Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas?

Yamaguchi, Takehiko; Suzuki, Sumie; Ishiiwa, Hiroaki; Ueda, Yoshihiko

doi:10.1111/j.1365-2559.2004.01877.x

Cited by 134 publications

(94 citation statements)

References 12 publications

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“…5,6 They 4 This previous case suggested a small intraosseous benign notochordal cell tumor (described as a benign notochordal lesion in the original paper) located adjacent to the coccygeal chordoma. In addition, two other small benign notochordal cell tumors were evident in the sacrum at a different level.…”

Section: Discussionmentioning

confidence: 96%

“…However, we documented a histologically confirmed case of classic chordoma arising in a precursor benign notochordal cell tumor and reported the clinicopathological details of intraosseous benign notochordal cell tumors. [4][5][6] Based on the evidence, we concluded that intraosseous benign notochordal cell tumors are benign notochordal cell lesions with potential neoplastic characteristics and possibly serve as the real precursors of classic chordomas. We recently found two interesting cases of microscopic incipient chordomas coexistent with benign notochordal cell tumors in the coccyx, which supports the above hypothesis.…”

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confidence: 95%

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Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors

et al. 2005

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Chordomas are rare malignant bone tumors primarily involving both ends of the axial skeleton that present as destructive bone lesions with a large soft tissue mass. Chordomas were previously believed to arise from notochordal remnants. However, recent studies suggest the possibility that chordomas arise from benign notochordal cell tumors. We present two cases of coccygeal incipient chordoma that strengthen the new hypothesis. The first case was an 83-year-old man who died of prostatic adenocarcinoma. The second case was a 79-year-old man who died of hepatocellular carcinoma. The coccygeal tumors were composed of intraosseous and extraosseous infiltrative lesions. The intraosseous lesions consisted of both benign notochordal cell tumor and incipient chordoma. The extraosseous lesions were consistent with incipient chordoma. In addition, two other small benign notochordal cell tumors were found at a different level in case 1. It is conceivable that pre-existing intraosseous benign notochordal cell tumors transform into incipient chordoma and then extend through the cortex into the surrounding soft tissue. The incidence of incipient chordoma appears much higher than expected because chordomas are rare tumors with an incidence of one case per 1 000 000 persons per year. We suspect that unknown factors transform incipient chordoma into classic chordoma.

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Section: Discussionmentioning

confidence: 96%

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confidence: 95%

Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors

et al. 2005

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“…These observation were made at autopsy [1]. Since then, a number of such microscopic remnants have been found incidentally at autopsy, generally less than 1 cm [1,8]. More recently, larger intravertebral notochordal lesion seen radiologically have been described.…”

Section: Discussionmentioning

confidence: 99%

“…Benign notochordal cell tumors are intraosseous lesions which can occur along the midline of the craniospinal axis, particularly in the saccrococcygeal or sphenooccipital region [8,10]. A recent autopsy series found BNCTs in 20% of 100 cadavers, with a mean age of 63 years (7 to 82 years), with a distribution of 12% in saccrococcygeal vertebrae, 11.5% in the clival region, 5% in cervical vertebrae, 2% in lumbar vertebrae and none in the thoracic vertebrae [8].…”

Section: Discussionmentioning

confidence: 99%

Case Report: An Unusual Osseous Lesion

Rivière¹,

Rousset²,

Bauchet³

et al. 2013

OJPathology

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The intraosseous benign notochordal cell tumor (BNCT) is an intravertebral lesion derived from notochordal tissue. The notochord develops in humans during the third week of embryonic life and persists in adults as the nucleus pulposus of the vertebral disks. The most common locations of such tumors are the saccrococcygeal region and the skull base. Most tumors are asymptomatic and small. Magnetic resonance imaging is useful in their detection and precise localization. Histologically, these lesions consist of sheets of adipocyte-like vacuolated or less vacuolated eosinophilic tumor cells with eccentrically located round nuclei without myxoid matrix. The tumor cells express the epithelial markers, vimentin and S100 protein. Main differential diagnosis is chordoma. Intraosseous BNCT do not require any surgical management and should be recognized by pathologists to prevent unnecessary radical surgery. These lesions should be followed-up with conventional MRI.

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“…Chordomas have a predilection for the craniospinal axis and are thought to arise from notochordal rests which evolve into nucleus pulposus, explaining the predominant midline and paramedian locations [6]. Newer literature challenges this traditional concept suggesting that chordomas arise from pre-existing benign notochordal cell tumours [7][8][9][10]. Chordomas present between the fourth and seventh decade with a male to female ratio of 2:1 [6,11].…”

Section: Discussionmentioning

confidence: 99%

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

et al. 2015

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Chordomas are rare malignant bone tumours with a predilection for the axial skeleton, especially the sacrum and skull base. Median survival in patients with metastatic disease is usually dismal. Treatment is challenging due to the propensity for local recurrence, metastatic disease as well as lack of clear consensus regarding the optimal management. Our case report highlights two cases of sacral chordoma with locally recurrent and widespread metastatic disease, stable on molecular targeted therapy. Keywords: Chordoma, metastasis, imatinib, positron emission tomography ÖzetKordomalar özellikle sakrum ve kafa tabanı ile eksenel iskelet yerleş-me eğiliminde olan, nadir görülen malign kemik tümörleridir. Metastatik hastalığı olan hastalarda sağkalım ortanca değeri genellikle umutsuzdur. Tedavi, lokal nüks, metastatik hastalık yanı sıra optimum tedaviye ilişkin net konsensus eksikliği nedeniyle zordur. Bizim olgu sunumumuzda moleküler hedefli tedavinin istikrarlı yerel tekrarlayan ve yaygın metastatik hastalığı olan sakral kordomayla ilgili iki olgu bulunmaktadır. Anahtar Kelimeler: Kordoma, metastaz, imatinib, pozitron emisyon tomografiCorrespondence to: Saurabh Rohatgi,

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Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas?

Cited by 134 publications

References 12 publications

Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors

Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors

Case Report: An Unusual Osseous Lesion

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

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