Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient.
Amy ArmstrongFouad BoulosSakil KulkarniJanis StollMaria Bernadette Majella DoyleAdeel KhanMai HePublished in: Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society (2023)
Sclerosing epithelioid fibrosarcoma (SEF) is a rare but aggressive sarcoma. We report the first case of hepatic SEF in pediatric patient, which is also the second case in literature. A 17-year-old previously healthy female presented with a liver mass measuring 13.7 cm in greatest dimension and mild elevation of liver enzymes and cancer antigen 19-9. Needle biopsy revealed multiple cores of liver parenchyma mostly replaced by densely hyalinized fibrotic tissue and areas of small-to-medium sized epithelioid cells with eosinophilic and clear cytoplasm. Immunohistochemistry (IHC) demonstrated diffuse strong cytoplasmic staining of MUC4, suggesting a working diagnosis of sclerosing epithelioid fibrosarcoma (SEF)/low-grade fibromyxoid sarcoma (LGFMS). Liver explant demonstrated a well-circumscribed, nodular mass with firm, gray-white cut surface, and similar histopathology as seen in needle biopsy with no convincing evidence suggesting LGFMS. Sequencing panel revealed EWSR1::CREB3L1 gene fusion and confirmed the diagnosis of SEF. Post-operative cancer antigen 19-9 normalized 3 months after transplant; follow-up 3 and 6 months post-transplant imaging at that time showed no concern for disease recurrence.
Keyphrases
- low grade
- ultrasound guided
- high grade
- single cell
- systematic review
- induced apoptosis
- high resolution
- idiopathic pulmonary fibrosis
- cell death
- cell cycle arrest
- squamous cell carcinoma
- systemic sclerosis
- endoplasmic reticulum stress
- photodynamic therapy
- childhood cancer
- fluorescence imaging
- genome wide identification