Who's the Father?: The Duality of Esophageal Spindle-Cell Squamous Carcinoma

Introduction: Spindle-cell squamous carcinoma (SpCC) is an extremely rare variant of SCC previously described in literature with a series of confounding names as a result of its intriguing yet confusing origin. Furthermore, the rarity of SpCC along its dual cellular component has led to diagnostic challenges of this highly aggressive entity. Herein a curious case of an African American male with atypical chest pain diagnosed with spindle-cell squamous carcinoma of the esophagus. Case Description/Methods: A 53-year-old male with history of tobacco and alcohol abuse presented to ER with atypical chest pain and questioning revealed epigastric pain, worsening heartburn, weight lost, and difficulty swallowing since 3 months ago. EKG without evidence of acute ischemia. Physical exam and labs unremarkable. CT chest with evidence of mid-to-distal esophageal distention with associated distal nodular lesion of 1.5 cm in size. EGD revealed distal esophageal bulky polypoid lesion. Biopsy suggested spindle cell malignancy, yet inconclusive due to scant amount of tissue. Second EGD with evidence of friable lesion obstructing 75% of esophageal lumen. Biopsy revealed expression of P40, P63, Ck5/6 and morphology suggestive of SpCC. Patient referred to Oncology service and chemotherapy-radiotherapy started with remission of disease. A year later patient presented with severe dysphagia. Initial EGD negative for strictures, tumor, or fistula. Bronchoscopy negative for fistulization. Due to worsening dysphagia, patient re-admitted and CT chest concerning for fistulous tract. New EGD demonstrated tracheoesophageal fistula at 31 cm and AGILE stent placed with improvement of dysphagia. Discussion: SpCC is a rare subtype of SCC but with similar risk factors such as tobacco use and alcohol consumption. The spindle cell component simulating a sarcoma and a non-specific clinical presentation precludes a correct diagnosis. Our patient initially presented with atypical chest pain, and review of systems revealed symptoms of progressive dysphagia and weight loss which guided the diagnosis. A second biopsy performed, and several immunohistochemistry tests confirmed the presence of both a carcinomatous and sarcomatous component. As a result, our case is an example of one of the rarest malignancies affecting the esophagus and its complications. It also highlights the importance of a thorough history taking, adequate tissue sampling, use of staining techniques to reach a correct diagnosis, as well as incorporation of innovative medical devices.Figure 1.: Spindle-cell squamous carcinoma lesion with tracheoesophageal fistula complication and subsequent Agile Stent placement.