PMC6817479_01
Hyperpigmentation of the patient's skin (A), knuckles and nails (B), and palms (C) was photographed in January 2016.
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F1
By the end of 2015, his skin color had darkened to a great extent (Figures 1A-C).
The morning serum ACTH level was monitored from April 2013 to March 2019. Bilateral laparoscopic adrenalectomy was performed in August 2013. Note that his ACTH level increased to over 1,250 pg/mL after January 2015, with the highest level reaching 21,500 pg/ml. However, the ACTH level dropped significantly after the patient underwent CAPTEM treatment. The last follow-up test indicated that the ACTH level dropped to 3,660 pg/ml in March 2019.
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F2
His morning ACTH level had increased to over 1,250 pg/ml (Figure 2).
(A-C) Cardiac MRI findings acquired at different time points. The tumor was located at the junction between the anterior wall of the left ventricle and the middle septum (arrows). The tumor was 18 x 23 x 27 mm in size when initially found in January 2016 (A). It had increased in size to 20 x 24 x 28 mm in November 2016 (B). After combined chemotherapy with capecitabine/temozolomide for 12 months, the tumor size was 16 x 22 x 22 mm in January 2018 and remained stable to March 2019 (C). (D-H) The pathological findings of the bone biopsy of the metastatic vertebrae. Hematoxylin and eosin (HandE) staining showed an invasive tumor cell growth pattern, with abundant eosinophilic cytoplasm and finely granular nuclear chromatin. Mitosis and necrosis were frequent (D). Positive immunohistochemistry staining for ACTH (E), chromogranin A (F), and synaptophysin (G). The Ki-67 index was approximately 20% (H) (Magnification, x 200).
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F3
Cardiac MRI revealed a mass 18 x 23 x 27 mm in size at the junction between the anterior wall of the left ventricle and the middle septum, involving the adjacent myocardium and pericardium (Figure 3A). In November 2016, cardiac MRI revealed a slightly enlarged, irregular cardiac lesion (20 x 24 x 28 mm) at the same location (Figure 3B). The tumor cells were well-differentiated, and mitosis and necrosis were frequent (Figure 3D). The tumor cells showed positive staining for ACTH, chromogranin A and synaptophysin (Figures 3E-G), as well as cytokeratin. The Ki-67 index was 20% (Figure 3H). Beginning in March 2017 to March 2019, he underwent 24 cycles of chemotherapy without any evidence of further progression of the cardiac lesion (Figure 3C).