Keywords

Luteoma of pregnancy, benign tumors, ovary, hyperandrogenism

Pregnancy luteoma was first described by Sternberg and Barclay in 1966. Until now, fewer than 200 cases of pregnancy luteoma have been reported. In general, luteomas are asymptomatic and 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 stromal cells, which respond in an exaggerated manner to the elevated levels of gonadotropin during pregnancy.

Hypersecretion 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 and 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-year-old primigravida 34 weeks gestation case of polycystic ovary syndrome (PCOS), came for regular antenatal care (ANC) to our hospital. She had spontaneous conception. First and second trimesters were uneventful. One week prior to our hospital ANC, she was admitted with history of fever, vomiting and pain abdomen at a local hospital and treated symptomatically. Medical history - hypothyroid on eltroxin 50 µg o.d. - 3 years. GPE - patient was found to be hirsute. Body mass index (BMI) - 23.66 m2.

Per abdomen - uterus was 34 weeks size relaxed head lower pole. Fetal heart rate (FHR) good. Blood investigation - normal, obstetric scan - single live intrauterine gestation corresponding to gestational age of 34 weeks. EFW 2 kg. Amniotic fluid index (AFI) 11.8, two large heterogeneous predominantly hyperechoic bilateral lobulated adnexal masses in maternal abdomen showing mild internal vascularity, likely of ovarian origin. Possibilities included ovarian hyperstimulation with hemorrhagic change/Krukenberg’s tumors. Cancer antigen (CA) 125 - 27.4 U/mL.

Patient was counseled and we proceeded with magnetic resonance imaging (MRI) for further evaluation of bilateral ovarian masses. MRI reported as bilateral large lobulated soft tissue signal intensity mass lesions in both adnexae, right measuring 11.9 × 8.6 cm and left measuring 9 × 5.8 cm. Areas of hemorrhages were seen inside the masses. Ovaries were not seen separately from the lesions. There was no calcification/necrosis within and there was no ascites. We discussed the case with the patient and attendants and they were counseled regarding ovarian masses. It was planned to continue pregnancy up to term and terminate by elective lower segment cesarean section (LSCS). At term, patient underwent elective LSCS with bilateral partial oophorectomy.

Findings: Straw-colored minimal peritoneal fluid, which was sent for cytology. A live active baby was delivered with good Apgar score and a birth weight of 2.175 kg. Ambiguous genitalia were noted. No uterine anomaly. Right and left ovaries were enlarged to a size of 12 × 10 cm (Fig. 1 a and b). On vaginal toileting after cesarean section, patient was found to have clitoromegaly. Intraoperative and postoperative period was uneventful. Sutures were removed on Day 6 and patient was discharged with HPR of right and left ovariectomy specimens suggestive of pregnancy luteoma. Peritoneal fluid was negative for malignant cells. Hormonal profile with dehydroepiandrosterone (DHEA) sulfate, 17A-hydroxyprogesterone, total testosterone was found to be normal.

DISCUSSION

Luteoma of pregnancy is a rare condition. It most often occurs in the 3rd and 4th decades and is associated with increased prevalence in African American population and in the multiparous state. Pregnancy luteoma is a non-neoplastic lesion of ovary occurring during pregnancy and 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 postpartum tubal ligation. In 25% of cases, luteomas are hormonally active, leading to secretion of androgens causing masculinization in mothers and female infants (60-70% cases).

Pregnancy luteomas are variable in size ranging from microscopic to over 20 cm in diameter. In our case, bilateral ovaries measured 12 cm 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 and finally granular. The nuclei may be slightly pleomorphic and 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% to 8% with arrheno- blastomas, granulosa - theca tumor, Krukenberg tumor, papillary mucinous cystadenocarcinoma and mucinous cystadenoma 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 and hyperreactio luteinalis.

Young et al recommended an ultrasound if an enlarged ovary was palpable during an initial pelvic examination to identify the size and whether it was 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 and multinodularity are more common in luteomas that in other ovarian tumors.

The etiology of pregnancy luteoma remains unclear. It is hypothesized that they arise from stromal cells, which were present before pregnancy and respond in an unusual manner to elevated levels of gonadotropins encountered during pregnancy. PCOS 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 and Afro-Caribbean ethnicity.

During a normal pregnancy, maternal circulating testosterone level can increase in third trimester. Serum levels of total testosterone may rise up to 7 times the nonpregnant levels and 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 and mimics either a solid or complex cystic ovarian neoplasm. It regresses in postpartum 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.

SUGGESTED READING

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