Introduction: The majority of esophageal cancers are classified histologically as squamous cell carcinomas or adenocarcinomas. We present a case of a 69-year-old man, presented with progressive dysphagia, found to have an esophageal carcinosarcoma, a rare etiology of esophageal cancer.
Case Description/Methods: A 69-year-old man, an every day smoker with a history of ongoing alcohol use, presented for the evaluation of unintentional weight loss and progressive dysphagia of 2 months duration. Upper endoscopy revealed a large, 6 cm in diameter, partially obstructing ulcerating mass in the mid esophagus. PET/CT revealed intense FDG uptake in the esophageal mass, mediastinal lymph nodes, T6 and T10 ribs suggestive of metastatic disease. Surgical pathology of the tumor was consistent with a high grade squamous cell carcinoma mixed with a high grade spindle cell sarcoma (carcinosarcoma). The patient was started on chemoradiation including Paclitaxel, Carboplatin. Improvement of symptoms and CT findings was documented with treatment.
Discussion: Esophageal cancer ranks seventh in incidence and sixth in cancer-related mortality. The majority of esophageal cancers are classified histologically as squamous cell carcinomas or adenocarcinomas. When two distinct malignant components, both carcinomatous and sarcomatous are present histologically, the tumor is classified as esophageal carcinosarcoma (ECS). The pathogenesis is debatable, with some arguing that it is an initial carcinoma, facilitating a simultaneous secondary development of sarcoma, while others hypothesize a single cell origin. Carcinosarcomas were documented in tumors of the uterus, breast, thyroid, lung and gastrointestinal tract. ECSs have a rare incidence, accounting for approximately 1.5% of all esophageal tumors. It has been noted that ECS tumors have different clinical behaviors when compared to squamous cell carcinomas or adenocarcinomas. ECS tumors were noted to have a calculated doubling time of 2.2 months, with predominantly exogenous polypoid growth, perhaps explaining the earlier symptom of dysphagia, earlier diagnosis and more favorable 5 year survival rate. Given their rarity, no standard protocol has been developed to treat ECS tumors. Resection, regional lymph node dissection, radiotherapy and chemotherapy have all been utilized.
Our case presentation further enriches the literature regarding rare, advanced esophageal adenocarcinoma tumors responding to systemic therapy.
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