that the pediatrician had considered this within normal limits until she began to have associated cyclic vaginal bleeding. The past history revealed no serious illnesses, no childhood diseases, and no operations. Examination revealed an alert 2j/2-yearold girl with essentially normal stature and build except for definite symmetrical glandular enlargement of both breasts (Fig. 1). There was slight fuzzy hair on the pubis From the Department Gynecology, Piedmont
of Obstetrics
and
Hospital.
Presented at the Twenty-second Annual Meeting of the South Atlantic Association of Obstetricians and Gynecologists; Hollywood, Florida, Ian. Jl-Feb. 3, 1960.
and in the axillae. Her weight was 40 pounds, height 39f/4 inches, and chest measurement 23f/2 inches. Abdominal examination was essentially normal and rectal examination failed to reveal any pelvic mass or the suggestion of a foreign body in the vagina. The clitoris was not hypertrophied but was believed to be slightly larger than normal for this age group. There was a bloody vaginal discharge on the vulva and the vaginal introitus was patent and easily admitted a cotton-tipped applicator. The tentative diagnosis was granulosa cell tumor and the following studies were obtained: Papanicolaou smear on April 3, 1959 (shortly after third episode of bleeding): “Estrogen level definitely increased. Mature cells. No tumor cells found.” Papanicolaou smear on April 28, 1959, for progesterone effect: “Much greater estrogen effect than expected. No progesterone effect noted.” Friedman test: negative; x-ray examination of long bones: “bone age at least 5 years”; lateral skull films: normal pituitary; 17-ketosteroids: 0.9 mg. per 24 hours (adult normal 3 to 10); 17-OH corticosteroids: 1.2 mg. per 24 hours (adult normal 4 to 12) ; protein-bound iodine: 3.7 fig per cent (normal 3.5 to 8.0) ; urine: reaction 4.5, specific gravity 1.030, albumin, sugar, and acetone negative, microscopic negative; blood count: hemoglobin 12.3 Gm., hematocrit 37.0, white blood count 5,550, 26 segmented cells, 2 eosinophils, 70 lymphocytes, 2 monocytes, and platelets adequate.
638
Coppedge
and
Octobcl, 1’960 i\m. J. Obst. & Gynec.
Hasty
Fig. 1. Breast development
The patient was posted for exploratory laparotomy at about the time she should menstruate so that suction biopsy of the endometrium during the latter phase of the cycle could be obtained under anesthesia. The operation was performed on May il. 1959, at the Piedmont Hospital in Atlanta, Georgia. The cervix was small, clean, and pink, and the uterine cavity was sounded to a depth of 2f/g inches. The suction biopq was done without difficulty and a good strip of endometrium was obtained. A low midline incision was then done and the uterus appeared to be 2 to 3 times the size one would expect in this age group. The tubes were also larger than normal and both ovaries were about 2 times normal size for this age group, measuring about 2.5 by 3 cm. each, glistening white-yellow in colot and polycystic (Fig. 2). There was no evidence of corpus luteum formation in either ovary. No distinct ovarian tumor could be felt. Wedge resections of both ovaries were done with many small follicle cysts seen. The left ovary contained one small 3 mm. well-circumscribed yellow cyst or tumor deep in the ovarian stroma and also a small 2 mm. firm area on its pole. These were widely resected in addition to the generous wedge resection. The adrenal glands, kidneys, and
liver were palpated and considered to be normal. A cyst of Morgagni of the right tube was excised and an appendectomy was performed. The patient withstood the procedure well and her postoperative course was uneventful. She was discharged on the fifth postoperative day. The patient was checked at monthly intcrvals followinn the operation and she had
Fig. 2. Findings ovaries.
at operation
showing
enlarged
Volume Number
80 4
no further vaginal bleeding and her breasts seemed to regress. We assumed at this time that we had removed a small functioning ovarian tumor. The pathologic diagnoses were as follows: the endometrium was consistent with the proliferative phase. The specimen from the pole of the left ovary consisted of ovarian cortical tissue, and blocks from both ovaries showed numerous primordial follicles. Some of the follicles were cystically dilated, accounting for the small cysts noted grossly. In the 3 mm. yellow tumor from the left ovary there was a group of oval or polyhedral cells which had abundant pale cytoplasm and small round basophilic nuclei (Figs. 3 and 4). This group of cells had no sharply defined border. Adjacent to the cellular cluster, a small amount of fibrous tissue was seen. This group of pale cells probably accounted for the yellowish tan appearance noted grossly. No wet tissue remained, hence a fat stain could not be performed. This cellular cluster was interpreted as possibly representing a luteoma. The slides of ovarian tissue were forwarded to Dr. Donald Woodruff at Johns Hopkins Hospital and he reported that he found nothing that would suggest true tumor formation. He felt that “there is rather definite hyperactivity of the ovarian stroma and the patchy arrangement of this would rather suggest that it should be called Yhecosis. ” On Aug. 19, 1959, 3% months after the operation, the patient again started menstrual-type bleeding. Papanicolaou smears at this time continued to show a high estrogen level. Approximately 25 days after the she again had vaginal August bleeding, bleeding. At this time follicle-stimulating hormone and estrogen level studies were obtained from the Department of Medicine of Emory University Medical School at the Grady Memorial Hospital in Atlanta. The estrogen level on a 24 hour urine specimen was 2.7 which is considered a low pg of estriol, normal for a menstruating woman. The follicle-stimulating hormone determination
Estrinism
Fig. 3. power).
in 21/,-year-old
Photomicrograph
Fig. 4. Photomicrograph power)
of
ovarian
of ovarian
girl
tissue
tissue
639
(low
(high
by the Steelman-Pohley method was 3 rat units per day compared to a normal of 4 to 7 units per day in a menstruating woman. Since the follicle-stimulating hormone level is considered to be zero until the age of puberty, the final diagnosis is a constitutional type of precocious puberty rather than some type of functioning ovarian tumor. We
wish
cooperation
to
acknowledge
of Dr. Walter
rector, Piedmont Preedy, Assistant University School
the
assistance
and
Bloom, Research Di-
Hospital, and Dr. John Professor of Medicine, of Medicine.
R. K. Emory
640
Coppedge
and
Hasty
REFERENCES
Morris, John McLean, and Scully, Robert E.: Endocrine Pathology of the Ovary, St. Louis, 1958, The C. V. Mosby Company. Novak, Emil, and Novak, Edmund: Gynecologi< and Obstetric Pathology With Clinical and
‘Endocrine Relations, Philadelphia, 1958. \+‘. B. Saunders Company. Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence, Springfield. Ill.. 1950. Charles C: Thomas, Publisher, pp. 156 I 5 T. Woodruff, Donald : Personal rorrlrnurlicatioI1.
minimal
ovarian
w. w.
COPPEDGE,
LEWIS
B.
Atlanta,
changes
suggestive
of thecosis
M.D.
HASTY,
M.D.
Georgia
T H I s is a case report of a white baby whom we delivered on July 12, 1956. The mother’s prenatal course was essentially normal and labor began spontaneously 3 days before the expected date of confinement. It was an uneventful 3 hour labor with a normal spontaneous delivery, this being the third term pregnancy. The birth weight of the infant was 6 pounds, 13 ounces. On March 6, 1959, when the patient was about 21/z years of age, we saw her because of a second episode of vaginal bleeding, the first bleeding having occurred 28 days previously. The mother reported the first bleeding had lasted for 7 days and resembled normal menstrual flow. The mother also reported that the patient had been developing normally except that some slight breast enlargement had been noted for the past 6 months and that the pediatrician had considered this within normal limits until she began to have associated cyclic vaginal bleeding. The past history revealed no serious illnesses, no childhood diseases, and no operations. Examination revealed an alert 2j/2-yearold girl with essentially normal stature and build except for definite symmetrical glandular enlargement of both breasts (Fig. 1). There was slight fuzzy hair on the pubis From the Department Gynecology, Piedmont
of Obstetrics
and
Hospital.
Presented at the Twenty-second Annual Meeting of the South Atlantic Association of Obstetricians and Gynecologists; Hollywood, Florida, Ian. Jl-Feb. 3, 1960.
and in the axillae. Her weight was 40 pounds, height 39f/4 inches, and chest measurement 23f/2 inches. Abdominal examination was essentially normal and rectal examination failed to reveal any pelvic mass or the suggestion of a foreign body in the vagina. The clitoris was not hypertrophied but was believed to be slightly larger than normal for this age group. There was a bloody vaginal discharge on the vulva and the vaginal introitus was patent and easily admitted a cotton-tipped applicator. The tentative diagnosis was granulosa cell tumor and the following studies were obtained: Papanicolaou smear on April 3, 1959 (shortly after third episode of bleeding): “Estrogen level definitely increased. Mature cells. No tumor cells found.” Papanicolaou smear on April 28, 1959, for progesterone effect: “Much greater estrogen effect than expected. No progesterone effect noted.” Friedman test: negative; x-ray examination of long bones: “bone age at least 5 years”; lateral skull films: normal pituitary; 17-ketosteroids: 0.9 mg. per 24 hours (adult normal 3 to 10); 17-OH corticosteroids: 1.2 mg. per 24 hours (adult normal 4 to 12) ; protein-bound iodine: 3.7 fig per cent (normal 3.5 to 8.0) ; urine: reaction 4.5, specific gravity 1.030, albumin, sugar, and acetone negative, microscopic negative; blood count: hemoglobin 12.3 Gm., hematocrit 37.0, white blood count 5,550, 26 segmented cells, 2 eosinophils, 70 lymphocytes, 2 monocytes, and platelets adequate.
638
Coppedge
and
Octobcl, 1’960 i\m. J. Obst. & Gynec.
Hasty
Fig. 1. Breast development
The patient was posted for exploratory laparotomy at about the time she should menstruate so that suction biopsy of the endometrium during the latter phase of the cycle could be obtained under anesthesia. The operation was performed on May il. 1959, at the Piedmont Hospital in Atlanta, Georgia. The cervix was small, clean, and pink, and the uterine cavity was sounded to a depth of 2f/g inches. The suction biopq was done without difficulty and a good strip of endometrium was obtained. A low midline incision was then done and the uterus appeared to be 2 to 3 times the size one would expect in this age group. The tubes were also larger than normal and both ovaries were about 2 times normal size for this age group, measuring about 2.5 by 3 cm. each, glistening white-yellow in colot and polycystic (Fig. 2). There was no evidence of corpus luteum formation in either ovary. No distinct ovarian tumor could be felt. Wedge resections of both ovaries were done with many small follicle cysts seen. The left ovary contained one small 3 mm. well-circumscribed yellow cyst or tumor deep in the ovarian stroma and also a small 2 mm. firm area on its pole. These were widely resected in addition to the generous wedge resection. The adrenal glands, kidneys, and
liver were palpated and considered to be normal. A cyst of Morgagni of the right tube was excised and an appendectomy was performed. The patient withstood the procedure well and her postoperative course was uneventful. She was discharged on the fifth postoperative day. The patient was checked at monthly intcrvals followinn the operation and she had
Fig. 2. Findings ovaries.
at operation
showing
enlarged
Volume Number
80 4
no further vaginal bleeding and her breasts seemed to regress. We assumed at this time that we had removed a small functioning ovarian tumor. The pathologic diagnoses were as follows: the endometrium was consistent with the proliferative phase. The specimen from the pole of the left ovary consisted of ovarian cortical tissue, and blocks from both ovaries showed numerous primordial follicles. Some of the follicles were cystically dilated, accounting for the small cysts noted grossly. In the 3 mm. yellow tumor from the left ovary there was a group of oval or polyhedral cells which had abundant pale cytoplasm and small round basophilic nuclei (Figs. 3 and 4). This group of cells had no sharply defined border. Adjacent to the cellular cluster, a small amount of fibrous tissue was seen. This group of pale cells probably accounted for the yellowish tan appearance noted grossly. No wet tissue remained, hence a fat stain could not be performed. This cellular cluster was interpreted as possibly representing a luteoma. The slides of ovarian tissue were forwarded to Dr. Donald Woodruff at Johns Hopkins Hospital and he reported that he found nothing that would suggest true tumor formation. He felt that “there is rather definite hyperactivity of the ovarian stroma and the patchy arrangement of this would rather suggest that it should be called Yhecosis. ” On Aug. 19, 1959, 3% months after the operation, the patient again started menstrual-type bleeding. Papanicolaou smears at this time continued to show a high estrogen level. Approximately 25 days after the she again had vaginal August bleeding, bleeding. At this time follicle-stimulating hormone and estrogen level studies were obtained from the Department of Medicine of Emory University Medical School at the Grady Memorial Hospital in Atlanta. The estrogen level on a 24 hour urine specimen was 2.7 which is considered a low pg of estriol, normal for a menstruating woman. The follicle-stimulating hormone determination
Estrinism
Fig. 3. power).
in 21/,-year-old
Photomicrograph
Fig. 4. Photomicrograph power)
of
ovarian
of ovarian
girl
tissue
tissue
639
(low
(high
by the Steelman-Pohley method was 3 rat units per day compared to a normal of 4 to 7 units per day in a menstruating woman. Since the follicle-stimulating hormone level is considered to be zero until the age of puberty, the final diagnosis is a constitutional type of precocious puberty rather than some type of functioning ovarian tumor. We
wish
cooperation
to
acknowledge
of Dr. Walter
rector, Piedmont Preedy, Assistant University School
the
assistance
and
Bloom, Research Di-
Hospital, and Dr. John Professor of Medicine, of Medicine.
R. K. Emory
640
Coppedge
and
Hasty
REFERENCES
Morris, John McLean, and Scully, Robert E.: Endocrine Pathology of the Ovary, St. Louis, 1958, The C. V. Mosby Company. Novak, Emil, and Novak, Edmund: Gynecologi< and Obstetric Pathology With Clinical and
‘Endocrine Relations, Philadelphia, 1958. \+‘. B. Saunders Company. Wilkins, L.: The Diagnosis and Treatment of Endocrine Disorders in Childhood and Adolescence, Springfield. Ill.. 1950. Charles C: Thomas, Publisher, pp. 156 I 5 T. Woodruff, Donald : Personal rorrlrnurlicatioI1.