A Case of Giant Ecchordosis Physaliphora

Ling, Shane S.; Sader, Chady; Robbins, Peter A.; Rajan, Gunesh

doi:10.1097/mao.0b013e318068b2c8

Cited by 34 publications

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“… NR NR NR Cell-poor lobules with large eosinophilic cytoplasm and intracytoplasmic mucus droplets (i.e., physaliphorous cells) KLI, EMA (+), Vimentin and S-100 (+), MIB-1 (-) N/A Miki, et al, Minim Invas Neurosurg, 2008 [ 8 ] 59, M Dizziness, gait disturbance, dementia Triventricular hydrocephalus. Small tumor with a component resembling bone on the dorsal side of the medial clivus NR NR NR Meshwork of physaliphorous cells containing numerous vacuoles NR ETV + tumor resection Ling, et al, Otol Neurotol, 2007 [ 21 ] 45, M Sudden left-sided hearing loss w/ non-pulsatile tinnitus 30 x 30 x 14 mm irregularly shaped pre-pontine lesion. Mass effect on the pons, w/ remodeling of dorsal wall of clivus and invagination into (R) sphenoid sinus Hypointense Hyperintense No Irregular clusters, cords, and interlacing strands of cells dispersed within an abundant myxoid matrix.…”

Section: Reviewmentioning

confidence: 99%

“…It was not until 2007 when Ling, et al published the first case report of a giant EP, where the word “giant” explicitly appears in both title and text. That particular mass measured 30 x 30 x 14 mm [ 21 ]. The most recent case of a giant EP in which the EP measured 2.1 cm x 1.8 cm x 1.7 cm (6.426 cm 3 ) was described by Choudhri, et al Therefore, the literature suggests that anything greater than 3 cm in diameter or 6 cm 3 in volume is consistent with a so-called “giant” EP, and we have included this in the radiologic features under our proposed diagnostic criteria [ 1 ].…”

Section: Reviewmentioning

confidence: 99%

“…Imaging alone has the potential to guide management of these clival lesions. Asymptomatic EP is managed by serial imaging, often remaining stable over years, whereas chordomas display progression in as little as one year by way of either mass effect or metastasis [ 21 ].…”

Section: Reviewmentioning

confidence: 99%

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

Lagman

Varshneya

Sarmiento

et al. 2016

Cureus

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Ecchordosis physaliphora (EP) is a benign notochordal remnant derived from ectopic nests found along the craniospinal axis. It typically presents asymptomatically and is diagnosed using classic radiologic features, particularly location, T1-hypointensity, T2-hyperintensity, and lack of enhancement following gadolinium (Gd) contrast administration. Distinguishing EP from its malignant counterpart, chordoma, is of paramount importance, given the aggressive nature of the latter. Advances in imaging and immunohistochemistry have aided in diagnosis to an extent but, to our knowledge, identification of the genetic fingerprint of EP has yet to take place. Further cytological analysis of these lesions in search of a genetic link is warranted. We propose here a set of diagnostic criteria based on features consistently cited in the literature. In this literature review, 23 case reports were identified and collated into a summary of symptomatic cases of ecchordosis physaliphora. An illustrative case report of two patients was also included.

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Section: Reviewmentioning

confidence: 99%

Section: Reviewmentioning

confidence: 99%

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

Lagman

Varshneya

Sarmiento

et al. 2016

Cureus

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“…13 46 Mass effect has frequently been associated with expansile lesions such as chordomas, 2 but this can also occur in benign notochord tumors, although more rarely. 6 21 28 Similarly, lesion size has limited use as giant EP, that is, more than 2 cm exists 9 21 22 23 as do small chordomas. 9 12 Differentiating EP from chordoma on imaging approaches the impossible.…”

Section: Discussionmentioning

confidence: 99%

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

Sooltangos

Bódi

Ghimire

et al. 2021

J Neurol Surg B Skull Base

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Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

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“…Typically, EP are small, well-circumscribed, extra-axial, intradural lesions in the prepontine cistern, which have a predilection for the midline posterior clivus at the level of Dorello’s canal [ 85 , 87 , 88 ]. On MRI, EP show high signal intensity on T2-weighted images and low signal intensity on T1-weighted images, similar to chordoma, but do not show contrast enhancement [ 85 , 86 , 88 – 92 ]. Differentiating EP from intradural chordoma is essential, as EP are benign and usually asymptomatic and therefore do not need treatment [ 93 ].…”

Section: Differential Diagnosismentioning

confidence: 99%

State-of-the-Art Imaging in Human Chordoma of the Skull Base

Santegoeds

Temel

Beckervordersandforth

et al. 2018

Curr Radiol Rep

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Purpose of ReviewChordoma are rare tumours of the axial skeleton which occur most often at the base of the skull and in the sacrum. Although chordoma are generally slow-growing lesions, the recurrence rate is high and the location makes it often difficult to treat. Both computed tomography (CT) and magnetic resonance imaging (MRI) are crucial in the initial diagnosis, treatment planning and post-treatment follow-up.Recent FindingsBasic MRI and CT characteristics of chordoma were described in the late 1980s and early 1990s. Since then, imaging techniques have evolved with increased resolution and new molecular imaging tools are rapidly evolving. New imaging tools have been developed not only to study anatomy, but also physiologic changes and characterization of tissue and assessment of tumour biology. Recent studies show the uptake of multiple PET tracers in chordoma, which may become an important aspect in the diagnosis, follow-up and personalized therapy.SummaryThis review gives an overview of skull base chordoma histopathology, classic imaging characteristics, radiomics and state-of-the-art imaging techniques that are now emerging in diagnosis, treatment planning and disease monitoring of skull base chordoma.

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A Case of Giant Ecchordosis Physaliphora

Abstract: The distinction between giant ecchordosis physaliphora and intradural chordoma is questioned and exemplified by our case. It is possible they represent the same entity of disease, which must be distinguished from classical malignant osseous (extradural) chordoma.

Cited by 34 publications

References 0 publications

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

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

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor

State-of-the-Art Imaging in Human Chordoma of the Skull Base

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