Intradural prepontine growth: Giant ecchordosis physaliphora or extraosseous chordoma?

Abstract

with neuropathological features common to both chordoma and ecchordosis physaliphora. 1999; 19, 336-340 An unusual case of a 54-year-old woman who presented with a one-and-a-half year history of dizziness and in whom neuroimaging studies revealed an intradural prepontine growth without radiological evidence of bone involvement, is reported. A marked histological resemblance to both ecchordosis physaliphora and chordoma was observed. The medical literature concerning similar lesions is reviewed. Neuropathology

Sudden death due to subarachnoid bleeding from ecchordosis physaliphora

“…The histology of EP is very similar to that of intradural chordoma, its malignant counterpart [5,7,12] and both present physaliphorous cells arranged in a myxoid matrix. Immunohistochemistry is useful to support the diagnosis [9].…”

“…Immunohistochemistry is useful to support the diagnosis [9]. In particular, the low proliferative activity supports the diagnosis of EP [2,7,12].…”

“…The frequency of this finding varies between 0.5 and 2% of autopsies [8]. EP is usually asymptomatic and only few authors have reported EP-associated symptoms due to tumour expansion and compression of the surrounding structures [1,12,13]. Fatal subarachnoid bleeding due to rupture of an EP has been described only once [14].…”

Benign Notochordal Lesions of the Posterior Clivus: Retrospective Review of Prevalence and Imaging Characteristics

“…This challenge has led some researchers to propose various terms to encompass symptomatic intradural extraosseous physaliphorous lesions such as intradural benign chordoma, BNCT, or giant symptomatic EP. 10,12,16,23 Cases 4 and 5 likely fall within this sphere. It may be helpful, then, to consider such atypical cases as lesions within an intermediate category or along a benign spectrum of retroclival notochordal lesions.…”

Unexpected sudden death due to intracranial chordoma: An autopsy case

“…The histology of chordoma is very similar to that of ecchordosis physaliphora [17][18][19]; both consist of vacuolated cells in a myxoid matrix [20]. Chordoma is characterised by neoplastic cell arrangements in cords and lobules within the myxoid matrix and higher EMA and S-100 positivities of about 50-95% [25,26].…”