case_images: PMC9492358_01
case_images: CT abdomen of Patient A on 9 August 2012. A 6.4 x 5.2 x 5.8 cm heterogeneously enhancing mass in the right adrenal gland with pre-contrast HU 35.4 (red arrow).
case_images: CRIE2022-7395050.001.jpg
case_images: PMC9492358_01_CRIE2022-7395050.001.jpg
case_images: fig1
case_images: Cushing's syndrome. Computed tomography (CT) of the abdomen performed for suspected urinary tract pathology showed a 6 cm right adrenal mass (Figure 1).
case_images: 18FDG-PET-CT scan of Patient A on 6 July 2016. CT abdomen of Madam F on 9 August 2012. An 8 cm liver mass (SUVmax 8.4) was found at segment 8.
case_images: CRIE2022-7395050.002.jpg
case_images: PMC9492358_01_CRIE2022-7395050.002.jpg
case_images: fig2
case_images: ACTH-independent hypercortisolism with marked hypertension and fluid overload in addition to clinical (hirsutism) and biochemical hyperandrogenism (Table 1). 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18FDG-PET/CT) scan showed a large solid mass at the right lobe of the liver (maximum standardized uptake value [SUVmax] 8.4), a small nodule around the previous right adrenalectomy bed (SUVmax 2.1), and multiple foci of hypermetabolic peritoneal lesions in both sides of the abdominal cavity (highest SUVmax 4.6) (Figure 2). A laparoscopic peritoneal biopsy was performed.
case_images: Photomicrographs of peritoneal nodule biopsy of Patient A. (a) Peritoneal nodule section (H&E x 40). (b) Tumor cells arranged in the cords and trabeculae (H&E x 200). (c) Hyperchromatic nuclei of tumor cells. The presence of mitosis was noted in a tumor cell (red arrow) (H&E x 400). (d) Ki-67 proliferative index was 50% (IHC x 400).
case_images: CRIE2022-7395050.003.jpg
case_images: PMC9492358_01_CRIE2022-7395050.003.jpg
case_images: fig3
case_images: Ki-67 proliferative index was 50% (Figure 3).
case_images: Photomicrographs of right adrenal tumor of Patient A on pathology review. (a) Diffuse growth pattern (H&E x 40). (b) Regular nuclei and eosinophilic cytoplasm of tumor cells (H&E x 400).
case_images: CRIE2022-7395050.004.jpg
case_images: PMC9492358_01_CRIE2022-7395050.004.jpg
case_images: fig4
case_images: Although frankly malignant features were not present in the original adrenal tumor, the presence of a diffuse growth pattern and <25% clear tumor cells (Figure 4), which were not mentioned previously, were suspicious of malignancy.
case_images: PMC9492358_02
case_images: (a) CT pulmonary artery of Patient B on 25 August 2011 showing extensive bilateral pulmonary embolism (red arrow). (b) CT abdomen of Patient B on 13 October 2011. A 3.6 x 3.5 cm lobulated heterogeneously enhancing lesion with a focal calcified spot at the lateral limb of left adrenal with 30HU in pre-contrast scan (blue arrow).
case_images: CRIE2022-7395050.005.jpg
case_images: PMC9492358_02_CRIE2022-7395050.005.jpg
case_images: fig5
case_images: She was later found to have extensive bilateral acute pulmonary embolism and was treated with intravenous thrombolysis followed by anticoagulation (Figure 5(a)). After stabilization, an abdominal US examination done for malignancy screening showed a 3 cm left suprarenal which was later confirmed to be a left adrenal mass on a CT scan (Figure 5(b)).
case_images: Photomicrograph of left adrenal tumor of Patient B. (a) Tumor cells in trabecular pattern (H&E x 100). (b) Tumor cells demonstrate nuclei of variable size and eosinophilic granular cytoplasm (H&E x 400).
case_images: CRIE2022-7395050.006.jpg
case_images: PMC9492358_02_CRIE2022-7395050.006.jpg
case_images: fig6
case_images: The tumor cells were polygonal, with pale to amphophilic cytoplasm, as well as round, deeply stained nuclei with nuclear pleomorphism (Figure 6).
case_images: CT abdomen of Patient B on 30 December 2016. (a) Presence of liver metastasis. (b) Nodule at left adrenal bed and peritoneal nodule posterior to spleen. (c) Right descending pulmonary artery embolism.
case_images: CRIE2022-7395050.007.jpg
case_images: PMC9492358_02_CRIE2022-7395050.007.jpg
case_images: fig7
case_images: An abdominal CT showed local recurrence of a 2 cm nodule at the left adrenal bed, a 4 cm liver mass, multiple peritoneal nodules, and recurrent pulmonary embolism (Figure 7). Apixaban was started for the treatment of pulmonary embolism, and US-guided fine needle aspiration of the liver mass in February 2017 showed tumor cells with round nuclei and finely vacuolated cytoplasm that were arranged in anastomosing cords with delicate vasculature, resembling the previous left adrenal tumor (Figure 8).
case_images: Photomicrograph of liver mass biopsy of Patient B. (a) Tumor cells with round nuclei and finely vacuolated cytoplasm that were arranged in anastomosing cords with delicate vasculature, resembling the previous left adrenal tumor (Figure 6) (H&E x 400). (b) Immunohistochemical staining showed diffuse positivity for Melan A.
case_images: CRIE2022-7395050.008.jpg
case_images: PMC9492358_02_CRIE2022-7395050.008.jpg
case_images: fig8
case_images: An abdominal CT showed local recurrence of a 2 cm nodule at the left adrenal bed, a 4 cm liver mass, multiple peritoneal nodules, and recurrent pulmonary embolism (Figure 7). Apixaban was started for the treatment of pulmonary embolism, and US-guided fine needle aspiration of the liver mass in February 2017 showed tumor cells with round nuclei and finely vacuolated cytoplasm that were arranged in anastomosing cords with delicate vasculature, resembling the previous left adrenal tumor (Figure 8).
case_images: PMC9492358_03
case_images: (a) Non-contrast CT abdomen of Patient B in October 2017 showing reduction of liver metastasis 8 months after mitotane (compared with Figure 7). (b) Contrast CT abdomen of Patient B in April 2018 showing the progression of liver metastasis 14 months after mitotane.
case_images: CRIE2022-7395050.009.jpg
case_images: PMC9492358_03_CRIE2022-7395050.009.jpg
case_images: fig9
case_images: February 2017 and titrated gradually to 3 g daily, which was the maximally tolerated dose to achieve a mitotane level of approximately 11 micrograms/ml. Follow-up 18FDG-PET/CT 8 months later (in October 2017) showed a mild interval reduction of liver metastasis (Figure 9(a)) together with stable disease in the left adrenal bed and peritoneal metastases. Despite the initial response, Patient B experienced significant progression of the disease since April 2018 with the growth of liver metastasis up to about 8 cm (Figure 9(b)).