P0757 - Undifferentiated Embryonal Sarcoma of the Liver in a Pregnant Adult

Sameeha Khalid, DO, Timothy Wang, MD, Marina Roytman, MD
University of California San Francisco Fresno, Fresno, CA

Introduction: Undifferentiated embryonal sarcoma of the liver is an aggressive tumor, typically occurring in children. Cases of embryonal sarcoma in adults are exceedingly rare. Correct diagnosis is often delayed and is only confirmed by histologic examination. We present this case of a pregnant woman found to have a large embryonal sarcoma.

Case Description/Methods: A 25-year-old female, 6 weeks gestation, presented with acute abdominal pain radiating to the right shoulder. Physical examination revealed right upper quadrant tenderness to palpation and no signs of advanced liver disease. Laboratory tests revealed elevated ALT 81 IU/L. Initial ultrasound of the abdomen demonstrated a 13 cm liver mass. After the pregnancy was determined to be non-viable, a triple-phase CT of the liver suggested the liver mass was most compatible with a cavernous hemangioma. Tumor markers were negative. Tumor board recommended repeat imaging in 3 months. The patient re-presented to the hospital 1 month later with ongoing abdominal pain. Labs were notable for alkaline phosphatase 195 IU/L, ALT 154 IU/L, AST 136 IU/L, total bilirubin 2.2 mg/dL. MRI showed interval growth of the liver mass to 18 cm. Given rapid growth, biopsy of the liver mass was performed revealing undifferentiated embryonal sarcoma. Hospital course was complicated by bilateral pulmonary emboli requiring heparin drip and subsequently primary tumor rupture requiring IR embolization. The patient was initiated on neoadjuvant chemotherapy with doxorubicin/ifosfamide/mesna per a clinical trial with plans for possible future resection.

Discussion: Although embryonal sarcoma is more frequent in pediatric populations, it can rarely present in adults as well. Our patient’s initial CT showed features consistent with hepatic hemangioma including peripheral nodular arterial phase hyper-enhancement and progressive centripetal filling on portal venous phase. However, it was unusual for the patient to present with acute pain without a significant amount of tumor hemorrhage to explain this. It was further unexpected to see rapid interval growth of the mass within a month. This case highlights the importance of a high degree of clinical suspicion for an atypical presentation of a presumed benign liver mass as well as the need for a multi-disciplinary team of hepatologists, radiologists, pathologists, and surgeons to diagnose and treat this rare malignancy.



Disclosures:


Sameeha Khalid, DO, Timothy Wang, MD, Marina Roytman, MD. P0757 - Undifferentiated Embryonal Sarcoma of the Liver in a Pregnant Adult, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.