ECE2014 Poster Presentations Clinical case reports Pituitary/Adrenal (50 abstracts)
A huge metastatic adrenocortical carcinoma presenting with Cushing's syndrome and inferior vena cava thrombosis: case report
Mina Gulfem Kaya 1 , Fatma Dilek Dellal 1 , Arife Ulas 2 , Mehmet Gumus 3 , Aydan Kilicarslan 4 , Bulent Yalcin 2, , Reyhan Ersoy 1, & Bekir Cakir 1,
1Department of Endocrinology and Metabolism, Ataturk Education and Research Hospital, Ankara, Turkey; 2Department of Medical Oncology, Ataturk Education and Research Hospital, Ankara, Turkey; 3Department of Radiology, Faculty of Medicine, Yildirim Beyazit University, Ankara, Turkey; 4Department of Pathology, Ataturk Education and Research Hospital, Ankara, Turkey; 5Department of Medical Oncology, Faculty of Medicine, Yildirim Beyazit University, Ankara, Turkey; 6Department of Endocrinology and Metabolism, Faculty of Medicine, Yildirim Beyazit University Ankara, Turkey.
Background: Adrenocortical carcinoma (ACC) is a very rare but typically aggressive malignancy. About one to three quarters of ACC is functioning with excess hormonal production.
Case: A 44-year-old female patient was admitted to out-patient clinic with obesity, oligomenorrhea and abdominal pain. She had central obesity, buffalo hump, and palpable flank mass. Hypertension, transient ischemic attack, and epilepsy history was present.
Serum cortisol, DHEAS, and testosterone levels were elevated. Cushing’s syndrome was diagnosed by elevated urine free cortisol and a non-suppressible dexamethasone test. Serum aldosterone and renin activity, 24 h urinary catecholamines were normal.
Abdominal CT detected a 16×11 cm hypodense solid mass lesion at the right adrenal gland. It showed inhomogeneous appearance, irregular margin, neovascularization, liver invasion, and inferior vena cava (IVC) thrombus extension (2.3–2.2 cm). It was adherent to the liver, kidney, and IVC.
Because of the unresectable adrenal mass, CT-guided abdominal mass biopsy was performed, and the diagnosis of ACC was made on the basis of pathology and immunohistochemistry. According to the AJCC staging system, the tumor was classified as T4NXM1, stage IV.
Mitotane 4.5 g/day and glucocorticoid was started. Also, coumadin was started due to presence of thrombus. The mean level of total testosterone and DHEAS tended to decrease during the follow-up.
After 5 month, radiotherapy and chemotherapy (cisplatin and etoposide per three cycle) were given to patient. Tumor regressed to 10–7.6 cm with IVC thrombus extension (2.2–1.4 cm). The patient showing partial remission underwent operation, but because of invasion to adjacent tissues, the tumor could not be resected.
Conclusion: Adrenal tumors in association with venous thrombosis are a rare pathological condition. No effective adjuvant treatment is currently available. We report a case of stage IV adrenocortical cancer with IVC thrombus extension and partial clinic response to the chemoradiotheraphy.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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