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Application of the En Bloc Concept Combined with Anatomic Resection in Laparoscopic Hepatectomy
Published on: March 10, 2023
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[A Case of Hepatic Undifferentiated Embryonal Sarcoma in an Adult]
Tomoya Miura1, Shigeru Ottomo, Ataru Sato
1Dept. of Gastrointestinal Surgery, Digestive Disease Center, Sendai Kousei Hospital.
Gan to Kagaku Ryoho. Cancer & Chemotherapy
|May 23, 2023
Summary
A rare undifferentiated embryonal sarcoma of the liver (UESL) was diagnosed in a 20-year-old woman. Surgical resection led to a favorable outcome, challenging the typical poor prognosis in adults.
Area of Science:
- Hepatobiliary Medicine
- Pediatric Oncology
- Surgical Pathology
Background:
- Undifferentiated embryonal sarcoma of the liver (UESL) is a rare, aggressive malignant mesenchymal tumor predominantly seen in infants and children.
- Adult UESL is exceptionally rare and historically associated with a poor prognosis, making diagnosis and management challenging.
Observation:
- A 20-year-old woman presented with abdominal pain and a cystic liver lesion, initially suspected to be a hemorrhagic cyst.
- Advanced imaging, including contrast-enhanced CT, MRI, and PET-CT, revealed a solid liver mass with significant 18F-fluorodeoxyglucose uptake.
- Diagnostic workup included advanced imaging modalities to characterize the liver mass.
Findings:
- Histopathological evaluation of the surgically resected right hepatic lobectomy specimen confirmed the diagnosis of undifferentiated embryonal sarcoma of the liver (UESL).
- The patient, despite refusing adjuvant chemotherapy, remained recurrence-free at 30 months post-surgery, indicating a potential for favorable outcomes with aggressive management.
Implications:
- This case highlights the possibility of adult UESL, a diagnosis often overlooked due to its rarity in this age group.
- The successful surgical management and long-term disease-free survival in this adult patient suggest that complete resection may be a viable therapeutic strategy, even in the absence of adjuvant therapy.
- Further research into adult UESL is warranted to better understand its biology, refine diagnostic criteria, and optimize treatment protocols for improved patient outcomes.
