case_images: PMC10514511_01
case_images: Timeline of laboratory results. (A) The cortisol and aldosterone concentrations plotted against time. Magnetic resonance imaging and computerized tomography showed multiple metastases in both the liver (B, D, F, H) and lungs (C, E, G, I) at diagnosis and following initiation of therapy.
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case_images: Plasma cortisol was 705.21 nmol/L at 8 a.m. and 680.50 nmol/L at 24 p.m. (reference range: 118.60-610.00 nmol/L), plasma adrenocorticotropic hormone (ACTH) was < 5 pg/mL (reference range: 0-46 pg/mL), indicating excessive cortisol and disturbed rhythm ( Figure 1A  ). Figures 1B, C  ). Fluorodeoxyglucose positron emission tomography showed a strong accumulation in the left adrenal mass (SUV max of 8.8,  Figure 3A ) and metastatic lesions in the lung (SUV max of 8.7,  Figure 3B ) and liver (SUV max of 17.1,  Figure 3C ), but no lesions were detected in the bone or lymph nodes. After the first cycle of EP-M, he was assessed as having progressive disease (PD): the maximum size of the liver lesions grew to 64 mm and that of the lung lesion grew to 16 mm ( Figures 1D, E  ). Figures 1F, G  ). Following eight cycles of EP, the patient continued to receive sintilimab and mitotane only, and eventually, sintilimab monotherapy was used to maintain SD ( Moreover, neither relapsed disease at the primary site nor new metastases were detected (  Figures 1H, I  ). On 24th June 2021, the patient's plasma aldosterone and cortisol decreased to 48 ng/L and 168 nmol/L, respectively ( Figure 1A ).
case_images: Red arrowheads indicate the primary tumor at diagnosis. Contrast-enhanced magnetic resonance imaging revealed a 158 mm x 112 mm heterogeneously enhanced left suprarenal mass with necrosis.
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case_images: T2-emphasizing phase images and low density on T1-emphasizing phase images (  Figure 2  ). There were 14 metastases in the liver, and the largest metastasis was about 44 mm in diameter.
case_images: Positron emission tomography-computed tomography showed primary tumor in the left adrenal gland (A) and multiple metastases in both lungs (B) and liver (C) before treatment. The red arrowheads showed the primary lesion of the left adrenal gland and the metastatic lesions of the lungs and liver.
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case_images: Figures 1B, C  ). Fluorodeoxyglucose positron emission tomography showed a strong accumulation in the left adrenal mass (SUV max of 8.8,  Figure 3A ) and metastatic lesions in the lung (SUV max of 8.7,  Figure 3B ) and liver (SUV max of 17.1,  Figure 3C ), but no lesions were detected in the bone or lymph nodes.
case_images: Presentation of the resected adrenocortical carcinoma (ACC) tumor tissue. (A) Resected (ACC) tumors. The size of the tumor is 17 cm x 10 cm x 8 cm. (B) Representative hematoxylin and eosin-stained photomicrograph of ACC (scale bar = 200 mum).
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case_images: When the patient's general condition improved, he then underwent the palliative left adrenalectomy on 22nd April 2021, and a subsequent tumor biopsy confirmed the diagnosis of ACC ( Figure 4A ), with a Weiss score of 7 points. The pathological results revealed vascular invasion with a Ki-67% of 75% and a mitotic count of 40/50 high-power field ( Figure 4B ).