Neoplasms of the Appendix: Pictorial Review with Clinical and Pathologic Correlation

Appendiceal neoplasms are uncommon tumors of the gastrointestinal tract that may manifest with symptoms of appendicitis, right lower quadrant pain, or palpable mass, leading to imaging or surgical intervention. The majority of appendiceal masses consist of primary epithelial neoplasms and neuroendocrine tumors (NETs). Epithelial neoplasms—mucinous and nonmucinous types—are more often detected at imaging than NETs due to their larger size and propensity for peritoneal spread and metastatic disease. Epithelial mucinous neoplasms are defined by the presence of mucin, detected at radiologic and pathologic examination. A mucocele or pseudomyxoma peritonei from epithelial mucinous tumors are the two most common cross-sectional imaging findings of appendiceal mucinous neoplasms. Nonmucinous epithelial tumors are less common and manifest as masses similar to colonic-type malignancies. NETs are often discovered incidentally at appendectomy due to their small size and nonaggressive behavior. Imaging findings of primary appendiceal tumors may overlap with those of acute appendicitis. Additionally, an appendiceal mass may cause acute appendicitis, obscuring the underlying mass. Other neoplasms including lymphoma, sarcoma, mesenchymal and nerve sheath tumors, or secondary malignant involvement of the appendix are rare. Treatment depends on the histologic subtype and extent of disease. Detailed description of organ, nodal, and peritoneal involvement informs surgical management with the goal of complete cytoreduction. Novel treatments such as hyperthermic intraperitoneal chemotherapy have increased survival for patients with mucinous tumors. ©RSNA, 2017