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Case Reports
. 2025 Jun 1;64(11):1653-1658.
doi: 10.2169/internalmedicine.4368-24. Epub 2024 Nov 8.

A Rare Case of Primary Hepatic Undifferentiated Pleomorphic Sarcoma: Exploring Cancer-related Gene Mutations

Hiroyuki Suzuki  1 , Michitaka Fukuda  1 , Tomotake Shirono  1 , Reiichiro Kondo  2 , Toshimitsu Tanaka  1 , Takashi Niizeki  1 , Jun Akiba  2   3 , Hironori Koga  1 , Takumi Kawaguchi  1
Affiliations
Case Reports

A Rare Case of Primary Hepatic Undifferentiated Pleomorphic Sarcoma: Exploring Cancer-related Gene Mutations

Hiroyuki Suzuki et al. Intern Med. .

Abstract

Hepatic undifferentiated pleomorphic sarcoma (UPS) is a rare malignant mesenchymal tumor with unclear cancer-related genetic mutations. In a 60-year-old Japanese woman with a rapidly growing, inoperable hepatic UPS, a genetic mutation analysis revealed KRAS and TP53 mutations. Despite initiating hepatic arterial infusion chemotherapy, the tumor continued to grow, and the patient's poor performance status complicated the transition to a phase I KRAS mutation drug trial, leading to death eight months after the symptom onset. A timely genetic mutation analysis may facilitate effective treatment transitions in hepatic UPS despite the lack of established treatments.

Keywords: KRAS; TP53; cancer genomic medicine; cancer-related gene mutation; hepatic undifferentiated pleomorphic sarcoma.

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Conflict of interest statement

Takumi Kawaguchi: Honoraria, ASKA Pharmaceutical, Taisho Pharmaceutical, Kowa, AbbVie, Eisai, Novo Nordisk Pharma, Janssen Pharmaceutical, Otsuka Pharmaceutical and EA Pharma.

Figures

Figure 1.
Figure 1.
Radiological findings at our hospital. (A) Ultrasonography: An occupying lesion lacking a clear septum is found in the right lobe of the liver. (B) Dynamic computed tomography shows no evidence of vascular occlusion within the tumor or dilation of the bile ducts at the periphery of the tumor. Delayed enhancement effects were mainly observed at the periphery of the enhanced portion. (C) Positron emission tomography shows a high degree of accumulation at the margins of the tumor in the liver. The maximum standardized uptake value was 22.93. (D) Dynamic magnetic resonance imaging: The tumor was depicted as having a low signal intensity on T1-weighted imaging (T1WI) and heterogeneous high signal intensity on T2-weighted imaging (T2WI). Diffusion-weighted imaging (DWI) showed a patchy, high signal intensity and decreased diffusion.
Figure 2.
Figure 2.
Pathological findings. (A, B) Hematoxylin and Eosin (H&E) staining. Atypical cells with a high nuclear/cytoplasm ratio proliferate in a nest-like manner (A), and there are also findings of complex spindle-shaped atypical cells (B). (C) Positive vimentin staining results. The scale bar represents 100 μm.
Figure 3.
Figure 3.
Clinical course of the patient after the symptom onset. UPS: undifferentiated pleomorphic sarcoma, CDDP: cisplatin, 5-FU: 5-fluorouracil, HAIC: hepatic arterial infusion chemotherapy
See this image and copyright information in PMC

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