PMC9343591_01
Enhanced MRI of the abdomen. (A) T1WI, (B) T2WI, (C) Enhancement phase, (D) Delay phase: the pancreatic head could be seen a clear outline of about 2.5cm in diameter with long T1T2 signal, the lower segment of the right liver had oval shadow of about 1.4cm in diameter with long T1T2 signal, the spleen showed multiple irregular T1 signals and long T2 signals; (E) T1WI, (F) T2WI, kidneys could be seen round T1 signal and low T2 signal foci, and the enhanced arterial phase showed obvious enhancement. (red arrow: pancreatic tumor; blue arrow: splenic tumor; green arrow: liver tumor; yellow arrow: kidney tumor).
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Enhanced MRI of the abdomen confirmed multiple space-occupying changes in the liver, spleen, pancreas, and kidneys ( Figure 1 ).
Tumor specimens of surgical excision. (A) A tumor with a diameter of about 1 cm was seen in the caudate lobe of the liver with a gray-white nodule in the center; (B) The cut surface of the splenic tumor was grayish-red, gray-brown and medium in quality; (C) The pancreatic tumor was located on the body of the pancreas with a diameter of about 2.5 cm. The cut surface was grayish yellow and soft with bleeding.
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After opening the abdomen, a tumor of diameter 2.5 cm was found in the body of the pancreas ( Figure 2 ).
Pathology result of the pancreas. (A) Pancreatic tumor cells were mostly distributed in strips or diffuse sheets, with the same cell size; (B) Immunohistochemistry CK(PAN)(+); (C) Immunohistochemistry SY(+); (D) Immunohistochemistry beta -Catenin (cell membrane+).
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Microscopic examination of the paraffin-embedded histopathological section showed that the tumor cells of the pancreas ( Figure 3 ) were mostly distributed in strips or diffuse sheets, with the same cell size and characteristic salt and pepper-like nuclei.
Pathology result of the liver. (A) The boundary between the liver tumor and the surrounding liver tissue was unclear. The tumor was the mixture of epithelioid cells, perivascular muscle-like cells and adipocytes in different proportions; (B) Immunohistochemistry CK (hepatocyte+); (C) Immunohistochemistry Melan A(+); (D) Immunohistochemistry SMA (part+).
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The tumor and the surrounding tissues of the liver were unclear ( Figure 4 ).
Pathology result of the spleen. (A) The splenic tumor was in the shape of a round nodule, with a clear boundary with the surrounding normal spleen tissue. The tumor lost the structure of the red and white pulp of the normal spleen under the microscope. The cells were mainly composed of oval tissue-like cells and spindle-shaped muscle-like cells. Blood vessels were abundant; (B) Immunohistochemistry CD8(+); (C) Immunohistochemistry CD68(+); (D) Immunohistochemistry SMA (interstitial +).
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The splenic tumor was a round-shaped nodule ( Figure 5 ) with clear boundary.
The result of genetic mutation detection. (A) BAP1 gene c.1111dupA site with insertion mutation; (B) TSC2 gene c.4700G>A (guanine>adenine) site with heterozygous mutation.
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The results showed that the tumor susceptibility syndrome-related genes BRCA1 related protein 1 (BAP1) and tuberous sclerosis complex 2 gene (TSC2) each presented with a heterozygous variant ( Figure 6 ).
Diagnosis and treatment algorithm based on this case report.
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Tumor markers and ultrasound were re-examined at 3 and 6 months after surgery, and there was no indication of recurrence. The patient's diagnosis and treatment processes are shown in  Figure 7 .