S4454 A Rare Case of a Near-Occluding Esophageal Leiomyosarcoma

Introduction: Esophageal leiomyosarcoma is a rare, slow-growing malignant neoplasm with smooth muscle origin and late metastasis. The main symptoms in patients with this type of tumor are not different from other esophageal masses. The diagnosis of leiomyosarcoma is usually on a histopathologic basis and on rare cases by endoscopic ultrasound-guided fine-needle aspiration biopsy. Esophageal leiomyosarcoma is the most common of all esophageal sarcomas. However, its incidence rate is low - less than 0.2%. Case Description/Methods: This is a case of a 75-year-old woman who presented with progressive dysphagia, postprandial nausea and vomiting, constipation, and a gradual decline in oral intake of both solids and liquids, epigastric pressure-like discomfort, anorexia, and weight loss. Her past medical history included spastic cerebral palsy, hypertension, gastroesophageal reflux disease, and chronic hypokalemia. Computed tomography scan showed a significantly dilated esophagus measuring about 5.5 cm with a large volume of hypoattenuating material displacing the oral contrast with raised suspicion for achalasia or an esophageal dysmotility disorder. Chest imaging also revealed prominent paraesophageal lymphadenopathy and multiple bilateral lung novules measuring up to 7 mm. Given these findings, an upper endoscopic evaluation was performed which revealed a large fungating necrotic mass in the esophagus located 20 cm from the incisors. Pathology showed a poorly differentiated malignant neoplasm consistent with esophageal leiomyosarcoma positive for smooth muscle actin (SMA), caldesmon, and calponin (focal/weak), and negative for desmin, S100, CD117, DOG-1, CK5/6, P63 and CK AE1/AE3. Discussion: Esophageal leiomyosarcoma is an extremely rare tumors, with approximately 200 total cases reported worldwide which is most commonly found in the lower third of the esophagus. Diagnostic measures include barium swallow, computed tomography scan and endoscopy. The mainstay for treatment of esophageal leiomyosarcoma is surgical resection. Although esophageal leiomyosarcoma exhibits poor sensitivity to radiation, radiotherapy may also be used adjunctively or in inoperable or extensive leiomyosarcomas to achieve tumor control. Esophageal leiomyosarcoma exhibits a better prognosis than other esophageal cancer subtypes due to its slow growing nature and late metastases. This patient, in light of an advanced disease elected for palliative management and supportive care. In such a case, early diagnosis may have prompted a more aggressive treatment approach with higher prognostic probability.