Which ovarian tumor is most likely to secrete Dehydroepiandrosterone (DHEA)?

Steroid Cell Tumors Are Most Likely to Secrete DHEA

Steroid cell tumors of the ovary are the ovarian tumors most likely to secrete dehydroepiandrosterone (DHEA). 1

Androgen-Secreting Ovarian Tumors

Ovarian tumors that secrete androgens represent a diverse group of neoplasms, primarily from the sex cord-stromal category. These tumors can produce various androgens with different clinical manifestations:

• Steroid cell tumors are specifically associated with DHEA secretion and are often classified under the broader category of sex cord-stromal tumors 1, 2
• These tumors typically occur in younger patients (<40 years) and present with evidence of hormone production, usually androgenic in nature 1
• Steroid cell tumors have a higher malignancy rate (25-43%) compared to other sex cord-stromal tumors, which may reflect their more aggressive and poorly differentiated nature 1

Differential Diagnosis of Androgen-Secreting Ovarian Tumors

Different ovarian tumors secrete different predominant androgens:

• Steroid cell tumors → primarily secrete DHEA 1, 3
• Sertoli-Leydig cell tumors → primarily secrete testosterone 3
• Leydig cell tumors (hilus cell tumors) → primarily secrete testosterone 1
• Granulosa cell tumors → primarily secrete estrogen, but can occasionally secrete androgens 4, 5

Clinical Presentation

Patients with steroid cell tumors typically present with:

• Virilization due to excess androgen production 2, 6
• In some cases, Cushing's syndrome due to cortisol secretion 1
• Symptoms may include hirsutism, deepening of voice, male-pattern baldness, and clitoromegaly 3, 6
• Laboratory findings include elevated serum testosterone (>7 nmol/L) and elevated DHEA-S levels 2

Diagnostic Approach

When suspecting an androgen-secreting ovarian tumor:

• Measure serum testosterone, DHEA-S, and other ovarian and adrenal androgens 2
• Perform functional endocrine testing including low-dose dexamethasone suppression test (lack of suppression suggests tumor) 6
• Imaging studies including transvaginal ultrasound, CT, or MRI of the pelvis 2
• In cases where imaging is inconclusive, selective venous sampling may be necessary to localize the source of androgen production 6

Management

The primary treatment for steroid cell tumors is surgical:

• In younger patients with localized disease, fertility-sparing surgery should be considered 1
• For advanced disease, the standard approach is similar to that used for epithelial ovarian cancer 1
• In selected cases, second or subsequent surgeries may be recommended to excise recurrent isolated masses 1
• For patients who are poor surgical candidates, GnRH agonists may help control hyperandrogenism 2

Prognosis and Follow-up

• Historically, patients with sex cord-stromal tumors had poor survival (1-2 years) 1
• With aggressive surgery and platinum-based regimens, outcomes have improved significantly 1
• Long-term follow-up is essential as recurrence can occur as late as 20 years after initial diagnosis 1

Important Considerations

• Steroid cell tumors are sometimes difficult to diagnose histologically due to less distinctive patterns of differentiation 1
• The term "steroid cell tumor" is often used when there is obvious evidence of hormone production but the tumor lacks distinctive histological features of other sex cord-stromal tumors 1
• In rare cases, patients may have concurrent androgen-secreting tumors from both ovarian and adrenal sources, requiring careful diagnostic workup 3