Esophageal carcinoma cuniculatum associated with non-necrotizing granulomatous inflammation and lymphadenopathy: Clinicopathologic features and diagnostic challenges

Objective: Rare disease Background: Esophageal carcinoma cuniculatum (CC) is an exceptionally rare, well-differentiated squamous cell carcinoma (SCC) with initial microscopic evaluation often yielding inconclusive diagnoses due to its characteristically bland histomorphologic appearance on superficial endoscopic biopsy. This can lead to delayed diagnosis and pose challenges in further management of these cases. Case Report: We present the case of a 52-year-old man with symptoms of dysphagia and odynophagia. The initial chest CT scan showed gastroesophageal (GE) junction wall thickening and regional lymphadenopathy. Esophagogastroduodenoscopy (EGD) revealed an esophageal mass, but the mucosal biopsies were inconclusive. Repeat endoscopic biopsies also failed to yield a definitive diagnosis. Under strong clinical suspicion for malignancy, an esophagogastrectomy was performed, which yielded the diagnosis of CC, and the associated enlarged lymph nodes revealed non-necrotizing granulomatous lymphadenitis. Conclusions: Only 15 cases of esophageal CC have been described in the literature. This particular case is unique due to the associated abundant lymphoplasmacytic and granulomatous inflammation and involvement of regional lymph nodes by non-necrotizing granulomas not previously described.