P3976 - Unmasking an Obstructive Esophageal Mass: A Rare Case of Esophageal Epithelioid Angiosarcoma Diagnosed With EUS-Guided Biopsy

Harneet Sangha, BS¹, Tavishi Girotra, MBBS², Shreya Chopra, MBBS², Navansh Johnson, ², Anusha Singhania, MBBS², Mohit Girotra, MD^1
1Washington State University Elson S. Floyd College of Medicine, Spokane, WA; ²Digestive Health Institute, Swedish Medical Center, Seattle, WA

Introduction: Gastro-intestinal (GI) angiosarcomas are extremely rare soft tissue tumors arising from endothelial cells of blood and lymphatic vessels and constitute < 1% of all sarcomas. They most commonly originate in the liver and spleen, with occurrences in the small and large intestine being even more uncommon. Epithelioid angiosarcoma (EA) is a morphological variant of angiosarcoma and the etiology remains largely unknown.



We present a case of Esophageal EA in an older man diagnosed via Endoscopic ultrasound (EUS).

Case Description/Methods: A 94-year-old man with hypertension, basal cell carcinoma, previous hip replacement, and subdural hematoma now presented with progressive dysphagia (solids > liquids, 4 months duration and progressive), weight loss of 20 lbs., and shortness of breath. Computed tomography (CT) revealed a large para-esophageal mass/lymph node; however, no clear esophageal narrowing was observed.


EUS identified a single small 8 mm lymph node in the peri-gastric region, approximately 41 cm from the incisors. Additionally, a large 26x16 mm round heterogeneously hypoechoic mass/lymph node was noted in the para-esophageal region, at 36-38 cm from the incisors, causing extrinsic compression on esophagus lumen. This lesion could not be differentiated from serosal surface of the esophageal wall, and FNB was obtained with 22G core needle.

Pathology confirmed a malignant neoplasm composed of epithelioid-spindled cells with numerous mitoses, and Immunohistochemical staining showed positive vascular markers (ERG & CD31), focally positive CD34 & CD117, and cytoplasmically positive for WT-1. These findings were diagnostic of epithelioid angiosarcoma (EA).

After multidisciplinary consultation with surgical oncology, medical oncology, and palliative care teams, given lack of quality treatment options at his age and comorbidities, the patient elected for hospice.

Discussion: Esophageal EA is a rare condition, and there are three previous reported cases, mostly in thoracic surgery literature.



Our case would be the first diagnosed with EUS-guided biopsy, highlighting the rarity of the condition and utility of EUS in diagnosis.



Mostly EA present with GI bleeding, but esophageal EA may present with dysphagia. Diagnosing EA is challenging, but imaging, histopathology, and markers like ERG, CD31, CD34 prompt management decisions which include combination of resection and adjuvant chemotherapy/radiotherapy; and unresectable cases may receive targeted immunotherapy.



Disclosures:


Harneet Sangha, BS¹, Tavishi Girotra, MBBS², Shreya Chopra, MBBS², Navansh Johnson, ², Anusha Singhania, MBBS², Mohit Girotra, MD¹. P3976 - Unmasking an Obstructive Esophageal Mass: A Rare Case of Esophageal Epithelioid Angiosarcoma Diagnosed With EUS-Guided Biopsy, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.