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Benign causes of maternal virilization in pregnancy, such as luteoma of pregnancy and hyperreactio luteinalis, are generally believed to resolve completely post partum and not to recur. We present the fifth case in the literature of recurrent maternal virilization in pregnancy. These lesions should be viewed as potentially recurrent and cases should be managed accordingly.

INCONGRUITIES IN sexual characteristics have tong stimulated interest. Hermaphroditism was a common theme in Graeco-Roman culture,’ and de Ribiera painted the bearded Magdalena Ventura nursing her infant in 163 1.’ Virilization in pregnancy is rare and is usually of ovarian origin.3 Of 44 ovarian lesions causing virilization during gestation, Verhoev,en and associates” found eight luteomas of pregnancy. The following case illustrates the unusual occurrence of maternal and fetal virilization caused by a luteoma of pregnancy and extends the number and breadth of endocrinologic studies in patients with this uncommon entity.

The clinical course of a 26-year-old primigravid woman with a virilizing luteoma of pregnancy is described. It was demonstrated by endocrine studies that the tumor secreted an excessive amount of testosterone. After removal of the luteoma in week 32 of pregnancy, the virilizing symptoms of the mother completely regressed. A normal male infant was delivered in week 39. From 100 luteomas of pregnancy described in the literature, 26 cases have been cited to elicit endocrine virilizing activity. An evaluative synopsis indicates that including the case described in this paper only 13 can be regarded as a clinical, morphologic, and functional entity. This entity should be differentiated from the inhomogeneous group of luteomas as the “androluteoma syndrome of pregnancy.” It is characterized by the following major criteria: virilization, beginning with the second trimester of pregnancy; histologic criteria of a luteoma of pregnancy; production of testosterone, leading to excessive plasma levels (exceeding the concentration of androstenedione), and masculinization of female fetuses.

Like arrhenoblastomas, the adrenal-like ovarian tumors tend to masculinize their hosts but the tendency toward the production of a bizarre type of fat distribution, the occurrence of purplish striae, arterial hypertension, polycythemia and so forth, relate them more closely to adrenal cortical carcinomas from a functioning standpoint. Histologically, the tumors do not appear to arise through luteinization of pre-existing granulosa-cell neoplasms and an origin from adrenal cortical rests seems more logical than does a derivation from corpora lutea. An origin by way of teratomatous development likewise remains unproved. Some of the tumors appear to be cytologically malignant and in one instance death resulted from metastasis.

A female newborn infant with ambiguous external genitals associated with a pregnancy luteoma is described. This association appears to be extremely rare. The mother of the patient demonstrated progressive virilization during her last trimester. Pregnancy luteoma has been observed to occur more frequently in Negroes. The cause of this apparent ethnic predilection is obscure.

A case of a patient with bilateral, multicentric “pregnancy luteomas” of the ovary has been presented. These rare tumors were associated with androgenic hormone secretion as evidenced by masculinization of her female infant and a very high urinary 17-ketosteroid level on the day of her delivery. This hormone level declined after unilateral oophorectomy 4 days post partum. In addition, the tumors were found to have regressed in the postpartum period as demonstrated when her second ovary was removed.

Three cases of a benign ovarian tumor, designated as “luteoma of pregnancy” are reported. All three tumors were discovered at the time of cesarean section and clinically were nonfunctioning. This tumor appears to be part of a distinct clinical-pathological entity that has only recently been defined.

1. Nine cases of luteoma of pregnancy are reported; the clinical characteristics and pathological findings, including histochemical and electron microscopic studies, are discussed. 2. Luteoma of pregnancy is a distinct entity which can and should be differentiated clinically and pathologically from other “luteomas.” 3. This entity is directly related and limited to pregnancy, and seemingly causes no ill effect upon mother or child. 4. This study suggests that luteoma of pregnancy may represent an exaggeration of the luteinization reaction of normal pregnancy. More specifically, it is thought to represent a hyperplastic luteinization of developing and atretic follicles similar to, but more marked than, that occurring in “hyperreactio luteinalis.” 5. The endocrinologic aspects of pregnancy luteoma are unclear, and evidence at hand does not warrant the implication of a single hormone. Hormone studies in patients with pregnancy luteoma are needed for further evaluation of the pathogenesis. 6. This lesion is benign and regularly regresses following pregnancy. If encountered at operation, no extensive therapy is required, but it is necessary to obtain a biopsy to confirm the diagnosis.

Steroid hormone content and steroid synthesis in vitro were studied in a clinically and morphologically typical luteoma of pregnancy. The only Δ 4 -3-ketosteroid present was tentatively identified by paper and thin-layer chromatography as Δ 4 -androstene-3,17-dione. Net synthesis (μg) of this androgen was augmented in vitro by HCG. No radioactive steroid formation from acetate-1- 14 C could be established after analysis by reverse isotope dilution. The biochemical and histologic data suggest that the luteoma of pregnancy originates from the ovarian stroma. The augmentation of steroidogenesis by HCG in vitro in this study and the clinical observation of postpartum regression in other studies are compatible with the concept that the placental gonadotropin plays some role in development and maintenance of this specific ovarian lesion.

1. The luteoma of pregnancy is a benign tumor of lutein-like cells occurring most often in patients of high parity and usually discovered at laparotomy at or near term. 2. Twelve examples of this lesion are presented of which 6 were multinodular and 4 of these were bilateral. 3. The pregnancy luteoma has characteristic microscopic features including acidophilic granular cytoplasm with sparse lipid, and a distinctive pattern of reticulum fibers. 4. The tumors most likely are derived from theca-lutein cells of atretic follicles stimulated by chorionic gonadotropin, although some may be derived from stroma-lutein cells. In one instance, the pregnancy luteoma originated in the theca interna of a corpus luteum of pregnancy. 5. There is evidence that the lesion spontaneously regresses following the drop in chorionic gonadotropin after delivery.