Diffuse Pagetoid Squamous Cell Carcinoma in Situ of the Esophagus: A Rare Case Report and Review of Literature

Squamous cell carcinoma in situ (SCCIS) with diffuse pagetoid features has been well-described in skin and external genitalia. Diffuse pagetoid SCCIS of the esophagus is extremely rare with only two cases published in the English literature. In this article, we report a rare case of diffuse pagetoid SCCIS of the esophagus in an 89-year-old female with no significant past medical history who presented with dysphagia. Endoscopic examination of the esophagus was remarkable for multiple clean base ulcers spanning 4 cm in the proximal esophagus. Biopsy showed enlarged and hyperchromatic dysplastic cells in the basal half of the epithelium with scattered large individual pagetoid cells as well as several apoptotic dyskeratotic cells in the superficial half of the epithelium. Immunohistochemically, the dysplastic cells were positive for CK7 and p40 with overexpression of p53, and were negative for cytokeratin 20, SOX10, GATA3, CDX2, TTF1. Kreyberg stain was negative for mucin. The histologic features and immunohistochemical profile supported the diagnosis of esophageal diffuse pagetoid SCCIS.