Steroid cell tumor: a rare cause of hirsutism in a female

Abstract

SummaryOvarian steroid cell tumors are very rare functioning sex-cord stromal tumors. They comprise <0.1% of all ovarian tumors. Previously designated as lipoid cell tumors, one-third of these tumors are considered malignant with the mean age of presentation at around 40 years. We present a case of a 28-year-old female with 2-year history of hirsutism, virilization, and amenorrhea. She was diagnosed with left ovarian tumor, for which she underwent left salpingo-oophorectomy. Histopathology revealed not otherwise specified subtype of steroid cell tumors. The patient resumed menses 2 months after the features of masculinization regressed. Within 1 year of surgery, the patient successfully conceived a full-term baby without any complications. In a young female, the neoplastic etiology of a rapid virilization or menses changing should always be kept in mind. Though commonly observed in adult females, steroid cell tumors have very good surgical outcomes if age at presentation is less and tumor is unilateral, and there are no evidences of bilateral malignancy. Bilateral salpingo-oophorectomy is not required.Learning points In a case of severe rapid hirsutism and virilization with serum testosterone level more than 200 ng/dl or more than threefold of the normal range, neoplastic conditions should always be suspected.Steroid cell tumor in young women without evidence of malignancy on histopathology has excellent surgical outcomes.Unilateral salpingo-oophorectomy is the surgery of choice.As the frequency of bilateralism is only 6%, prophylactic unaffected side oophorectomy need not be done.

Steroid cell tumour of the ovary: a case report with review of literature

“…12 Steroid cell tumours are divided into three subtypes according to their cell of origin: stromal luteoma arising from ovarian stroma, Leydig cell tumour arising from Leydig cells and steroid cell tumour not otherwise specified (NOS) when the lineage of the tumour is unknown. 13,14 Macroscopically steroid cell tumours are often yelloworange, reflecting their high lipid content, with hemorrhagic and cystic areas rarely seen. 15 The tumour is usually confined to the ovary, but few cases with extraovarian steroid cell tumour have been reported.…”

Leydig cell tumour and mature ovarian teratoma: rare androgen-secreting ovarian tumours in postmenopausal women

“…The measurement of serum androgens is important to differentiate between ovarian or adrenal source 8. Marked elevation of testosterone levels, especially above 200 ng/dL (2 ng/mL), should raise the suspicion of an androgen-producing tumour (adrenal or ovary) 3. A normal serum level of DHEA-S typically excludes an adrenal cause, while a value <600 µg/dL makes an adrenal neoplasm the most probable diagnosis 9…”

“…A Leydig stromal cell tumour is a rare ovarian tumour that belongs to the group of sex-cord stromal tumours and accounts for <0.1% of all ovarian tumours 1 3. More than 75% of patients show signs of virilisation, resulting from autonomous androgen secretion.…”

“…Hyperandrogenism can result in a virilisation syndrome, which is characterised by clitoral hypertrophy, deepening of the voice, increased libido, breast atrophy, severe hirsutism and male-pattern baldness 1 2. These tumours should be suspected when the onset of androgenic symptoms is sudden and rapidly progressive,3 especially if associated with signs of virilisation or defeminisation. In most cases, hormonal abnormalities include increased serum testosterone levels in the presence of normal dehydroepiandrosterone-sulfate (DHEA-S) 3…”

“…Among functional ovarian neoplasms, sex-cord stromal tumours are the ones most commonly associated with hyperandrogenism 3. According to the WHO, Leydig cell tumours are classified in this group and account for 0.1% of all ovarian tumours 1 4…”