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Diagnostic & Interventional Imaging

Essential image/Abdominal imaging

Right-sided adrenocortical carcinoma with inferior vena cava tumor thrombus

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A 60-year-old man with no remarkable past history underwent computed tomography (CT) examination of the thorax, abdomen and pelvis for the investigation of thoracic pain. CT revealed a heteroge- neous mass of the right adrenal gland measuring 120 x 100 mm in the axial plane, without fat border between the mass and the liver (Fig. 1). Magnetic resonance imaging (MRI) confirmed heteroge- neously enhancing mass of the right adrenal gland, with visible extension into the inferior vena cava (IVC) on T2-weighted and con- trast-enhanced MRI. In addition, MRI revealed contour disruption and bulking of the tumor suggesting direct liver involvement [1]. 18

Fluorodeoxyglucose (18F-FDG) PET/CT examination confirmed marked uptake of 18F-FDG by adrenal mass and tumor thrombus. Hormonal work-up found isolated autonomous excess of glucocorti- coids. The diagnosis of adrenocortical carcinoma (ACC) was strongly suspected. The patient underwent open surgery for en bloc resection of the right adrenal gland, the right kidney, involved liver paren- chyma and endoluminal tumor bud into the IVC under cross-clamp- ing. The diagnosis of ACC with partial rupture of fibrous capsule and direct liver involvement was further histopathologically confirmed. Weiss score was calculated at 8/9 and Ki67 index estimated at 10%.

Fig. 1. 60-year-old man with adrenocortical carcinoma of right adrenal gland and inferior vena cava tumor thrombus. A, CT image obtained in the axial plane during the arterial phase of enhancement following intravenous administration of iodinated contrast material shows large, heterogeneous adrenal mass (arrows) that compresses the right lateral sec- tion of the liver. The mass shows internal heterogeneous enhancement. B, Fat-suppressed T2-weighted MR image (BLADE; TR/TE = 2380/110 msec) in the axial plane shows hetero- geneous adrenal tumor protruding into the inferior vena cava (arrow). C, Fat-suppressed 3D T1-weighted MR image (VIBE; TR/TE/a = 4/2 msec/15°) obtained in the axial plane during the late phase (5 min) of enhancement following intravenous administration of a gadolinium-based contrast agent (gadoterate meglumine, Dotarem®, Guerbet) shows het- erogeneous right adrenal mass with tumor thrombus (arrow) extending into the inferior vena cava. D, 18F-FDG PET/CT fused image in the axial plane shows marked uptake of 18F- FDG by tumor thrombus (arrow). E, Photograph shows intraoperative view of tumor thrombus (arrow) within inferior vena cava lumen. F, Photograph shows gross specimen after en bloc resection of right adrenal gland and right kidney. Weiss score was calculated at 8/9 and Ki67 index estimated at 10%.

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ACC is a rare endocrine malignant tumor. Contrast-enhanced imaging often demonstrates heterogeneous enhancement with non-enhanc- ing areas secondary to hemorrhage or necrosis. IVC invasion at initial presentation is found in less than 20% of patients with right ACC and strongly suggests malignant ACC. ACC with IVC extension is classified as stage III according to the European Network for the Study of Adre- nal Tumors staging classification. Because of a high risk of recurrence, adjuvant chemotherapy is advocated even when surgical resection is complete (i.e., R0). IVC invasion is more common with right-sided ACC because right adrenal vein is short and drains immediately into the IVC. The diagnosis of tumor thrombus is confidently made using MRI and 18F-FDG PET/CT examination.

The authors declare that this report does not contain any personal information that could lead to the identification of the patient.

Funding

This work did not receive any grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author contributions

All authors attest that they meet the current International Com- mittee of Medical Journal Editors (ICMJE) criteria for Authorship.

Disclosure of interest

The authors declare that they have no competing interest.

Acknowledgments

The authors thank Prof. Benoit Terris, MD, PhD for providing pho- tograph of gross specimen.

Reference

[1] Kedra A, Dohan A, Gaujoux S, Sibony M, Jouinot A, Assié G, et al. Preoperative detection of liver involvement by right-sided adrenocortical carcinoma using CT and MRI. Cancers 2021;13:1603.

Martin Gaillard* Department of Digestive, Pancreatic, Hepato-biliary and Endocrine Surgery, Cochin Hospital, Assistance Publique - Hôpitaux de Paris (APHP), Paris 75014, France

Université Paris Cité, Faculté de Médecine, Paris 75006, France

Philippe Soyer

Université Paris Cité, Faculté de Médecine, Paris 75006, France Department of Body and Interventional Imaging, Hôpital Cochin, AP-HP, Paris 75014, France

Maxime Barat

Université Paris Cité, Faculté de Médecine, Paris 75006, France Department of Body and Interventional Imaging, Hôpital Cochin, AP-HP, Paris 75014, France

*Corresponding author at: Department of Digestive, Pancreatic, Hepato-biliary and Endocrine Surgery, Cochin Hospital, Assistance Publique - Hôpitaux de Paris (APHP), Paris 75014, France.

E-mail address: martin.gaillard@aphp.fr (M. Gaillard).