case_images: PMC11055651_01
case_images: Findings of dynamic CT. (a-b) A tumor showing cystic degeneration was found in the pancreatic tail that showed gradually progressive staining from the arterial phase to the equilibrium phase. (c-d) Multiple liver metastases were also present in this case, one of which in the liver S8 showed the same contrast enhancement pattern as the pancreatic lesion. (e) Significant bilateral adrenal gland enlargement is seen. (f) Ground-glass appearance of both lungs with some infiltrative opacities, possibly suggestive of pneumocystis carinii pneumonia. (g) Compression fractures of the thoracolumbar spine due to osteoporosis.
case_images: CRIE2024-5923680.001.jpg
case_images: PMC11055651_01_CRIE2024-5923680.001.jpg
case_images: fig1
case_images: CT to rule out suspected pulmonary embolism, which revealed a progressively staining tumor in the pancreatic tail, multiple liver tumors showing similar contrast effects to those of the pancreatic tumor, marked adrenal enlargement, osteoporotic changes, and ground-glass appearance and infiltrative opacities in both lungs (Figure 1). Cushing's syndrome and associated infection with pneumocystis carinii pneumonia was suspected.
case_images: Immunohistochemical examination of pancreatic tumor. ACTH-secreting pNET showing positive immunostaining of the tumor cells for SSTR2 (a), SSTR5 (b), Chromogranin A (c), and ACTH (d).
case_images: CRIE2024-5923680.002.jpg
case_images: PMC11055651_01_CRIE2024-5923680.002.jpg
case_images: fig2
case_images: In addition, some tumor cells showed positive immunostaining for ACTH, strongly positive staining of the cell membrane for SSTR2, and slightly weaker staining for SSTR5, but positive staining of the cell membranes was observed in more than 50% of tumor cells (Figure 2).
case_images: Octreoscan image. No significant accumulation was observed in the pancreatic tumor or liver metastases.
case_images: CRIE2024-5923680.003.jpg
case_images: PMC11055651_01_CRIE2024-5923680.003.jpg
case_images: fig3
case_images: ACTH-producing NET with liver metastasis. An Octreoscan (111In-pentetreotide SPECT) performed to investigate the usefulness of octreotide acetate and intranuclear therapy revealed no significant accumulation in either the pancreatic lesion or the liver metastases (Figure 3) (scintigraphy with DOTATOC (68Ga-DODATOC PET), which is thought to be more sensitive for detecting metastatic NETs than Octreoscan is not yet popularly used in Japan).
case_images: Schema for the SACI test and angiography. (a) Schema for the selective arterial calcium injection (SACI) test. Stars indicate the arteries tested in the current patient. (b) A catheter was inserted from the femoral vein into the middle hepatic vein and a catheter was inserted percutaneously and placed transhepatically into the main trunk of the portal vein, and the ACTH concentrations were measured. (c) SMA angiography. The replaced common hepatic artery (CHA) and dorsal pancreatic artery (DPA) are depicted. (d) Selective angiography from DPA showed that the pancreatic tumor was stained in a basket shape.
case_images: CRIE2024-5923680.004.jpg
case_images: PMC11055651_01_CRIE2024-5923680.004.jpg
case_images: fig4
case_images: A 4Fr. catheter was inserted percutaneously and was placed transhepatically into the main trunk of the portal vein (PV) (Figure 4(b)). Superior mesenteric artery (SMA) angiography with a 4Fr. catheter indicated that the main feeding vessel for the tumor was the dorsal pancreatic artery (DPA) (Figures 4(c) and 4(d)). Using a microcatheter, the distal splenic artery (SPA dis), proximal splenic artery (SPA pro), proper hepatic artery (PHA), gastroduodenal artery (GDA), and dorsal pancreatic artery (DPA) were selected (Figure 4(a)) and after 0.025 mEq/kg (2.9 mL) of calcicol injection into each, blood samples were collected from the MHV and PV (preload, and 30, 60, 90, and 120 seconds postload).
case_images: Results of the SACI test. (a) ACTH concentration in the inferior vena cava before the calcium injection: changes in the ACTH concentrations of the portal vein and middle hepatic vein after selective arterial calcium injection are shown. (b-c) The graph shows the ratio of ACTH concentration before and after the SACI test in PV and MHV. (d) Differences in the ACTH concentrations in the portal vein and middle hepatic vein after selective arterial calcium injection.
case_images: CRIE2024-5923680.005.jpg
case_images: PMC11055651_01_CRIE2024-5923680.005.jpg
case_images: fig5
case_images: Comparison of the levels in the proximal and distal inferior vena cava prior to the calcium injection showed an increase in the ACTH concentrations, suggesting increased ACTH concentration in the portal or hepatic venous system (Figure 5(a)). The results of the selective arterial calcium injection showed that the injection into the DPA increased the ACTH concentrations in the PV and MHV (Figures 5(b) and 5(c)). In addition, no significant ACTH concentration differences were observed in the MHV and PV after calcium loading of either artery (Figure 5(d)).
case_images: Changes in the ACTH level during the course of treatment. No decrease in the blood ACTH levels was observed after treatment with lanreotide or sunitinib.
case_images: CRIE2024-5923680.006.jpg
case_images: PMC11055651_01_CRIE2024-5923680.006.jpg
case_images: fig6
case_images: ACTH levels, ACTH production was not suppressed even after administration of sunitinib, which is a kinase inhibitor, and in fact, it shows a rapid increase following this treatment (Figure 6). As the cortisol levels decreased, the general condition of the patient transiently improved to the point where she was able to live independently without the need for assistance from others.