Transnasal endoscopic resection of symptomatic ecchordosis physaliphora

Abstract

Laryngoscope, 124:1325–1328, 2014

Proposed Diagnostic Criteria, Classification Schema, and Review of Literature of Notochord-Derived Ecchordosis Physaliphora

“…Immunohistochemical methods have aided in demonstrating the aforementioned common notochordal origin of both EP and chordoma, allowing differentiation from other masses, such as neuroenteric cysts, but have provided little in the way of distinguishing EP from chordoma. Despite similar staining characteristics (cytokeratin, EMA, S-100, galectin, and brachyury positivity and GFAP negativity), the literature does describe subtle features, which favor the diagnosis of EP (e.g., a lower Ki-67 index, a very low MIB count, and absence of mitosis) [ 25 - 26 ]. Furthermore, electron microscopy of EP features unique rough endoplasmic reticulum (RER) mitochondrial complexes, intracytoplasmic invaginations, numerous intracytoplasmic vacuoles, and plasma membranes with pinocytotic vesicles and cell junctions.…”

Ecchordosis physaliphora presenting with abducens nerve palsy

“…1 with endonasal trans-sphenoidal surgery; the fourth was treated with recession-resection of the horizontal rectus muscles. 2,4,6,8 The patient who had strabismus surgery was stable for 5 years.…”

Resección endoscópica de cordoma del clivus. Descripción de un caso

“…Most frequent sites include lung, bone, liver and lymph nodes [3]. Differential diagnoses include ecchordosis physaliphora and benign notochordal cell tumor (BNCT), both benign lesions derived from remnant cells of the primitive notochord and primarily involving the clival region [6,7]. BNCT may also undergo malignant transformation to classic chordoma.…”

“…However, contrast enhancement is not pathognomonic of chordoma, and the differential diagnosis with these two benign entities can't be definitive only with image studies. As so, suspected clival lesions must be biopsied for a definitive diagnosis [6,7]. The recommended treatment for clivus chordomas is complete surgical resection and postoperative RT [4,9].…”