Hyperreactio luteinalis in a spontaneously conceived singleton pregnancy

Abstract

Hyperreactio luteinalis is a rare condition in pregnancy that is caused by high β-human chorionic gonadotropin (β-hCG) levels or abnormal sensitivity of the β-hCG receptor. It is mostly seen in patients with trophoblastic disease, multiple pregnancy or after fertility treatment. We describe our imaging findings and management of a case of hyperreactio luteinalis diagnosed in a singleton spontaneously conceived pregnancy.A 28-year-old primigravida was referred to the gynecological ultrasound department of the University Hospitals, Leuven, for the evaluation of bilateral adnexal masses at 14 weeks' gestation. Her pregnancy had been spontaneously conceived and she had no personal or family history of ovarian, breast or colon cancer. There was no history of subfertility and the patient did not use any kind of medication. The adnexal masses were first visualized in a regional hospital when she presented to the emergency room with abdominal pain. Vaginal examination revealed large mobile bilateral masses that filled the pouch of Douglas.Transabdominal and transvaginal gray-scale and color Doppler ultrasound examination was performed using a GE Voluson E8 Expert scanner (4-8-MHz transabdominal transducer and 6-12-MHz transvaginal transducer (GE Healthcare Technologies, Milwaukee, WI, USA)). Large bilateral multilocular masses measuring 141 × 134 × 103 mm (volume, 1019 cm 3 ) in the left ovary and 127 × 120 × 87 mm (volume, 694 cm 3 ) in the right ovary were visualized. Both masses contained more than 10 locules with rather thick (>3 mm) septa and anechoic cyst fluid. The cyst wall was regular (Figure 1) and the color score was 3 (moderately vascularized) in both masses. The fetus was normal in size for gestational age and no structural abnormalities were visualized. There was a small quantity of free fluid in the pouch of Douglas (30 × 22 mm) but no ascites. Both ultrasound examination and an X-ray of the chest were negative for metastases and pleural effusion. Blood analysis showed no abnormalities but a raised level of serum CA-125 of 442 kU/L.Owing to the progressive growth and the size of the masses, reaching the level of the diaphragm, and the large number of locules, the masses were suspected to be mucinous borderline tumors of the intestinal type and the patient was referred for surgery. Surgery was performed at 18 weeks of gestation using a midline incision. The right mass reached the level of the liver and the left mass filled the entire paracolic area (Figure 2). There was no ascites and no macroscopic signs of metastases. A right adnexectomy was performed and, on frozen section, the intraoperative diagnosis was hyperreactio luteinalis (Figure 3). The left mass was punctured but left in situ. Postoperative recovery and the subsequent course of the pregnancy were uneventful. The patient had a spontaneous vaginal delivery of a 3800-g girl at 40 weeks. At 9 weeks' postpartum, the patient was asymptomatic and ultrasound examination showed that the left adnexa was normal and contained a hemorrhagic corpus lute...

Androgens in pregnancy: roles in parturition

“…The most common hyperandrogenic conditions are known as ‘non-tumour ovarian hyperandrogenism’ and include PCOS, hyperreactio luteinalis (HL) and pregnancy luteoma (Kanova and Bicikova, 2011). Numerous cases of HL, occurring predominantly during the second or third trimester and caused by high β-human chorionic gonadotrophin (β-hCG) levels, have been reported (Haimov-Kochman et al , 2004; Van Holsbeke et al , 2009; Amoah et al , 2011). These studies highlight that, in spite of the protective mechanisms, a small fraction of women with HL exhibit virilization and/or hirsutism, although their female fetuses are hardly ever virilized (Hensleigh et al , 1975; Foulk et al , 1997; Holt et al , 2005; Angioni et al , 2007; Van Holsbeke et al , 2009; Veleminsky, 2010; Abe et al , 2011; Amoah et al , 2011; Annamalai et al , 2011).…”

Hyperreactio luteinalis: An often mistaken diagnosis

“…HL can also mimic ovarian malignancies like mucinous borderline tumor of intestinal type;[5] however, they are differentiated by the fact that they have smaller thin-walled cysts and not as much solid component as seen in HL. [6] They can be differentiated from the same based on β hCG levels, ovarian tumor markers, and imaging modality correlation like USG and magnetic resonance imaging (MRI). [78]…”

“…[9] Based on the size of these multicystic ovaries, the patients may have pain or may undergo torsion, cyst rupture, or pelvic entrapment of enlarged ovary, which then becomes a surgical emergency. [10] These patients may develop hyperandrogenism due to elevated β hCG levels[6] and require appropriate management.…”

Hyperreactio luteinalis in pregnancy

“…The presence of many thin-walled luteal cysts with characteristic ''spoke wheel'' appearance, as well as subsequent regression and resolution, suggested HL (2).…”