Pseudoendocrine sarcoma: a rare new entity with unique radiologic and pathologic/molecular characteristics.

Abstract

Pseudoendocrine sarcoma is a rare, recently described intermediate grade sarcoma of uncertain phenotypethat most commonly affects theparaspinal location in older patients with a distinctive endocrine/paraganglioma-like morphology and unique CTNNB1 point mutation. While these tumors appear as epithelial or even benign endocrine tumors, these lack markers for such and are highlighted by nuclear expression of beta-catenin. This case is the first among the previously reported only twenty-five cases of this entity,including one original series and a few case reports, to correlate the radiologic imaging with the pathologic features. Furthermore, this case illustrates the oldest-to-date patient with this unique location as a palpable painful chest wall/paraspinal location, with new morphologic observations and, finally, this isonly the second case to havethisspecific CTNNB1 hotspot point mutation for this rare entity.