Introduction

Pregnancy luteoma was first described by Sternberg and Barclay in 1966. Until now fever than 200 cases of pregnancy luteoma have been reported. In general, luteomas are asymptomatic & found incidentally at the time of cesarean section or postpartum tubal ligation. Luteoma of pregnancy is a benign, hyperplastic tumor like lesion of the ovary.

The etiology is unclear and it has been postulated that the pregnancy luteoma arises from pre existing luteinized stomal cells, which respond in an exaggerated manner to the elevated levels of gonadotropin during pregnancy.

Hyper secretion of androgens occurs in approximately 25% of women with pregnancy luteoma; from 10% to 50% of these women will show clinical signs of hyperandrogenism and 60% to 70% of female infants born to masculinized mothers will themselves exhibit some degree of virilization.

Most cases resolve completely in about 3 months postpartum. It may be a diagnostic & management challenge as it can mimic the presentation of malignant ovarian tumors. An accurate diagnosis is important to avoid unnecessary surgery.

Case Report

A 29 years old primigravida 34weeks gestation case of PCO, came for regular ANC to our hospital. She had spontaneous conception. I & II trimesters were uneventful. One week prior to our hospital ANC, she was admitted with history of fever, vomiting & pain abdomen at a local hospital & treated symptomatically. Medical history – hypothyroid on eltroxin 50µgOD - 3yrs. GPE – patient was found to be hirsute. BMI – 23.66m2

Per abdomen – uterus was 34 weeks size relaxed head lower pole. FHR good. Blood investigation- Normal, obstetric scan - single live intrauterine gestation corresponding to gestational age of 34weeks. EFW 2kg. AFI 11.8cm. large heterogeneous predominantly hyperechoic bilateral lobulated adnexal masses in maternal abdomen showing mild internal vascularity likely ovarian origin. Possibilities include ovarian hyper stimulation with hemorrhagic change / kruckenberg’s tumors. CA 125 – 27.4 U/ml. Patient counseled & proceeded with MRI for further evaluation of bilateral ovarian mass. MRI reported as bilateral large lobulated soft tissue signal intensity mass lesions in both adnexae, right measuring 11.9 x 8.6 cms and left measuring 9 x 5.8 cms. Areas of haemorhages seen inside the masses. Ovaries not seen separately from the lesions. No calcifications / necrosis within. No ascites. Discussed & counseled regarding ovarian mass with patient & attendants. Planned to continue pregnancy upto term & terminate by elective LSCS. At term patient underwent elective LSCS with bilateral partial oophorectomy. Findings: straw coloured minimal peritoneal fluid for cytology sent. Live active baby delivered with good Apgar. Baby weight 2.175kg. Ambiguous genitalia noted. No uterine anomaly. Right & left ovary enlarged into 12 x 10cm size. On vaginal toileting after cesarean section, patient found to have clitoromegaly. Intraoperative & postoperative period was uneventful. Suture removal done on day 6 & patient discharged with HPR of right & left ovariectomy specimens suggestive of pregnancy luteoma. Peritoneal fluid negative for malignant cells. Hormonal profile with DHEA sulfate, 17A hydroxy progesterone, total testosterone – found to be normal.

Discussion

Luteoma of pregnancy is a rare condition. It most often occurs in the 3rd & 4th decades & is associated with increased prevalence in African American population & in the multiparous state. Pregnancy luteoma is a non-neoplastic lesion of ovary occurring during pregnancy & is known to spontaneously regress which begins within days after the delivery. It is multinodular in half the cases and bilateral in a third of cases. Serum androgen levels decrease rapidly after delivery usually reaching normal concentration within 2 weeks postpartum. To date fewer than 200 cases have been reported in literature. Most patients are asymptomatic with enlarged ovary discovered incidentally during cesarean section or at time of post partum tubal ligation. In 25% of cases, luteomas are hormonally active, leading to secretion of androgens causing masculinization in mothers & female infants (60-70% cases).

Pregnancy luteomas are variable in size ranging from microscopic to over 20cm in diameter. In our case bilateral ovaries measured 12cm in dimension. On gross examination, cut surfaces of luteomas are solid, soft, tan or flesh colored with hemorrhagic foci. Microscopically, luteomas are sharply circumscribed nodules composed of polygonal cells arranged in sheets, cords or small clusters or they surround follicle like spaces containing colloid like material. The cytoplasm is abundant eosinophilic & finaly granular. The nuclei may be slightly pleomorphic & hyperchromatic, Hence it was diagnosed as pregnancy luteoma.

The occurrence of an ovarian tumor presenting during pregnancy seems to be rare with the incidence ranging from 1:815 to 1:2200. Among these, the incidence of malignancy ranges from 2-8% with arrheno blastomas, granulosa - theca tumour, krukenberg tumor, papillary mucinous cystaadeno ca and mucinous cystadenoca. were commonly seen during pregnancy.

The differential diagnosis for pregnancy luteomas includes granulose cell tumors, thecomas, sertoli leydig cell tumors, pure leydig (hilar) cell tumors, unclassified sex cord – stromal tumors, stromal hypertherosis, stromal luteomas & hyperreactio luterinalis.

Young et al. recommended an ultra sound if an enlarged ovary palpable during an initial pelvic examination to identify the size & whether cystic or solid. The ultrasonographic features of luteoma of pregnancy have been described as that of a solid mass which can be unilateral or bilateral with either single or multiple nodules. Bilaterality & multinodularity are more common in luteomas that in other ovarian tumours.

The etiology of pregnancy luteoma remains unclear. It is hypothesized that they arise from stromal cells, which were present before pregnancy & respond in an unusual manner to elevated levels of gonadotropins encountered during pregnancy. Polycystic ovary syndrome is one condition predisposing a woman to form a luteoma during pregnancy. High levels of hormones in PCOS is responsible for this. Women who have already had a luteoma during a previous pregnancy have a high risk of having another luteoma. Other risk factors associated with luteomas are multiple pregnancies, advanced maternal age & Afro – Caribbean ethnicity.

During a normal pregnancy, maternal circulating testosterone level can increase in 3rd trimester. Serum levels of total testosterone may rise upto 7times the non-pregnant levels & this physiological condition does not cause virilization. Virilization during pregnancy is a rare clinical event. It is most commonly caused by pregnancy luteoma or hyperreactio luteinalis . Pregnancy luteomas typically undergo spontaneous postpartum regression usually within 3 months of delivery. Serum testosterone levels usually return to normal by 2 weeks postpartum.

Luteoma of pregnancy must be differentiated in pregnant females with ovarian masses as recognition of this entity will obviate unnecessary oophorectomy. It should be considered in the differential diagnosis of ovarian masses in females who are pregnant or have been recently pregnant.

Conclusion

Luteoma of pregnancy is a rare condition which represents an unusual response to the altered hormonal environment in pregnancy & mimics either a solid or complex cystic ovarian neoplasm. It regresses in post partum period. It should be considered in the D/D to avoid unnecessary radical surgery. In difficult clinical cases with atypical presentation biopsy of this lesion with intraoperative frozen section may allow preservation of the ovary.

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