Polypoid leiomyosarcoma of the esophagus treated by endoscopic submucosal dissection

Abstract

We report a rare case of polypoid leiomyosarcoma of the esophagus that was treated by endoscopic submucosal dissection (ESD). A 63-year-old man with complaints of progressive dysphagia was referred to Hyogo Cancer Center for treatment of esophageal tumor. Esophagoscopy revealed a polypoid tumor 25 mm in diameter on the left side of the upper esophagus. Despite several biopsy specimens, the diagnosis could not be confirmed. Computed tomography showed a protruded, homogeneously enhancing mass in the upper esophagus, but no lymph node enlargement or metastasis. After 1.5 months, the esophagogram showed a filling defect 47 mm in diameter in the upper esophagus. Given this rapid tumor growth, en bloc resection was done by ESD for therapeutic diagnosis. After this treatment, the tumor seemed to grow larger, showing a short stalk and occupying the esophageal lumen. Histopathologically, the tumor comprised pleomorphic spindle cells with mitosis. Tumor invasion involved the lumina propria mucosae and contact with the muscularis mucosae, but not involving the submucosa. Immunohistochemical examination showed positive staining for smooth muscle actin and HHF35, but negative for desmin, caldesmon, CD34, c-kit, DOG1, ALK, S-100 protein and cytokeratin. These histopathological findings were compatible with a diagnosis of esophageal leiomyosarcoma derived from the muscularis mucosae.

Esophageal intramural squamous cell carcinomas presenting as subepithelial lesions removed by endoscopic submucosal dissection

“…Tatsushi et al [ 17 ] first reported an intraluminal polypoid form of esophageal leiomyosarcoma treated with endoscopic resection with an electric snare in 2008. Yamamoto et al [ 18 ] performed en bloc resection by ESD for the therapeutic diagnosis of leiomyosarcoma in cases where several biopsy specimens could not confirm the pathology results and the tumor had rapidly grown. Additionally, esophageal GISTs can present as SELs and some of them have the potential to become malignant.…”

Histopathological landscape of rare oesophageal neoplasms

“…The epithelioid variant is described to feature proliferation of round or polygonal cells with eosinophilic or clear cytoplasm arranged in nests, sheets or cords with moderate to severe cytologic atypia, tumour cell necrosis or more than 3 mitoses per 10 high-power fields[ 12 ]. On immunohistochemistry, leiomyosarcomas are positive for SMA (95%), muscle-specific actin (91%), calponin (88%), desmin (73%), caldesmon (66%) and myosin (64%) and negative for CD34, CD117, DOG1 and S100[ 137 , 140 ].…”

“…Treatment: ESD and radical surgical resection, eventually followed by adjuvant radiotherapy, are the first-line therapeutic approaches for OLMS[ 138 - 140 ]. OLMSs have a better prognosis than other oesophageal malignancies: In the Chinese medical literature, the 3-, 5- and 10-year survival rates are reported to be 80.0%, 58.3% and 31.1%, respectively[ 138 ].…”