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See also chondroid chordoma below
General
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- Rare malignant midline bone tumor arising from fetal notocord, usually within vertebral bodies, but possibly also in intervertebral discs or presacral soft tissue
- May arise from intraosseous benign notochordal cell tumors (Am J Surg Pathol 2007;31:1573, Mod Pathol 2005;18:1005)
- Usually males, age 40-60 years
- Slow growing with repeated recurrences; late distant metastases to skin, bone, ovary (Arch Pathol Lab Med 1990;114:208)
- Invasiveness may be due to expression of cathepsin K (Hum Pathol 2000;31:834)
- Sites: 50% sacrococcygeal (ages 40-59 years), 35% spheno-occipital / clivus (particularly children, image of clivus) and 15% thoraco-lumbar spine
- Sacrococcygeal: sacrum destroyed by osteolytic tumor; tumor may extend into retroperitoneum, presents as palpable extrarectal mass
- Spheno-occipital: presents as nasal, paranasal or nasopharyngeal mass involving cranial nerves
- Posterior mediastinum: Xray presentation is well-circumscribed, encapsulated soft tissue mass separate from spine (Hum Pathol 1995;26:1354)
- References: Arch Pathol Lab Med 1988;112:553 (stains), Mod Pathol 1997;10:545 (keratin stains)
Xray images
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Sagittal MRI of sacral tumor
Prognostic factors
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- Poor prognostic factors: large tumor size, positive surgical margins, tumor necrosis, high proliferative activity and areas of dedifferentiation; also up regulation of N cadherin and down regulation of E cadherin (Am J Surg Pathol 2005;29:1422)
Case reports
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Treatment
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Gross description
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- Soft, gelatinous, hemorrhagic and gray tumor
Gross images
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Drawing of clival chordoma
Micro description
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- Cords and lobules of physaliferous (having bubbles or vacuoles) cells separated by fibrous septa with extensive myxoid stroma
- Cells may be very large, with vacuolated cytoplasm and prominent vesicular nucleus (Arch Pathol Lab Med 2004;128:1457)
- Also small tumor cells with small nucleus; rare mitotic figures
Micro images
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Cytology images
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Positive stains
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- S100, keratin (CK 8/18, CK19, AE1-AE3), EMA, 5' nucleotidase, glycogen, neural-type cadherin (Arch Pathol Lab Med 2002;126:425), variable CK903, vimentin, CEA and lysozyme
- Brachyury may distinguish chordoma from histologic mimics, including clear cell renal cell carcinoma, chondrosarcoma, chordoid meningioma (Am J Surg Pathol 2009;33:669, Mod Pathol 2011;24:425)
Negative stains
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Molecular description
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Electron microscopy description
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Differential diagnosis
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- Chondrosarcoma:: not midline, no fibrous septa, EMA and keratin negative
- Metastatic renal cell carcinoma prominent vascularity, not lobulated, S100 negative or other carcinoma
- Parachordoma: soft tissue tumor composed of epithelioid cells, smaller "glomoid" cells and spindle cells and negative for CK7, CK19, CK20, CEA
- Myxopapillary ependymoma: negative for epithelial markers
- Signet cell adenocarcinoma of rectum
Chondroid chordoma
General
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- Chordoma with prominent cartilaginous component - a controversial entity
- Usually spheno-occipital region
- Better prognosis than classic chordoma
- Some consider them to be chondrosarcomas
- References: Am J Clin Pathol 1992;16:1144
Micro description
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- Matrix of amorphous blue ground substance with lacunae containing enlarged and slightly atypical cells
- Admixed areas of conventional chordoma
Cytology description
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Positive stains
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- Both components: keratin, EMA and vimentin; often S100, occasionally CEA
Electron microscopy description
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- Well formed tonofilament desmosome complexes, crystalline tubular structures within prominent and dilated rough endoplasmic reticulum, intracytoplasmic glycogen aggregates and abundant fibrillogranular matrix (Am J Surg Pathol 1983;7:625)
End of CNS tumor > Mesenchymal and other tumors > Chordoma
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