Multicentric Chordoma

Grossbach, Andrew J.; Baimeedi, Praveen; McDonald, W. Scott; Bergman, Thomas

doi:10.1227/neu.0b013e31822a994d

Cited by 9 publications

(9 citation statements)

References 17 publications

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“…Other metastatic sites were rarely reported in literature as heart, mandible, skin, lung, and the cerebrospinal fluid [ 5 , 6 , 7 , 8 , 9 ].Moreover, our case developed also a metachronous chordoma in the clivus. Synchronous and metachronous development of chordomas along the neural axis has been rarely reported [ 10 , 11 , 12 , 13 , 14 ].…”

Section: Discussionmentioning

confidence: 99%

Sacral Chordoma with Bilateral Inguinal Lymph Node Metastasis and Metachronous Tumor of the Clivus: A Case Report

Elghareeb¹,

Allah²,

Abdallah³

et al. 2021

JOCR

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Introduction: Chordoma is a malignant neoplasm that arises from notochord remnants. Its incidence is highest above the age of 50 and behaves as a locally aggressive tumor with a slow growth rate. In most cases, complete surgical resection followed by radiotherapy offers the best chance of control. Developing metachronous tumors or distant metastasis is uncommon. Case Report: A 56-year-old male patient of sacral chordoma was treated by surgery and radiotherapy. He developed later bilateral inguinal lymph node metastasis and metachronous clivus chordoma. Conclusion: Chordomas are rare. Multiplicity of primary disease and distant metastasis could happen, so regular follow-up is warranted and more effective therapeutic modalities are needed. Keywords: Chordoma, sacrococcygeal tumors, notochord tumors, lymph node metastasis, clivus tumors.

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Section: Discussionmentioning

confidence: 99%

Sacral Chordoma with Bilateral Inguinal Lymph Node Metastasis and Metachronous Tumor of the Clivus: A Case Report

Elghareeb¹,

Allah²,

Abdallah³

et al. 2021

JOCR

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“…In support of the multicentricity hypothesis, chordoma is believed to originate from remnants of the embryonal notochord, and metachronous lesions can occur at neuraxial bones. Furthermore, wide dissemination, including that in the appendicular skeleton and lung, should be screened if the tumors located at multiple neuraxial skeletons are not multicentric but multiple metastatic lesions [9,10]. erefore, multiple chordomas at neuraxial bones without other lesions at the appendicular skeleton or pulmonary lesions are thought to be multicentric lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Multicentric chordomas with synchronous multiple presentations at first diagnosis have been rarely reported [8][9][10]. Methods for distinction between multicentric or metastatic multiple chordoma tumors have not yet been established.…”

Section: Introductionmentioning

confidence: 99%

Genetic Testing Distinguishes Multiple Chondroid Chordomas with Neuraxial Bone Metastases from Multicentric Tumors

Kobayashi

Shin

Makise

et al. 2020

Case Reports in Genetics

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Background. Chordomas are rare malignant bone tumors preferentially forming in neuraxial bones. Chondroid chordoma is a subtype of chordoma. Chordomas reportedly present as synchronous multiple lesions upon initial diagnosis. However, it remains unknown whether these lesions are multicentric or metastatic multiple chordoma tumors. Case Presentation. Here, we present the case of a 57-year-old woman with multiple chordomas at the clivus, C6, and T12 upon initial presentation. Sequential surgeries and radiotherapy were performed for these lesions, and postoperative histological diagnosis revealed that all lesions were chondroid chordomas. Next-generation sequencing revealed that these lesions harbored a common somatic mutation in epidermal growth factor receptor (EGFR), c.3617A>C, which is not considered a pathogenic chordoma mutation, thus indicating that these lesions were not multicentric but rather multiple metastatic tumors. Subsequent multiple metastases to the lung and appendicular and axial bones were detected 15 months after the initial surgery. Recurrent lesions at the clivus progressed despite EGFR-targeted therapy, surgery, and radiotherapy. Conclusion. The present evidence indicates that multiple chordomas in this case were caused by multiple metastases rather than multicentric lesions. Multiple presentations of chordoma imply systemic dissemination of tumor cells, and novel efficient systemic therapy is required to treat this disease.

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“…However, very few cases of multicentric chordoma have been described. [14][15][16] CT and MR imaging delineate the exact extent of clival chordomas and the spinal lesions. On T1-weighted images, these appear iso-/hypointense in comparison with healthy brain tissue and hyperintense on T2-weighted images.…”

Section: Discussionmentioning

confidence: 99%