Abstract
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.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1602226377106882.
Highlights
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence rate of 1 to 2 cases per million person-years [1,2,3]
In this paper we report the case of an adrenocortical carcinoma extending into the inferior vena cava and metastasis to a lung, with a fatal outcome
Histopathological examination of the thrombus from the inferior vena cava revealed a tumour mass with necrosis, intermingled with fibrin (Figure 2I)
Summary
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence rate of 1 to 2 cases per million person-years [1,2,3]. Dissection of the inferior vena cava revealed a grey mass bound to its wall in the area of prior surgical excision. Left lung dissection revealed a solid whitish well-circumscribed tumour of 2 cm in diameter in the superior lobe. The bronchi of both lungs contained a thick transparent secretion. A few scattered cells among the tumour mass revealed weak chromogranin A immunoreactivity (Figure 5H). Histopathological examination of the thrombus from the inferior vena cava revealed a tumour mass with necrosis, intermingled with fibrin (Figure 2I). Microscopic examination of the lung tumour revealed a solid pattern similar to the predominant area of the primary tumour with cell nests and a vascular network
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