Icahn School of Medicine at Mount Sinai (Elmhurst Hospital Center) Elmhurst, NY, United States
Award: Presidential Poster Award
Jonathan Vincent M. Reyes, MD, MHA1, Tasur Seen, MD1, Bhanu Singh, MD1, Vennis Lourdusamy, MD1, Ahmed Al-Khazraji, MD2, Moiz Ahmed, MD1, Aaron Walfish, MD1 1Icahn School of Medicine at Mount Sinai (Elmhurst Hospital Center), Elmhurst, NY; 2Elmhurst Hospital Center, Elmhurst, NY
Introduction: Sarcomatoid tumors are rare biphasic tumors characterized by a mixture of malignant epithelial and mesenchymal cells that are usually identified in the lungs. In some instances, the tumors can also be found in the skin, bone, breast, GU tract, GI tract (GIT), thyroid gland, and salivary glands. When found in the small intestine, they have an incidence estimated to be 0.5 - 0.8 per 100,000 per year.
Case Description/Methods: A 61-year-old male presented with complaints of non-specific abdominal pain and unintentional weight loss of 30 lbs. Labs revealed normocytic anemia with a hemoglobin of 9.5. CT of the abdomen and pelvis demonstrated a 7.8 x 7.0 x 7.1 cm irregular, a heterogeneous mass contiguous with the duodenum, gastric antrum, and pancreas. Esophagogastroduodenoscopy revealed an edematous pylorus with a medium-sized fungating, polypoid, and ulcerated mass in the anterior wall of the duodenal bulb. Specimens were taken and underwent immunohistochemical staining. Findings were positive for s100 and negative for MCK, CD117, CD34, and SMA; The spindle cells were focally positive for EMA and negative for AE1/AE3, CAM5.2, and sox10. The final pathologic diagnosis was sarcomatoid carcinoma with osteoclast-like giant cells.
Discussion: 3 unique features of sarcomatoid tumors include the presence of a genuine sarcomatous component, no transitional zone between carcinomatous and sarcomatous components, and immunohistochemistry of the sarcomatous component that is positive for mesenchymal markers and negative for epithelial markers. Sarcomatoid carcinoma of the GIT is rare, typically occurring in the stomach, gallbladder, or esophagus. It affects males more than females. The average age of diagnosis is 57 years old. Risk factors have not yet been established. The tumor tends to have an aggressive clinical course with an overall 5-year survival rate of about 20%. Immunohistochemistry is necessary for diagnosis as small intestine tumors have only minor histological differences. Immunohistochemical studies of the tumor show pleomorphic cells positive for cytokeratin AE1/AE3, CAM 5.2, and vimentin and negative for desmin, muscle-specific actin, CD34, DOG1, c-kit, and S-100. The only treatment is surgery with wide excision. Monoclonal antibodies against programmed death protein (PD-1) and its ligand, PD-L1, have promising antitumor activity in several other malignancies and may represent possible future non-surgical treatment options for this tumor.
Disclosures: Jonathan Vincent Reyes indicated no relevant financial relationships. Tasur Seen indicated no relevant financial relationships. Bhanu Singh indicated no relevant financial relationships. Vennis Lourdusamy indicated no relevant financial relationships. Ahmed Al-Khazraji indicated no relevant financial relationships. Moiz Ahmed indicated no relevant financial relationships. Aaron Walfish indicated no relevant financial relationships.
Jonathan Vincent M. Reyes, MD, MHA1, Tasur Seen, MD1, Bhanu Singh, MD1, Vennis Lourdusamy, MD1, Ahmed Al-Khazraji, MD2, Moiz Ahmed, MD1, Aaron Walfish, MD1. P3031 - A Rare Presentation of Sarcomatoid Carcinoma of Duodenum, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.