Symptomatic Cushing syndrome and hyperandrogenemia revealing steroid cell ovarian neoplasm with late intra-abdominal metastasis

BMC Endocrine Disorders

Mei Zhu¹  Mingcai Qiu¹  Menghua Yuan¹ 

[1] Postal address: Department of Endocrinology, General Hospital of Tianjin Medical University, NO. 154 Anshan Road, Heiping District, Tianjin 300052, China

关键词: Hyperandrogenemia;    Cushing syndrome;    Intra-abdominal metastasis;    Not otherwise specified;    Steroid cell ovarian neoplasm;   

Others  :  1085246
DOI  :  10.1186/1472-6823-14-12

 received in 2013-11-03, accepted in 2014-02-07,  发布年份 2014

【 摘 要 】

Background

Steroid cell tumors of ovary account for less than 0.1% of all ovarian tumors and these tumours may present at any age in association with interesting presentations related to hormonal activities. The subtype, not otherwise specified (NOS), is associated with androgenic changes in 56-77% and Cushing syndrome in 6-10%. Due to the rarity of available data regarding these tumors, little is known about their malignant potential and metastatic behaviour. We hereby report an unusual metastasis of steroid cell ovarian neoplasm presented with both Cushing syndrome and hyperandrogenemia.

Case presentation

A 31-year-old woman, who had a past medical history of ovarian tumor resection (left ovarian thecoma was initially diagnosed at that time), presented with hirsutism, hypertension and menstrual disorder. Also, laboratory work-up revealed hypercortisolism and androgen excess. Computerized tomography (CT) of the abdomen showed abdominal paraaortic masses, multiple intrahepatic nodules and retroperitoneal lymph nodes enlargement. Positron emission tomography/computed tomography (PET/CT) scan demonstrated metastatic lesions. Her ovarian tumor sections were re-examined and pathology result was corrected to steroid cell tumor (NOS) associated with active cell growth and necrosis. Subsequent excision of metastatic lesions yielded clinical improvement promptly and metastasis of steroid cell tumor was confirmed by postoperative pathological studies. However, one year after the surgical management of metastasis, recurrence happened while radiotherapy was ineffective. The patient finally died of tumor metastatic recurrence.

Conclusion

This case reports a rare coexistence of Cushing syndrome and hyperandrogenemia which occurs based on metastasis of steroid cell ovarian neoplasm. It presents a real diagnostic challenge to both clinicians and pathologists. Therefore, it is very important to establish a final diagnosis by pathological studies along with clinical manifestations and imaging findings. Besides, it is necessary to improve follow-up of patients with this kind of tumors.

【 授权许可】

   
2014 Yuan et al.; licensee BioMed Central Ltd.

【 图 表 】

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