scholarly journals Diagnostic Dilemma in Two Cases of Hyperandrogenism

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Ibrahim Alali ◽  
Lilianne Haj Hassan ◽  
Ghadeer Mardini ◽  
Nermeen Hijazi ◽  
Lama Hadid ◽  
...  

Hirsutism is a common endocrine complaint affecting about 10 percent of women. It may be caused by multiple etiologies including adrenal and ovarian disorders. Usually, it is a result of a benign entity such as PCOs and idiopathic hirsutism. However, sometimes especially when it is severe and rapid in progression an androgen-secreting tumor should be excluded. Sertoli-Leydig cell tumors constitute fewer than 0.5 percent of ovarian tumors and it may be benign or malignant. In this article, we present two cases of hyperandrogenism caused by occult ovarian Leydig cell tumors. one of them was confounded by the presence of coincidental bilateral adrenal nodules that complicated the diagnostic process. Tumor dissection was curative in both cases and the diagnosis was confirmed by pathological and hormonal testing after surgery.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Mohamed K M Shakir ◽  
Rinsha P V Sherin ◽  
Travis Weaver ◽  
David LaChance ◽  
Thanh Duc Hoang

Abstract Severe hyperandrogenism in post-menopausal women is rare. It may be caused by either benign or malignant neoplasms of the adrenal or ovary. We report a rare case of a post-menopausal woman with hirsutism associated with virilization due to Leydig cell tumors (LCT) of both ovaries. Case report 61 yo female presented for evaluation of hirsutism. She had also been experiencing increased facial hair growth, deepening of voice, clitoromegaly, alopecia, and acne. Physical examination: normal vital signs. Patient had signs of virilization, including coarse hair along her upper lip, chin, lower abdomen and inner thigh with Ferriman-Gallwey score of 8, acne, and clitoromegaly. She had no signs of acanthosis nigricans or Cushing syndrome. Base line labs: Hemoglobin 16.2 gm/dL (ref 12.0 to 15.5), total testosterone 803 ng/dL (ref 3–41), free testosterone 20.2 pg/mL (ref 0.0–4.2), estradiol 77 pg/mL (<6.0−54.7), estrone 148 pg/mL (ref 7–40), FSH 11.5 mIU/mL (ref 25.8 - 134.8), LH 6.90 mIU/mL (ref 7.7 - 58.5), androstenedione 28 ng/dL (ref 17–99), DHEAS 99.9 mcg/dL (ref 19–205), dehydroepiandrosterone 512 ng/dL (ref 31–701), inhibin A 2.3 pg/mL (ref <5), inhibin B <7.0 pg/mL (ref 00-16.9), 17-alpha hydroxyprogesterone 187 ng/dl (ref <51). Her other serum markers such as anti -Mullerian hormone, alpha-fetoprotein, and hCG were normal. A CT scan of adrenals: normal. Similarly a transvaginal US did not show any ovarian pathology, however MRI of the pelvis showed homogeneous ovarian enhancement bilaterally and based on this information a diagnosis of ovarian hyperthecosis was considered and patient underwent laparoscopic bilateral oophorectomy. Pathology confirmed: LCT in both ovaries. Laboratory values performed 3 months later showed the following values: hemoglobin 14.2 gm/dL, total testosterone 13 ng/dL, free testosterone 1.4 pg/mL, LH 124 mIU/mL, FSH 99mIU/mL, estradiol <5.0 pg/mL. In 6 months she had significant improvement in hirsutism and virilization. Discussion Hyperandrogenism, especially serum testosterone in the male range with rapidly progressive hirsutism or virilization signs in a female indicates tumor etiology. Androgen-secreting ovarian tumors are usually small and often embedded in the ovary. Transvaginal US is useful in the diagnosis of ovarian tumors. However, in our case, transvaginal US failed while MRI scan showed bilaterally enlarged ovaries and with this information patient underwent bilateral oophorectomy. Although 5 cases of bilateral LCT are reported in the literature, LCT is unilateral 95% of the time, the pathogenesis of Leydig cell proliferation and LCT is unclear. In conclusion, androgen secreting tumors should be considered in women (especially in post-menopausal state) with hyperandrogenism and hirsutism. In fact, diffuse stromal Leydig cell hyperplasia and small Leydig cell tumors may be missed on imaging and in some cases only pathology can confirm the result.


Author(s):  
Bozo Kruslin ◽  
Zoran Gatalica ◽  
Ondrej Hes ◽  
Faruk Skenderi ◽  
Markku Miettinen ◽  
...  

1999 ◽  
Vol 161 (5) ◽  
pp. 1571-1572 ◽  
Author(s):  
MARK L. FALLICK ◽  
WILLIAM W. LIN ◽  
LARRY I. LIPSHULTZ

1985 ◽  
Vol 40 (6) ◽  
pp. 376-378
Author(s):  
ROBERT H. YOUNG ◽  
ROBERT E. SCULLY

1987 ◽  
Vol 137 (4) ◽  
pp. 747-748 ◽  
Author(s):  
Doug Corrie ◽  
John C. Norbeck ◽  
Ian M. Thompson ◽  
Francisco Rodriguez ◽  
Julius L. Teague ◽  
...  

2000 ◽  
Vol 156 (1) ◽  
pp. 347-353 ◽  
Author(s):  
Keith A. Fowler ◽  
Kiran Gill ◽  
Nameer Kirma ◽  
Dirck L. Dillehay ◽  
Rajeshwar Rao Tekmal

1983 ◽  
Vol 7 (8) ◽  
pp. 755-771 ◽  
Author(s):  
Robert H. Young ◽  
Robert E. Scully

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 5547-5547
Author(s):  
Alexander Nelson ◽  
Anne Harris ◽  
Dave Watson ◽  
Sean Robin Pyper ◽  
Nathan Hall ◽  
...  

5547 Background: Sertoli-Leydig cell tumors (SLCT) are rare ovarian sex cord-stromal tumors which occur primarily in adolescents and young adults and are associated with DICER1 pathogenic variants. Methods: Informed consent for participation in the International PPB/ DICER1 or OTST Registry was obtained. When available, pathology was centrally reviewed. Staging was evaluated by Registry review using the International Federation of Gynecology and Obstetrics (FIGO) classification system. Results: Eighty-three patients with stage I SLCT were enrolled. Median age at diagnosis was 15 (range 1-60) years. Most (57/83) patients had germline DICER1 testing; 35/57 (61%) had germline pathogenic variants. Fifty-six patients had Ia and 27 had Ic SLCT. The distribution of patients receiving chemo based on histology and stage is displayed in Table. One patient with poorly differentiated stage Ia SLCT with sarcomatous elements and no chemo at diagnosis recurred 6 months after surgery and died of disease. Three patients with local diagnosis of stage Ia SLCT, with data unavailable for Registry confirmation of stage, developed a subsequent SLCT 33 to 74 months after diagnosis; of these, 2 died, and 1 remains in treatment for recurrence. Available records and molecular testing in these 3 cases have not provided a distinction between recurrent and metachronous disease. Excluding the latter 3 patients, 3-year overall survival was 97.3% for stage Ia SLCT. Six patients with stage Ic SLCT recurred (Stage Ic1=5 and Stage Ic2=1) with a median time to recurrence of 25 (range 3-53) months. In stage Ic1, 18% (2/11) recurred after upfront chemo compared to 33% (3/9) after surgery alone. Of the 5 patients with stage Ic1 disease that recurred, 4 had intermediate and 1 had poorly differentiated SLCT. One patient had sarcomatous elements and 2 received upfront chemo. Two of the 5 patients are alive, neither received upfront chemo. One patient with poorly differentiated stage Ic2 SLCT with sarcomatous elements and no upfront chemo recurred and died of disease. Three-year event free and overall survival were 86.9 and 88.6% for stage Ic SLCT. Four patients had metachronous SLCT in the contralateral ovary confirmed by clinical review or somatic testing at a median time from diagnosis of 33 (range 28-104) months. All 4 have germline pathogenic variants and no evidence of disease at last follow-up. Conclusions: Individuals with early stage SLCT generally fare well, however, ongoing surveillance for recurrence and metachronous disease is indicated. Novel therapies are needed to address recurrent SLCT.[Table: see text]


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