case_images: PMC10516602_01
case_images: (A, B) A large heterogeneously enhancing mass was suspected to arise from the upper pole of the right kidney; a normal right upper renal pole could not be appreciated separate to the mass. (C, D) The mass has decreased in size following systemic therapy consistent with a good partial response to treatment. (E, F) A further reduction in size following additional systemic treatment.
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case_images: PMC10516602_01_fonc-13-1242560-g001.jpg
case_images: f1
case_images: A CT scan of the chest, abdomen and pelvis in the portal venous phase showed a large heterogeneously enhancing mass, again suspected to arise from the upper pole of the right kidney, with possible invasion into the right hepatic lobe both superiorly and inferiorly ( Figures 1A, B ). A CT scan after four cycles of ipilimumab and nivolumab showed a partial response to treatment ( Figures 1C, D ). Whilst the MDT discussions and surgical work-up was ongoing, the patient completed two cycles of single agent nivolumab (480mg every 4 weeks) ( Figures 1E, F  and  2D-F ).
case_images: (A, D) Coronal T1-weighted VIBE-Dixon images, 5 minutes post-contrast. (B, E) Coronal T2-weighted HASTE fat-saturated images. (C, F) Axial T2-weighted HASTE images. (A-C) A large mixed signal mass, again suspected to arise from the upper pole of the right kidney. 2A demonstrates contrast-enhancement within the liver parenchyma adjacent to the mass, which was initially suspected to represent hepatic parenchymal invasion. (D-F) A significant reduction in size to the mass post-treatment. Following systemic therapy there is the impression of a fat plane between the right kidney and the mass itself, particularly in 2D, such that the possibility of this mass arising from the adrenal gland, rather than kidney, could have been highlighted.
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case_images: PMC10516602_01_fonc-13-1242560-g002.jpg
case_images: f2
case_images: An upper abdominal MRI scan showed a lobulated mass, again suspected to arise from the upper pole of the right kidney, with broad contact to the under surface of the liver, and with suspected liver parenchymal invasion ( Figures 2A-C ). Whilst the MDT discussions and surgical work-up was ongoing, the patient completed two cycles of single agent nivolumab (480mg every 4 weeks) ( Figures 1E, F  and  2D-F ).
case_images: Right hepatectomy with en-bloc adrenalectomy showing an encapsulated tumour which was adherent to the liver. The tumour has a variegated appearance with solid, oedematous, haemorrhagic and necrotic areas. The tumour did not appear to invade the liver parenchyma macroscopically.
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case_images: The excision specimen revealed an encapsulated tumour which was adherent to the liver ( Figure 3 ).
case_images: Excision of the adrenal tumour showing features consistent with an adrenal cortical carcinoma. Note the clear cytoplasm, marked nuclear atypia and patchy lymphoplasmacytic infiltrate. Areas of cells with eosinophilic cytoplasm, frequent mitoses, confluent necrosis, oedema, hyalinisation and haemorrhage were noted in other sections.
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case_images: PMC10516602_01_fonc-13-1242560-g004.jpg
case_images: f4
case_images: On microscopic assessment, the tumour showed features similar to those seen in the pre-operative biopsy and consistent with an ACC ( Figure 4 ).