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Adrenal cortical carcinoma with extension into the inferior vena cava – case report and literature review

Lukasz Fulawka12*, Dariusz Patrzalek3 and Agnieszka Halon1

Author Affiliations

1 Department of Pathomorphology and Oncological Cytology, Wroclaw Medical University, ul. Borowska 213, 50-556 Wroclaw, Poland

2 Department of Pathomorphology, Lower Silesian Oncology Centre, pl. Hirszfelda 12, 53-413 Wroclaw, Poland

3 Department of Surgery, 4th Military Clinical Hospital in Wroclaw, ul. Weigla 5, 50-981 Wrocław, Poland

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Diagnostic Pathology 2014, 9:51  doi:10.1186/1746-1596-9-51

Published: 6 March 2014


Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence rate of 1 to 2 per million person-years. ACC most commonly arises sporadically, but may be associated with familial tumour syndromes. Clinical symptoms are mainly related to an excess of steroid hormones. We present an unusual case of adrenocortical carcinoma in a 27-year-old male who complained of non specific mass-effect related symptoms of slowly growing intensity differing from others described in literature because of the patient’s age and the sudden deterioration of the clinical course. The tumour was resected with the left kidney with an extension into the inferior vena cava. Histological examination revealed morphological features characteristic of an adrenal cortical tumour. The immunohistochemical results (positive reactions for vimentin, CD56, inhibin, melan A, synaptophysin, bcl-2, calretinin) confirmed the diagnosis. According to the most widely used modified Weiss criteria and the Van Slooten system, a diagnosis of adrenal cortical carcinoma was strongly confirmed. The postoperative condition was poor. Reoperation was conducted, including abdominal aorta thrombectomy and aortic prosthesis implantation. The patient died two days after the second operation. Autopsy revealed a metastatic tumour in the left lung and morphological symptoms of acute circulatory collapse due to a massive haemorrhage into the abdominal cavity, which was the direct cause of death.

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Adrenal cortical carcinoma; Adrenocortical carcinoma; Adrenocortical tumour; Weiss criteria; Van Slooten criteria