High-grade malignant peripheral nerve sheath tumor of the esophagus: a rare case highlighting the diagnostic value of immunohistochemistry. (PubMed, J Surg Case Rep)
This case report presents a highly unusual case of a primary high-grade malignant peripheral nerve sheath tumor (MPNST) of esophagus, a neoplasm of extreme rarity, with fewer than twenty histologically confirmed cases reported worldwide to date; detailing the successful management of this tumor through Orringer's transhiatal esophagectomy, complemented by comprehensive histopathologic and immunohistochemical evaluation. Our report emphasizes the crucial the role of immunohistochemistry, specifically the diagnostic value of S100, SOX10, and the exclusion of gastrointestinal stromal tumors markers (DOG1, CD117) in distinguishing MPNST, from morphologically similar esophageal submucosal tumors.