Work

A rare case of undifferentiated embryonal sarcoma of the liver in an elderly patient misdiagnosed as intrahepatic cholangiocarcinoma

Year: 2025

Type: article

Abstract: Undifferentiated embryonal sarcoma of the liver (UESL) is an exceptionally rare and aggressive malignancy, predominantly affecting children. Its occurrence in the elderly is exceedingly uncommon, posing significant diagnostic challenges. We present the oldest-reported case of UESL, initially misdiagnosed as intrahepatic cholangiocarcinoma (iCCA). An 88-year-old male presented with an upper abdominal mass and discomfort. Imaging (CT/MRI/PET-CT) and mildly elevated CA19–9 initially suggested iCCA (T1bN0M0, Stage IB). After 10 months of disease progression despite chemotherapy (capecitabine), left hemihepatectomy was performed. Histopathological examination revealed UESL (7.5 cm tumor; IHC: Vimentin+, CD10+, CD56+, Ki-67 80 %). The patient developed recurrent hepatic disease and pulmonary metastases 1.5 months postoperatively and succumbed to the disease shortly thereafter, with an overall survival of approximately one year. UESL in octogenarians is exceptionally rare, with nonspecific imaging and serological findings (e.g., mild CA19–9 elevation) leading to frequent misdiagnosis, often as iCCA. Pathological examination remains essential for definitive diagnosis. Despite radical resection, prognosis in elderly patients is poor due to aggressive biology and limited tolerance for adjuvant therapy. This case underscores the importance of considering UESL in atypical liver masses across all age groups. UESL should be included in the differential diagnosis of atypical liver masses, even in elderly patients. Early surgical resection offers the best chance for survival, though outcomes remain dismal in advanced age due to rapid recurrence and metastasis. Heightened awareness of this entity is crucial to mitigate diagnostic delays. • Oldest UESL case in an 88-year-old, expanding the age spectrum of this rare cancer. • Misdiagnosed as iCCA due to atypical imaging & mild CA19-9 rise, showing UESL's diagnostic complexity in elderly. • Rapid recurrence (liver & lung mets) 1.5 months post-surgery, poor prognosis in elderly despite resection. • UESL confirmed by IHC (Vimentin, CD56+, Ki-67 80%) after inconclusive imaging/serology. • Consider UESL in differentials for atypical liver masses in all ages to reduce diagnostic delay.

Source: International Journal of Surgery Case Reports

DOI: 10.1016/j.ijscr.2025.112059

Authors: Xiang-Hui Geng, Deng-Shuai Li, Xing-Fu Wang, Wei Zhong, Min-Feng Liang, Jie Lin

Institutions: First People's Hospital of Foshan, The First People's Hospital of Shunde

Language: English

Topic: Sarcoma Diagnosis and Treatment

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,090