Category: Other - Neuroendocrine tumors
To present a rare case of hyperandrogenism due to metastatic carcinoid tumor to the ovaries.
The clinical and paraclinical data of the patient are presented.
A 63-year-old female presented with rapid onset androgenic alopecia, increased libido, and hirsutism involving her back, breasts, and abdomen. Laboratory workup revealed a total testosterone 126 ng/dL (n 8-60), free testosterone 2.6 ng/dL (n 0.06-0.87) and DHEA-S 166 mcg/dL (n 9.7-159).
Ten months prior beeing seen in our clinic, she was evaluated at another hospital for abdominal pain and episodes of facial flushing. Abdomen and pelvis CT revealed multiple masses within the liver. Biopsy of one of these masses confirmed metastatic well-differentiated neuroendocrine tumor, Ki-67 was not reported. At that time she decided to come to our hospital for further evaluation. Functional PET imaging with 68-Ga dotatate and MRI of the abdomen and pelvis were obtained to aid in tumor localization and staging. PET showed dotatate-avid lesion in the jejunum likely to be primary carcinoid tumor and intense hypermetabolic sites involving the mesentery, left adnexa, and liver, suggestive of metastatic disease. MRI abdomen and pelvis revealed distal jejunal carcinoid with metastasis in the adjacent mesentery and liver as well as a left adnexal mass measuring 2.4 cm. She was started on Lanreotide to control flushing and tumor growth.
The markedly elevated testosterone level and the presence of ovarian mass on imaging, raised the concern of an androgen producing ovarian tumor. The patient underwent a bilateral saplingo-oophorectomy, and pathology revealed metastatic well-differentiated neuroendocrine tumor involving the ovaries and the fallopian tubes. Immunostaining was diffusely positive for CDX-2, favoring a gastrointestinal primary. Ki-67 immunostaining revealed only rare positive tumor cells (<3%), consistent with a well-differentiated tumor, grade 1. Six weeks after the surgery, repeat total and free testosterone levels were within normal limits.
Ovarian carcinoid tumors account for 0.3% of all ovarian tumors and 0.5% of all carcinoid tumors. Metastatic carcinoid tumors to the ovary are rare, usually bilateral and arise from primary ileal carcinoid tumor. Hyperandrogenism due to various types of ovarian tumors is well documented in the medical literature. However, androgen excess and virilization due to ovarian carcinoid tumors is extremely rare.
We present a 63 year old post-menopausal female with clinical and biochemical evidence of hyperadnrogenism due to a metastatic carcinoid to the ovaries. The patient underwent bilateral salpingo-oophorectomy with normalization of the testosterone levels.
Mayo Clinic Florida
Endocrinology fellow at Mayo Clinic, Florida.