case_images: PMC11361937_01
case_images: (A) Thoracic and abdominal CT scan before the therapy (2020-08) showing the following: mass in the left adrenal gland measuring approximately 110 mm x 87 mm suggestive of malignant tumor and multiple nodules in bilateral lungs suggestive of metastasis. (B) Thoracic and abdominal CT scan after four-cycle EP chemotherapy and anlotinib combined with sintilimab immunotherapy (2021-3): the left adrenal mass has significantly subsided, with a size of approximately 51 mm x 67 mm. Reduction in bilateral multiple pulmonary metastatic nodules. (C) Thoracic and abdominal CT scan after maintenance therapy for 8 months (2021-11): a mass in the left adrenal gland area with a cross-section of approximately 41 mm x 28 mm. The size of multiple bilateral lung metastasis further decreased. (D) Thoracic and abdominal CT scan after maintenance therapy for 24 months (2023-03): a mass in the left adrenal gland area with a cross-section of approximately 39 mm x 29 mm. The size of multiple bilateral lung metastasis further decreased. The whole red arrowheads show the before and after treatment of primary lesion of the left adrenal gland and the metastatic lesions of the lungs.
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case_images: Thoracic, upper and lower abdomen, and pelvic contrast-enhanced CT scan results showed multiple nodules in bilateral lungs suggestive of metastasis, enlarged mediastinal and right supraclavicular lymph nodes, and mass in the left adrenal gland measuring approximately 110 mm x 87 mm suggestive of malignant tumor ( Figure 1A ). Follow-up CT scan ( Figure 1B ) showed significant reduction in size of adrenal gland mass and disappearance of some lung metastases, indicating partial response to treatment. The size of multiple bilateral lung metastasis further decreased ( Figure 1C ). On 4 March 2023, the patient returned to receive treatment, and the therapeutic evaluation ( Figure 1D ) indicated stable disease (SD), without symptoms such as cough, chest tightness, or asthma.
case_images: The cortisol and estradiol plotted against time (02/08/2020 to 04/03/2023): the initial sex hormone laboratory assessment was dated 02/08/2020, followed by subsequent assessments at approximately 1-month (23/09/2020), 3-month (24/11/2020), 6-month (01/03/2021), 9-month (13/06/2021), and 15-month (15/11/2021) intervals from the initial test, culminating in the final follow-up assessment in 04/03/2023.
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case_images: The patient's adrenaline was 82.9 pmol/L (reference range, 0-605.9 pmol/L), noradrenaline was 2,243.2 pmol/L (reference range, 413.9-4,434.2 pmol/L), dopamine was 105.6 pmol/L (reference range, 0-196.00 pmol/L) ( Figure 2 ). Sex hormones, including cortisol and estradiol, decreased over time to the normal range ( Figure 2 ).
case_images: (A) Pathology and immunohistochemistry: the biopsy was performed on the mass in the left kidney area, which revealed pathological findings indicating malignancy likely originating from adrenocortical tissue. Immunohistochemistry results showing increased eosinophils with mucinous degeneration along with mild nuclear atypia and visible mitotic figures among tumor cells. (B) hematoxylin-eosin staining dyes x100. (C) Positive expression of Melan-A in ACC section x100. (D) Positive expression of Syn in ACC section x100. (E) Positive expression of Ki-67 (approximately 10%+) in ACC section x100. (F) Negative expression of RCC in ACC section x100.
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case_images: Under ultrasound guidance, a biopsy was performed on the mass in the left kidney area, pathologically revealing increased eosinophils with mucinous degeneration along with mild nuclear atypia and visible mitotic figures among tumor cells, which are rich in blood sinuses ( Figures 3A, B ).
case_images: Treatment events timeline for the case: the patient was treated with EP and sintilimab for six cycles; anlotinib was introduced after the third cycle. Maintenance treatment with sintilimab and anlotinib lasted for 1 year.
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case_images: From 24 September to 22 December 2020, the patient received "etoposide at a dose of 0.1g D1-4 + cisplatin at a dose of 40mg D1-3 + sintilimab injection at a dose of 200mg D1+ anlotinib capsules at a dose of 8mg D1~14 q3w" for four cycles ( Figure 4 ).