A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery

Abstract

Background:Ecchordosis physaliphora (EP) is a benign notochordal remnant that is usually asymptomatic; symptomatic cases are extremely rare. Most of the reported symptomatic cases were managed by resection via craniotomy.Case Description:We report a case of a 20-year-old male presenting with abducens nerve palsy. Magnetic resonance imaging performed on admission demonstrated a mass in the retroclival prepontine location. The patient was treated successfully by endoscopic endonasal trans-sphenoidal surgery (ETSS), his postoperative course was uneventful, and the abducens nerve palsy disappeared.Conclusion:ETSS has advantages not only for treatment but also for differentiation between EP and intradural chordoma. This is the first case of symptomatic EP successfully treated solely by ETSS.

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

“…Cells immersed in a myxoid and amphiphilic matrix and numerous, large intracytoplasmic mucin-containing vacuoles AE1 and AE3 clones, EMA (+), S-100 (-) Endoscopic endonasal surgery Kaul, et al, J Neurol Surg, 2013 [ 3 ] 52, F SBM, transclival pseudomeningocele Well-defined and corticated bony defect in the dorsal wall of the clivus, measuring approximately 6 mm, and a soft tissue mass in the sphenoid sinus Hypointense Hyperintense No NR NR Endoscopic endonasal approach Bolzoni-Villaret, et al Laryngoscope, 2014 [28] 51, F Recurrent CSF leakages Fluid collection in the right sphenoid sinus, with evident remodeling of the posterior wall. Clival bony defect with a lobulated, non-enhancing mass 12 x 6 mm NR Hyperintense NR NR NR Transphenoidal-transclival endoscopic approach (TTEA) Krisht, et al, J Neurosurg Pediatr, 2013 [ 18 ] 16, F Diplopia 3.0 x 1.7 x 1.8-cm (“giant ecchordosis physaliphora”), extra-axial epidural mass along the dorsal aspect of the clivus Intermediate Hyperintense No (unable to access full text) (unable to access full text) Transnasal transsphenoidal approach Yamamoto, et al, Surg Neurol Int, 2013 [ 17 ] 20, M Sudden onset diplopia (cranial nerve VI palsy) Lesion measuring 22 mm in diameter Hyperintense Hyperintense No Hypocellular physaliphorous cells with a lobular growth pattern and eosinophilic cytoplasm with vacuolated mucus droplets. Neither mitosis nor dyskaryosis was visible Cytokeratins (+), MIB-1 “not increased” Endoscopic endonasal transsphenoidal surgery (ETSS) Adamek, et al, Neurol Neurochir Pol, 2011 [ ...…”

“…Immunohistochemical analysis also showed characteristic features, which are discussed later. Two other cases are notable in that they demonstrated intermediate enhancement on T1 [ 2 , 17 ]. Krisht, et al and Ng, et al both presented cases of extradural EP with intermediate intensity on T1WI.…”

“…Other features of EP include the location and the associated bony stalk or pedicle. As far as location, EP is classically intradural and near the midline with respect to the craniospinal axis; however, it can be located in any combination of the extradural, subdural, and subarachnoid spaces [ 7 , 17 , 22 ]. EP in the subarachnoid or subdural space is typically connected by a thin pedicle or bony stalk, which is often described, but not often visualized, as an osseous stalk-like projection connecting the lesion (EP) to the dorsal aspect of the clivus; it is considered the pathological hallmark of EP by some authors [ 12 ].…”

Multiple Ecchordosis Physaliphora

“…To the best of our knowledge, only 4 cases of giant symptomatic EP, located in the retroclival prepontine region, have been reported. [ 2 3 4 5 ] Of the 4 giant reported cases and our present patient with symptomatic EP found in the retroclival prepontine location, with a size from 20 mm to 40 mm. Three were male and one was female with a mean age of 28 years (range: 12–63 years).…”

“…[ 1 ] Giant symptomatic EPs are extremely rare, and only four cases have been reported in the literature. [ 2 3 4 5 ] No cases of multiple EPs or EPs with metastasis have been reported.…”