Virilization secondary to massive ovarian edema is rare, and its etiology is still under investigation. It is becoming apparent that an impairment in the perfusion of the ovary and/or stromal edema may cause luteinization of stromal cells, and that the abnormal secretion of steroids by such cells could be directly responsible for the endocrinological changes observed in some women. Laboratory studies to substantiate this hypothesis, however, are not yet available. The present report describes a case of virilization secondary to massive ovarian stromal edema and the associated endocrinological changes that could explain some of the clinical characteristics of the disease. A 15-year-old patient was first seen for evaluation of deepening of the voice of 2 years' duration. Review of systems was otherwise normal. Gynecological history was normal, with regular periods after menarche at age 13. The patient had developed oligomenorrhea about 6 months previously. Physical examination revealed a normally developed 15-year-old girl with a deep voice and mild hirsutism over the face. Pelvic examination disclosed an enlarged clitoris, 3.2 cm in length and 1.2 cm in width, and a non-tender pelvic mass, 10.0 cm in length and 7.0 cm in width, occupying the cul-de-sac. No other abnormalities were noted. Laboratory studies were all within the normal range. Plasma testosterone was markedly elevated (2.1 ng/ml) and remain unchanged. At laparotomy, the distal portion of the left fallopian tube was edematous and lay on top of an enlarged ovary. There was evidence of partial torsion of the left adnexum (Table 1). The left ovary was enlarged and edematous, with multiple cystic structures over its surface. The cut section showed homogeneous, whitish-pink tissue with multiple follicular cysts. Microscopic examination of the left ovarian mass showed diffusely edematous stroma with distended blood vessels and scattered islands of lutein-like cells. The stromal cells were widely separated by edematous matrix. Primordial follicles and occasionally maturing follicles were seen in the narrow layer of the cortex. The ovarian capsule appeared normal. There was no evidence of neoplasia or necrosis. The cut surface of the right ovary showed multiple subcapsular cysts ranging from 0.3 to 1.0 cm in diameter. Microscopic examination revealed essentially normal histological features, but a mild degree of edematous stroma was noted in scattered areas of the inner cortex. A few corpora albicantia were seen only in the right ovary. Intraoperatively, simultaneous peripheral and left ovarian venous blood samples were obtained before 15 minutes after 10 units of synthetic adrenocorticotrophic hormone (ACTH) were injected into the left ovarian artery. Blood for steroid determinations was also drawn on postoperative days 1, 5, 6, and 15. Plasma was separated and kept frozen at —- 18°C until the time of analysis. All samples obtained were run over simultaneously for the determination of progesterone, 17-hydroxyprogesterone (17-OHP), androstenedione, testosterone, and cortisol. Measurements of plasma testosterone, 17-OHP, progesterone, androstenedione, and cortisol were performed by radioimmunoassay. The plasma concentrations of progesterone and 17-OHP in the peripheral and ovarian veins and their ovarianperipheral vein gradients were above the range previously reported for normal women during the follicular phase. The ovarian vein concentration and the ovarianperipheral vein gradient of both progesterone and 17-OHP increased considerably after the administration of ACTH into the left ovarian artery. Of the androgens measured, only testosterone was abnormally elevated in both the peripheral and the ovarian veins. The ovarian-peripheral vein gradient was increased also, which indicated the predominantly ovarian source of this androgen. Conversely, androstenedione, a precursor of testosterone in normal ovarian steroidogenesis, was not increased in either the peripheral or the ovarian vein. Plasma levels of cortisol were in accordance with the stress of surgery, and its secretion by the ovary was not demonstrated. The increase in the ovarian-peripheral vein gradient of cortisol observed before ACTH administration (26.0 ng/ml) was within the interassay variability. The A per cent increase of plasma cortisol observed in the ovarian vein after ACTH administration (35.0 ng/ml) was the reflection of its concomitant increase in the peripheral circulation. After surgery, the peripheral vein levels of 17-OHP and testosterone decreased to normal.
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Dec 1, 1986
P Serafini + 2
Open Access