Ectopic adrenocortical carcinoma located in the ovary

Aim Ovarian corticosteroid-producing tumors are exquisitely rare. Our aim was to describe the first case observed in our practice. Case history A 34-year-old female was referred for Cushing’s syndrome (CS) occurring in the postpartum period. Clinical examination showed severe CS with diabetes mellit...

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Bibliographic Details
Published inEuropean journal of endocrinology Vol. 174; no. 10; p. K17
Main Authors Chentli, Farida, Terki, Nadia, Azzoug, Said
Format Journal Article
LanguageEnglish
Published Bristol Oxford University Press 01.10.2016
Subjects
17β-Estradiol
Abdomen
Adrenal glands
Adrenocorticotropic hormone
Computed tomography
Corticosteroids
Cortisol
Cushing syndrome
Diabetes mellitus
Embolism
Exploration
Hemoperitoneum
Inhibin
Lymph nodes
Lymphatic system
Metastases
Metastasis
Nervous system diseases
Peritoneum
Pituitary
Postpartum
Surgery
Testosterone
Tumors
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Summary:Aim Ovarian corticosteroid-producing tumors are exquisitely rare. Our aim was to describe the first case observed in our practice. Case history A 34-year-old female was referred for Cushing’s syndrome (CS) occurring in the postpartum period. Clinical examination showed severe CS with diabetes mellitus, hypertension, and a large mass in the right lower abdomen. Biochemistry demonstrated corticotropin (ACTH)-independent CS (cortisol=1900ng/mL (n=50–250), ACTH<10pg/mL (n=20–46)) with estradiol and testosterone overproduction. Investigations Abdomen CT scan revealed a 14cm right ovarian mass and small adrenal glands. Surgical exploration found the ovarian tumor with hemoperitoneum and enlarged lymph nodes. Histological study confirmed adrenocortical tumor located in the ovary with a Weiss score >5, associated with peritoneal and lymph node metastases. Immunohistochemical staining was positive for inhibin-α, melan-A, and SF1, demonstrating tissue of adrenal origin. After surgery, plasma glucose level spontaneously returned to normal. However, the patient died on the second post-surgical day due to catastrophic pulmonary embolism. Conclusion In this reported case, clinical, hormonal, histological, and immunohistochemical findings confirmed a cortisol and sex hormone-producing ovarian tumor with peritoneal and lymph node metastases, a very rare but important condition to recognize.
ISSN:0804-4643
1479-683X