Stein-Leventhal syndrome

In untreated women, the main risk factors for endometrial carcinoma are age, obesity, nulliparity, late menopause (and possibly early menarche), the Stein-Leventhal syndrome, exposure to exogenous estrogens, radiation, and certain systemic diseases, including diabetes mellitus, hypertension, hypothyroidism, and arthritis (SED-14, 1451) (88). Certain of these risk factors indicate that an altered endocrine state with increased estrogen stimulation is a predisposing cause, and one might thus in theory expect estrogen treatment (and notably hormonal replacement therapy) to increase the risk (SEDA-22, 466). 

Fig. 7.13 Stein-Leventhal syndrome white, thickened capsule of the right ovary. Chnical suspicion of pronounced hver damage due to hormone therapy...

Polycystic ovarian disease (Stein-Leventhal syndrome)... 

Steele-Richardson-Olszewski syndrome Stein-Leventhal syndrome Stevens-Johnson syndrome Still s disease Stokes-Adams syndrome Vaquez disease Vincent s disease... 

Although most notable in Stein-Leventhal syndrome, which comprises the classical findings of amenorrhea, hirsutism, obesity, and sclerotic ovaries, a wide range of clinical presentations exist. Only one-quarter to one-half of the patients present the classical signs. Usually, infertility is the leading clinical problem of patients with PCOS. Recently, ultrasonographic studies reported a prevalence of polycystic ovaries in young women of at least 20%. However, there seems to be an overlap of polycystic ovaries and normal ovaries [62]. 

Fig. 9.24a,b. Polycystic ovaries in MRl. Transaxial T2-weighted images (a) and parasagittal T2 W1 (b) in a patient with Stein-Leventhal syndrome. Bilateral spherical ovaries are demonstrated showing numerous small follicles of uniform size. The latter are located in the periphery of the ovary and surround the ovarian stroma (asterisk), which typically is of very low signal intensity on T2-weighted images in PCO... 

Pathogenesis of Diseases of the Sex Glands 488 Hereditary Diseases of the Sex Glands Inborn Errors of Corticosteroid Metabolism Adrenal Carcinoma Virilizing Adrenal Adenoma Stein-Leventhal Syndrome Steroid Metabolism in Liver Disease... 

Thus, in the Stein-Leventhal syndrome, the A pathway has preponderance over the A pathway, yielding precursors of testosterone. Without 11-hydroxytestosterone oxidation, testosterone (a precursor of estrogen) is not converted to estrogen. The enzyme defects just described are probably restricted to only part of the organ because, as already mentioned, normal levels of estrogens are found in patients with Stein-Leventhal syndrome, and wedge section cures the disease [91]. 

Whether this molecular interpretation of Stein-Leventhal syndrome will survive the tide of further publications remains to be seen. In any event, the exact pathogenesis of the disease is not clear, but it has been proposed that the disease results from an aberration of the hypothalamic control of gonadotropin release with excess and uncyclic secretion of luteinizing hormone. 

The ovaries synthesize androgens. The levels of 17-ketosteroids in the urine were found to be decreased in ovariectomized individuals. Dehydroxyandrosterone has been found in ovarian blood, and the plasma levels of testosterone are higher in the normal than in ovariectomized females. When ovarian tissue from patients with Stein-Leventhal syndrome is incubated in vitro, testosterone and other androgens are formed. The three pathways for androgen synthesis in the ovary are outlined in Fig. 8-14. 

In rats, progesterone at an optimal ratio to estrogen can facilitate the release of LH and Induce ovulation.A new retroprogesterone derivative, RO-4-834S, was also foUQd to trigger ovulation in several cases of Stein-Leventhal Syndrome. ... 

In our earlier study (C. R. Acad. Sc. Paris 260 6698 1965) we treated 3 patients suffering from Stein-Leventhal syndrome with partially purified FSH and LH fractions obtained from human postmenopausal urinary gonadotropin (HMG) in order to induce ovulation. In 2 cases ovulation was induced, as was shown by the presence of a recently formed corpus luteum in the ovary, and the urinary levels of 11-deoxy-17-ketosteroids decreased after the treatment. 

In this paper we study the behavior of urinary androgen metabolites of patients without ovulatory cycle after treatment with a combination of FSH and LH fractions prepared from urine of postmenopausal women (Jutisz M. et aL C. R. Acad. Sc. Paris 259 1195, 1964) following the scheme we used in patients with the Stein-Leventhal syndrome. 

Patients were treated with this combination (2 patients suffering from sterility, 2 with Stein-Leventhal syndrome and 3 patients with breast carcinoma). 

In polycystic ovarian disease, both of the generally enlarged ovaries contain many small follicular cysts (2-6 mm), but larger cysts may also be present. Polycystic ovarian syndrome (Stein-Leventhal syndrome) is characterized by the association of polycystic ovaries with irregular menses, prolonged uterine bleeding, amenorrhea, anovulation, and often hirsutism and obesity. The clinical manifestation of this syndrome begins at or shortly after puberty. 

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