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Li-Fraumeni syndrome (LFS) is a hereditary cancer predisposition syndrome associated with germline mutations in tumor suppressor gene TP53 . Perivascular epithelioid cell tumors (PEComas) are a group of tumors by the World Health Organization Classification as mesenchymal tumors composed of histologically and immunohistochemically distinctive PECs. The present study reports a rare case of PEComa associated with LFS. A 32-year-old female patient was referred to Tokyo Metropolitan Tama Medical Center (Tokyo, Japan) in March 2022 for a detailed examination of a liver mass. The patient had received a diagnosis of LFS based on a history of sarcoma and germline variants of TP53 7 years previously. Magnetic resonance imaging revealed a ring-enhanced mass in the liver segment 8 (S8). This was observed in the arterial phase, followed by washout of contrast media in the venous phase. Owing to the possibility of malignancy (such as metastatic liver tumor or hepatocellular carcinoma), the patient was referred for diagnostic surgery. In August 2022, a laparoscopic partial hepatectomy of S8 was performed without complications and she was discharged on postoperative day 7. The pathological findings led to the diagnosis of PEComa. The patient is currently under follow-up at 1 year and 4 months postoperative. Laparoscopic hepatectomy was useful as a diagnostic treatment because it was relatively non-invasive. Mutations in TP53 are involved in the development of PEComa. Further cases and studies are required to clarify the relationship between LFS and PEComa., Competing Interests: The authors declare that they have no competing interests., (Copyright: © 2024 Takada et al.)

The degree of metastatic disease varies widely among patients with cancer and affects clinical outcomes. However, the biological and functional differences that drive the extent of metastasis are poorly understood. We analyzed primary tumors and paired metastases using a multifluorescent lineage-labeled mouse model of pancreatic ductal adenocarcinoma (PDAC)-a tumor type in which most patients present with metastases. Genomic and transcriptomic analysis revealed an association between metastatic burden and gene amplification or transcriptional upregulation of MYC and its downstream targets. Functional experiments showed that MYC promotes metastasis by recruiting tumor-associated macrophages, leading to greater bloodstream intravasation. Consistent with these findings, metastatic progression in human PDAC was associated with activation of MYC signaling pathways and enrichment for MYC amplifications specifically in metastatic patients. Collectively, these results implicate MYC activity as a major determinant of metastatic burden in advanced PDAC. SIGNIFICANCE: Here, we investigate metastatic variation seen clinically in patients with PDAC and murine PDAC tumors and identify MYC as a major driver of this heterogeneity. This article is highlighted in the In This Issue feature, p. 275 ., (©2021 The Authors; Published by the American Association for Cancer Research.)

Case Presentation: A 53-year-old male underwent distal pancreatectomy with splenectomy for pancreatic body cancer. An increasing mass in the soft tissue around the common hepatic artery was detected 1 year after the primary resection and he was referred to our hospital. A low density mass measuring 16mm in length was detected around the common hepatic artery by dynamic contrast enhanced computed tomography. We diagnosed as pancreatic cancer recurrence in the pancreas bed. We performed a recurred mass resection combined with celiac and common hepatic artery resection, portal vein resection and reconstruction. Pathological examination revealed the cancer recurrence in connective tissue including nerve plexus. Adenocarcinoma cells expanded along with the nerve plexus. The tumor invaded the adventitia of the common hepatic artery. R0 resection was confirmed without exposure of cancer cells to margin. He was discharged on postoperative day 12 without any complication. He survived for 6 months after recurrence resection without metastasis., Conclusion: We experienced a case of local recurrence of pancreatic cancer successfully performed R0 resection in combination with CHA and CEA resection.