Prognostic Implications of Gadolinium Enhancement of Skull Base Chordomas

Lin, Eaton; Scognamiglio, Theresa; Yu, Zhengping; Schwartz, Theodore H.; Phillips, C. Douglas

doi:10.3174/ajnr.a5714

Cited by 11 publications

(15 citation statements)

References 39 publications

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“…Chordoma is frequently hyperintense on T2-weighted imaging with hypointense septations and small foci of hypointensity corresponding to hemorrhage, calcification and mucus pooling [ 56 , 57 ]. Most chordomas will enhance moderately-to-intensely with gadolinium in a variable “honeycomb” pattern and some studies suggest that the degree of contrast enhancement correlates with aggressive tumor behavior and risk of recurrence/progression after surgical resection [ 58 ].…”

Section: Diagnosismentioning

confidence: 99%

“…Four subtypes of chordoma are currently recognized: conventional (which is the most common), chondroid (in which areas mimicking hyaline or myxoid cartilage are seen), poorly differentiated and dedifferentiated or sarcomatoid chordoma (chordoma associated with a high-grade sarcoma, which accounts for only 5% of cases and confers the worst prognosis) [ 2 , 58 , 59 , 60 ]. Because chondroid chordomas share pathological features with chondrosarcoma and because their clinical presentation and radiographic appearance are often similar, distinguishing between the two histologically may be challenging.…”

Section: Diagnosismentioning

confidence: 99%

“…Other reported prognostic factors for chordomas include pathology (with dedifferentiated chordomas having a poorer prognosis), history of prior resection (secondary patients referred due to recurrence after a previous resection have a poorer prognosis) and in sacral chordomas, the presence of muscle/sacroiliac joint involvement and higher location (above S3), all of which are reported to confer a poorer prognosis [ 12 , 58 , 59 , 78 ].…”

Section: Prognosismentioning

confidence: 99%

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Chordoma—Current Understanding and Modern Treatment Paradigms

Barber

Sadrameli

Lee

et al. 2021

JCM

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Chordoma is a low-grade notochordal tumor of the skull base, mobile spine and sacrum which behaves malignantly and confers a poor prognosis despite indolent growth patterns. These tumors often present late in the disease course, tend to encapsulate adjacent neurovascular anatomy, seed resection cavities, recur locally and respond poorly to radiotherapy and conventional chemotherapy, all of which make chordomas challenging to treat. Extent of surgical resection and adequacy of surgical margins are the most important prognostic factors and thus patients with chordoma should be cared for by a highly experienced, multi-disciplinary surgical team in a quaternary center. Ongoing research into the molecular pathophysiology of chordoma has led to the discovery of several pathways that may serve as potential targets for molecular therapy, including a multitude of receptor tyrosine kinases (e.g., platelet-derived growth factor receptor [PDGFR], epidermal growth factor receptor [EGFR]), downstream cascades (e.g., phosphoinositide 3-kinase [PI3K]/protein kinase B [Akt]/mechanistic target of rapamycin [mTOR]), brachyury—a transcription factor expressed ubiquitously in chordoma but not in other tissues—and the fibroblast growth factor [FGF]/mitogen-activated protein kinase kinase [MEK]/extracellular signal-regulated kinase [ERK] pathway. In this review article, the pathophysiology, diagnosis and modern treatment paradigms of chordoma will be discussed with an emphasis on the ongoing research and advances in the field that may lead to improved outcomes for patients with this challenging disease.

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Section: Diagnosismentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

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Chordoma—Current Understanding and Modern Treatment Paradigms

Barber

Sadrameli

Lee

et al. 2021

JCM

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“…With MR imaging analysis, the sampling error is greatly reduced because ROIs are selected by drawing the largest possible elliptic area within the tumor but avoiding areas of hemorrhage, necrosis, cyst, or calcification. 15,17,25 This feature may also indicate the emergence of new malignant cell populations within the recurrent tumor when the MRT tumors recurred as CDT tumors in some cases. Additionally, we found that most of the tumors that underwent multiple resections eventually evolved into CDTs (10/14) (Table 3).…”

Section: Discussionmentioning

confidence: 99%

“…14,15 Recent studies have suggested that MR imaging signal intensity ratios on different sequences (ratio of tumor-to-pons signal intensity on T1 FLAIR [R T1 ]; ratio of tumor-to-pons signal intensity on T2 [R T2 ]; ratio of tumor-to-pons signal intensity on enhanced T1 FLAIR [R EN ]) are useful in predicting outcomes. Tian et al 16 revealed that higher R T2 predicted diminished tumor progression and higher R EN predicted more rapid tumor progression; this correlation between R EN and recurrence or progression was also shown by Lin et al 17 However, neither of these studies showed evidence for the possible explanation of signal intensity difference.…”

mentioning

confidence: 80%

MRI Signal Intensity and Electron Ultrastructure Classification Predict the Long-Term Outcome of Skull Base Chordomas

Bai

Shi

Zhang

et al. 2020

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: MR imaging is a useful and widely used evaluation for chordomas. Prior studies have classified chordomas into cell-dense type and matrix-rich type according to the ultrastructural features. However, the relationship between the MR imaging signal intensity and ultrastructural classification is unknown. We hypothesized that MR imaging signal intensity may predict both tumor ultrastructural classification and prognosis.MATERIALS AND METHODS: Seventy-nine patients with skull base chordomas who underwent 95 operations were included in this retrospective single-center series. Preoperative tumor-to-pons MR imaging signal intensity ratios were calculated and designated as ratio on T1 FLAIR sequence (R T1 ), ratio on T2 sequence (R T2 ), and ratio on enhanced T1 FLAIR sequence (R EN ), respectively. We assessed the relationships among signal intensity ratios, ultrastructural classification, and survival. RESULTS:Compared with the matrix-rich type group, the cell-dense type chordomas showed lower R T2 (cell-dense type: 1.90 6 0.38; matrix-rich type: 2.61 6 0.60 P , .001). The model of predicting cell-dense type based on R T2 had an area under the curve of 0.83 (95% CI, 0.75-0.92). In patients without radiation therapy, both progression-free survival (P ¼ .003) and overall survival (P ¼ .002) were longer in the matrix-rich type group than in the cell-dense type group. R EN was a risk factor for progression-free survival (hazard ratio ¼ 10.24; 95% CI, 1.73-60.79); R T2 was a protective factor for overall survival (hazard ratio ¼ 0.33; 95% CI, 0.12-0.87); and R EN was a risk factor for overall survival (hazard ratio ¼ 4.76; 95% CI, 1.51-15.01). CONCLUSIONS:The difference in MR imaging signal intensity in chordomas can be explained by electron microscopic features. Both signal intensity ratios and electron microscopic features may be prognostic factors.

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Imaging of pituitary tumors: an update with the 5th WHO Classifications—part 2. Neoplasms other than PitNET and tumor-mimicking lesions

Tsukamoto¹,

Miki²

2023

Jpn J Radiol

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Many types of tumors can develop in the pituitary gland. In the recently revised 5th editions of the World Health Organization (WHO) classifications (2021 WHO Classification of Central Nervous System Tumors and the 2022 WHO Classification of Endocrine and Neuroendocrine Tumors), various changes have been made to the tumors other than pituitary neuroendocrine tumor (PitNET)/pituitary adenoma, as well as PitNET. Adamantinomatous craniopharyngioma and papillary craniopharyngioma are now considered separate tumors in the 5th edition of the WHO classification. Tumors positive for thyroid transcription factor 1, a marker of posterior pituitary cells, are now grouped together in the pituicyte tumor family in the 5th edition of the WHO classification of Endocrine and Neuroendocrine Tumors. Poorly differentiated chordoma is newly listed in the 5th edition of the WHO Classification of Endocrine and Neuroendocrine Tumors. In this paper, we present the latest WHO classification of pituitary tumors (adamantinomatous craniopharyngioma, papillary craniopharyngioma, pituitary blastoma, pituicyte tumor family, tumors of pituitary origin other than those of the pituicyte tumor family, germinoma, meningioma, chordoma, metastatic tumors, lymphoma, and pituitary incidentaloma), review diseases requiring differentiation from tumors (pituitary abscess, hypophysitis, pituitary hyperplasia, Rathke’s cleft cyst, arachnoid cyst, and aneurysm), and discuss diagnoses based on imaging findings.

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Prognostic Implications of Gadolinium Enhancement of Skull Base Chordomas

Abstract: Enhancement of skull base chordomas is a risk factor for tumor progression/recurrence following surgical resection.

Cited by 11 publications

References 39 publications

Chordoma—Current Understanding and Modern Treatment Paradigms

Chordoma—Current Understanding and Modern Treatment Paradigms

MRI Signal Intensity and Electron Ultrastructure Classification Predict the Long-Term Outcome of Skull Base Chordomas

Imaging of pituitary tumors: an update with the 5th WHO Classifications—part 2. Neoplasms other than PitNET and tumor-mimicking lesions

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