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SA MEDIESE TYDSKRIF DEEL 66 14 JUUE 1984

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Luteoma of pregnancy
H. S. CRONJE
Maternal virilism occurred in 2 patients, both with bilateral tumours, but the fetal outcome is unknown. In 4 patients the tumour was found accidentally, in 3 at caesarean section and in I at postpartum tuballigation. Bilateral tumours were present in 4 patients, one ofthem being the patient with hyperreactio luteinalis. A unilateral salpingo-oophorectomy was done in 5 patients, including 3 of those with bilateral tumours. In one of them, a specimen was taken from the other ovary. The 4th patient with bilateral tumours was treated by total abdominal hysterectomy and bilateral salpingo-oophorectomy. Finally, in I patient only a wedge biopsy was done. There were no recurrences or deaths after 2,3 and 13 years respectively in the 3 patients who were followed up. The previous diagnoses in these patients were luteinized granulosa cell tumour (cases 2, 3, 5 and 6; see Table I), luteoma of pregnancy (cases 1 and 7) and luteinized Stein-Leventhal syndrome (case 4).

Summary
Seven patients are presented. 6 with luteomas of pregnancy and 1 with hyperreactio luteinalis. The mean age was 31,7 years and 4 patients had bilateral tumours. Only 1 of these patients was treated with bilateral salpingo-oophorectomy, while 1 patient with a unilateral tumour had only a wedge biopsy. In all instances the remaining 'tumour' regressed, supporting the thesis that luteomas represent an unusual response of ovarian stromal cells to the altered hormonallevels of pregnancy. About 30% of patients with luteomas of pregnancy will be virilized. and about 50% of.female infants from these patients will be masculinized. About 112 patients with luteomas of pregnancy have been reported in the literature. The differential diagnosis includes theca lutein cysts, the oedematous ovary syndrome and luteinized forms of ovarian stromal tumours.
S Atr Med J 1984; 66: 59-60.

Discussion
In 1975 Garcia-Bunuel et aP reported the iargest published series ofluteoma of.pregnancy (20 patients). They reviewed the literature and found 54 previously recorded patients, 14 of whom were masculinized. Jenkins et al. 4 had reported 30 cases in 1968, but 29 were from the literature and only I their own. GarciaBunuel et aP included 22 of these 29 reported cases in their series. Since the latter report in 1975, 8 cases of luteoma of pregnancy have been reported in the literature, 5 of which were associated with maternal virilization. S- 12 Two of the latter, however, were probably hyperreactio luteinalis since the tumours were multicystic. s,7 These masculinized patients had male infants (sex unknown in I case). Clinical aspects of interest in patients with luteomas of pregnancy include a predominance of Black. patients,3,4 an association w.ith pre-eclampsia and obesity, and a 30% prevalence . of maternal masculinization during pregnancy.3 About 50% of female infants born to these masculinized mothers will be virilized. 13 Conservative surgery such as amputation of an enlarged clitoris may be necessary in a female infant} Male infants suffer no untoward effects and all mothers with masculinization return to normal after delivery. Pre-operative hormonal studies in masculinized patients revealed high levels of 17-ketosteroids, plasma testosterone, dihydrotestosterone, androstenedione and dehydro-epiandrosterone. 6 - 9 These elevated levels returned to normal within 3 days of removal of the tumour. Both ~-4 and ~-5 pathways were utilized in the synthesis of androgens. 8,9 Luteomas of pregnancy are bilateral in 45% of cases. 3 The tumours are usually smooth and solid, yellow to yellow-orange and about 6 - 10 cm in diameter. 14 Microscopically, a markedly uniform sheet of cells is seen, often composed ofill-defmed cords and nests. Classically, a rwo-eell population is present (Fig. I). The first type is a cell with deeply staining acidophilic cytoplasm, presumably of theca origin, which has invaded the granulosa layer during its luteinization. The second cell type is larger with less intense cytoplasmic eosinophilia, resembling luteinized granulosa cells. Reinke crystalloids have not been demonstrated in either cell. 3 Most tumours are not palpable during pregnancy and surgery is generally performed for other reasons. In about 60% of all

Luteoma of pregnancy was described by Sternberg I in 1963, and since then a further 112 cases have been reported. The question surrounding this entity is whether it represents a true neoplasm or only an abnormal response to the hormones of pregnancy. These 'tumours' may be endocrinologically active, as initially suggested by Malinak and Miller. 2 In this article 7 patients are described, 6 with luteoma of pregnancy and 1 with hyperreactio luteinalis. A review of the literature on luteoma of pregnancy is presented and the aetiology and differential diagnosis discussed.

Patients and methods


In reviewing 477 patients classified under granulosa and theca cell tumours of the ovary in the Emil Novak Ovarian Tumor Registry and in the ftles of the Surgical Pathological Laboratory of the Johns Hopkins Hospital, 6 patients were found with luteoma of pregnancy and I with hyperreactio luteinalis. This group of7 patients is the subject of analysis in this article. In each case clinical information and microscopic slides were available.

Results
The clinical data are summarized in Table 1. Five patients were Black, 2 White; the mean age was 31,7 years. They varied from para 1 to para 11 (parity unknown in 2) at the time of pregnancy.

Department of Obstetrics and Gynaecology, Johns Hopkins Hospital, Baltimore, Md, USA

H. S. CRONJE, F.CO.G. (SA), M.MED., (0 ET G), M.D. (Present address: Department of Obstetrics and Gynaecology, University of Stellenbosch and Tygerberg Hospital, Parowvallei, CP)

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SA MEDICAL JOURNAL VOLUII.1E 66 14JULY 1984

TABLE I. LUTEOMA OF PREGNANCY Case 1 2 3 4 5 6 7* Race Black Black Black White Black White Black Age (yrs) 42 20 22 38 27 31 41 Grav. Para Location, size Bilateral, 6 cm Unilateral Right, 13 cm Bilat., 6 and 10 cm Bilat., 5 and 6 cm Unilat., 7 cm Bilat., bluish, multicystic Treatment Follow-up

? ?
4 2 6 2 12

? ?
2 1 1 1 11

usa at C/S
Wedge

usa at C/S usa


TAH,BSO usa at C/S

3 yrs

13 yrs 2 yrs

usa

usa = unilateral salpingo-oophorectomy: 850 = bilateral salpingo-oophorectomy; TAH = total abdominal hysterectomy; C/S = caesarean section. ""Hyperreactio luteinalis (see text).

The differential diagnoses include the stromal luteoma,l> luteinized forms of thecoma, granulosa cell tumour and gonadal stromal tumour, and the large oedematous ovary syndrome. The latter, as well as the stromal luteoma, may consist of a two-cell population as seen in luteomas of pregnancy. These patients are not pregnant and the oedematous ovary syndrome has a distinct appearance with its oedematous stroma in contrast to the solid sheet of cells of the luteoma of pregnancy. Luteinized granulosa and theca cell tumours lack the two-cell population of the luteoma of pregnancy. The stromal luteoma and luteinized gonadal stromal tumour are probably variants ofthe same entity. In conclusion, the luteoma of pregnancy is a rare condition which probably represents an unusual response to the altered hormonal environment in pregnancy. It regresses in the postpartum period. About one-third of patients will become masculinized during pregnancy and half the female infants born to these mothers will also be masculinized. Luteomas should be differentiated from the theca lutein cysts, the oedematous ovary syndrome, and luteinized forms of ovarian stromal tumours.
Fig. 1. Luteoma of pregnancy. Note solid sheet of cells with dark (pink) and pale cells (X 400).

I wish to thank Professor J. D. Woodrufffor his assistance in the microscopic evaluation of these tumours.
REFERENCES
1. Sternberg WHo Nonfunctioning ovarian neoplasms. In: Grady HG, Smith DE, eds. The Ovary. (International Academy ofParhologisrs, Monograph No. 3). Baltimore: Williams & Wilkins, 1963: 209. 2. MaIinak LR, Miller GV. Bilateral multicentric ovarian luteomas of pregnancy associated with masculinization of a female infant. Am] Obs!er Gynecol 1965; 91: 251-259. 3. Garcia-Bunuel R, Berek JS, Woodruff JD. Luteomas of pregnancy. Obs!er Gyneco/1975; 45: 407-414. 4. Jenkins ME, Surana RB, Russell-Cutts CM. Ambiguous genitals in a female infant associated with luteoma of pregnancy: report of a case. Am] Obslel Gynecol 1968; 101: 923-928. 5. Tucker S, Buell J, Fisher HR. Luteoma of pregnancy: case report Am] Obsrer Gyneco11975; 121: 282. 6. Forti G, Burarti R, Colafranceschi M er al. Hormonal studies in a virilizing 'luteoma' of pregnancy ('nodular theca-Iuteinic hyperplasia ofpregnancy'). In: James VH er al., eds. The Endocrine Function of rhe Human Ovary. London: Academic Press, 1976: 417. 7. Polansky S, de Papp EW, Ogden EB. Virilization associated with bilateral luteomas of pregnancy. Obmr Gyneco11975; 45: 516-522. 8. Zander J, Mickan H. Luteoma of pregnancy with androgenic activity. In: de Watteville H er al., eds. Diagnosis and TTeatmenr of Ovarian Neoplasric Alrerations. Amsterdam: Excerpta Medica, 1975: 249-251. 9. O'MalJey BW, Lipsett MB, Jackson MA. Steroid content and synthesis in a virilizing luteoma.] Clin Endocrinoll967; 27: 311-319. 10. Rachman R, Tellem M. Bilateral ovarian luteomas with tubal pregnancy. Am] Obmr GynecoI1964; 88: 132-134. 11. B6hm W. Der Luteinzellrumor des Ovars bei Graviditiit. Zenrra/bl AlIg Parhol 1977; 121: 404-408. 12. Fonseca JJS, Quesada GZ. Luteoma of pregnancy: report of a case and review of literaruse.] RepTod Med 1975; 14: 76-79. 13. Hensleigh PA, Woodruff JD. Differential maternal-fetal response to androgenizing luteoma or hyperreactio luteinalis. Obscer Gynecol Surv 1978; 33: 262-271. 14. Tulzer H, Vim R. A very large luteoma gravidarum. ZentTalbl Gynako/1976; 98: 1659-1660. 15. Scully RE. Stromal luteoma of the ovary: a distinctive type oflipoid-eell tumor. Cancer 1964; 17: 769-778.

patients the tumour was found at caesarean section, with cephalopelvic disproportion as the indication in half of them. Unilateral salpingo-oophorectomy is the treatment most often applied. Other forms of treatment vary from wedge biopsies> to total abdominal hysterectomy and bilateral salpingo-oophorectomy. Tumours left behind regressed almost routinely. These patients have no difficulty in becoming pregnant again and the 'tumour' rarely recurs. 3 The correct treatment, therefore, is biopsy and conservation of the enlarged ovary. The origin of luteomas of pregnancy is presumably the theca and granulosa cells responding to the elevated hormonal levels of pregnancy.3 Chorionic gonadotrophin was suspected as the promoter, but administration of this hormone in late pregnancy has resulted only in a dramatic hyperreactio luteinalis or theca lutein cyst. Luteomas of pregnancy have not been produced experimentally. Theca lutein cysts, often seen in association with molar pregnancies, may represent a cystic form ofluteoma. They are characterized by similar clinical phenomena, although it has been stated that the cystic form 'protects' a female infant against masculinization if the mother is masculinized. l3 Similar cell types can be seen in both theca lutein cysts and in luteomas, and both regress postpartum. These tumours, therefore, represent a non-neoplastic hyperplasia of certain stromal cells, although uncertainty exists as to whether some are preceded by minute lesions like thecomas. One of the Emil Novak Ovarian Tumor Registry patients (case 4) with bilateral luteomas had a history of possible Stein-Leventhal syndrome.

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