Cytokeratin-positive interstitial reticulum cell tumor: recognition of a potential "in situ" pattern

Cytokeratin-positive interstitial reticulum cell (CIRC) tumor is a very rare accessory cell neoplasm of lymphoid organs derived from fibroblastic reticulum cells, which originate from mesenchymal stem cells. We describe the histologic, immunophenotypical, and molecular features of a CIRC tumor in a 67-year-old woman who underwent hysterectomy, bilateral adnexectomy, and pelvic lymphadenectomy for endometrial carcinoma. An enlarged pelvic node contained circumscribed neoplastic infiltrates in perifollicular and interfollicular areas consisting of large cells arranged in a reticular pattern with nuclear atypia, atypical mitoses, and apoptosis, but without glandular architecture or disruption of overall architecture. The atypical infiltrate coexpressed cytokeratin and vimentin, partially CD68, CD163, and lysozyme, but lacked markers of endometrial carcinoma, consistent with a diagnosis of CIRC tumor. Despite the obviously neoplastic cytological features, immunostains revealed the circumscribed and noninvasive pattern of the lesion, possibly representing an early "in situ" stage of CIRC tumor.