Rapidly Progressive and Severe Hirsutism from Hyperreactio Luteinalis within a Background of Polycystic Ovary Syndrome

Abstract

Objective: Gestational trophoblastic disease and hyperreactio luteinalis (HL) are rare, but important, etiologies of hyperandrogenism that need to be further studied.Methods: We present a case of rapidly progressing hirsutism and marked biochemical androgen excess in the context of pregnancy.Results: A 26-year-old woman with a past medical history of obesity, prediabetes, and polycystic ovary syndrome presented with worsening hirsutism and markedly elevated testosterone levels. She was subsequently found to be pregnant, with extremely elevated levels of serum β-human chorionic gonadotropin. Subsequent workup led to the identification of molar pregnancy and bilaterally enlarged ovaries, suggestive of HL. Following surgical intervention and therapy with methotrexate for invasive mole, she experienced improvement in both biochemical and clinical androgen excess features.Conclusion: With the prevalence of polycystic ovary syndrome, many women present to medical providers with hirsutism or other findings of hyperandrogenism. However, rapid progression of existing hirsutism or severe hirsutism should prompt more extensive evaluations to rule out rare etiologies. One such etiology found in pregnancy is HL, in which high levels of β-human chorionic gonadotropin can stimulate production of benign theca lutein cysts, leading to marked hyperandrogenism and virilizing symptoms. (AACE Clinical Case Rep. 2019;5:e86-e90) Abbreviations: 17-OHP = 17-hydroxyprogesterone; β-hCG = β-human chorionic gonadotropin; DHEA-S = dehydroepiandrosterone-sulfate; GTD = gestational trophoblastic disease; HL = hyperreactio luteinalis; MRI = magnetic resonance imaging; PCOS = polycystic ovary syndrome; SHBG = sex hormone-binding globulin; TSH = thyroid-stimulating hormone

Touch-me-not: Hyperreactio luteinalis during caesarean delivery of a spontaneously conceived singleton term pregnancy with chronic hypertension: A case report

“…6,8 It can also be associated with symptoms of maternal hirsutism or virilization, especially in females with PCOS. 9 However, virilization has been reported in only 25–30% pregnant women, 5,6 usually due to the increased levels of sex hormone binding globulin in pregnancy, which result in lower amounts of circulating free testosterone. 5 Foetal virilization has also been reported, albeit extremely rare 4 as foetal labioscrotal fusion occurs in the first trimester and HL is seen later in gestation.…”

Hyperreactio luteinalis associated with fetal hyperandrogenism and cystic hygroma

“… 15 Inpatient management, including analgesic therapy and fluid substitution, may be required. 16 Ovarian suppression with oral contraceptives can be used to accelerate resolution, 1 with surgical therapy being reserved for complicated cases with bleeding, torsion, or rupture. 17 In our case, termination of pregnancy was requested by the parents due to the presence of cystic hygroma.…”

“…10 A prior polycystic ovarian syndrome may predispose for HL during subsequent pregnancies. 1 Hyperreactio luteinalis presents most commonly in the second or third trimester. 3 Many cases are discovered incidentally, either with ultrasound or during cesarean section, whereas symptomatic cases usually present with abdominal discomfort or pain.…”

“…Under excessive stimulation, the ovarian granulosa and theca interna cells become hypertrophied and luteinized, which leads to the development of theca lutein cysts 10 . A prior polycystic ovarian syndrome may predispose for HL during subsequent pregnancies 1 …”

“…Hyperreactio luteinalis is a rare benign condition of pregnancy that is characterized by the formation of multiple large ovarian cysts due to hyperstimulation of the ovarian tissue. 1 Although the exact causes are unknown, this entity is attributed to elevated levels of human chorionic gonadotropin (hCG) or ovarian hypersensitivity to this hormone. 2 , 3 This disease can be associated with other hormonal derangements such as thyroid hormone abnormalities or hyperandrogenism.…”