AMH producing purely cystic virilizing adult granulosa cell tumor in 17 years old girl: a case report and review of literatures

Abstract

Background Androgen-producing granulosa cell tumor in adolescent girl is rare condition and clinical characteristics are not fully elucidated. Case presentation Seventeen years old girl complained of secondary amenorrhea was referred to our out-patient consultation. Markedly elevated serum testosterone, LH, and AMH levels were noted. Mild hirsutism and clitoromegaly were presented. Transabdominal ultrasonography and MRI revealed cystic mass occupied pelvic cavity probably originated from left ovary. Right ovary showed polycystic appearance. Laparoscopic left ovarian cystectomy was performed. After the surgery, her menstruation resumed along with normalized hormonal parameters, and clinical hyperandrogenism were improved. Since the scarcity of cellular lining of inner cyst wall, definitive pathological diagnosis was difficult. After the consultation with gynecological pathologist, the tumor was diagnosed as sex cord stromal tumor, highly suspicious for adult granulosa cell tumor. Residual left salpingo-oophorectomy was performed by additional laparoscopic surgery. Her serum testosterone and AMH levels were remained low with regular menstrual cycles and no evidence of recurrence. Conclusions Androgen-producing cystic granulosa cell tumor is rare gynecological disorders, which need both gynecologic oncological and endocrinological approach. Its clinical manifestations may bring some clues to the pathogenesis of ovulatory dysfunctions, such as polycystic ovary syndrome.

Adult-type granulosa cell tumor associated with elevated luteinizing hormone: Two rare case reports

“…Immunohistochemistry has confirmed that the tumor cells are negative for LH. [ 10 , 11 ] Some researchers have hypothesized that tumor cells produce factors that stimulate tumor is high. [ 6 ] In women with markedly elevated levels of T or its precursor androstenedione and normal levels of dehydroepiandrosterone, an androgen-secreting ovarian tumor is likely.…”

“…Immunohistochemistry has confirmed that the tumor cells are negative for LH. [ 10 , 11 ] Some researchers have hypothesized that tumor cells produce factors that stimulate the adenohypophysis to secrete a large amount of LH. [ 11 ] LH secretion is regulated by both the hypothalamus and pituitary gland.…”

“…[ 3 ] Many studies have confirmed the utility of inhibin B and AMH as biochemical markers for the diagnosis, treatment effect evaluation, and monitoring of AGCTs recurrence. [ 12 – 14 ] Increased serum AMH [ 7 , 10 ] and inhibin B [ 9 ] levels have been reported in AGCTs with elevated LH levels. The levels of AMH and inhibin B were both high in Case A and decreased to normal levels after surgery.…”

“…A left ovarian cystectomy was performed. A rapid frozen pathology examination of the resected tumor Nasu et al [7] 2007 28 Solid High Normal Normal Normal Ran et al [8] 2017 33 Solid High Normal Normal Low Gică et al [9] 2021 26 Cystic High Normal Normal Normal Gu et al [7] 2022 31 Cystic High Normal High Normal Kitajima et al [10] 2023 17 Cystic High Low High Normal E2 = estradiol, FSH = follicle-stimulating hormone, LH = luteinizing hormone, T = testosterone indicated a GCT and the possibility of juvenile type. Fertilitysparing surgery with complete staging was performed with the consent of the patient's family member.…”