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Table of Contents
Definition / general | Diagrams / tables | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Pernick, N. Chordoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorchordoma.html. Accessed April 20th, 2018.
Definition / general
- 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% thoracolumbar 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)
Diagrams / tables
Images hosted on other servers:
Drawing of clival chordoma
Radiology images
Images hosted on PathOut server:
Sagittal MRI of sacral tumor
Prognostic factors
- 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
- 47 year old man with infected pilonidal cyst (Case of the Week #110)
- 71 year old man with lumbosacral tumor with high grade malignant cartilaginous and spindle cell components (Am J Surg Pathol 1990;14:384)
- Tumor of distal ulna (chordoma periphericum, Am J Surg Pathol 2001;25:263)
- Spheno-occipital tumor evolving to an acute pontocerebellar hemorrhage (Arch Pathol Lab Med 1989;113:1075)
Treatment
- Aggressive surgery, often leading to long survival (Oncologist 2007;12:1344)
- In children, external radiation is often successful for base of skull tumors (Am J Surg Pathol 2006;30:811)
- Some tumors may dedifferentiate to high grade spindle cell sarcomas
Gross description
- Soft, gelatinous, hemorrhagic and gray tumor
Microscopic (histologic) description
- 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
Microscopic (histologic) images
Images hosted on PathOut server:
Sacral tumor
Pankeratin
Synaptophysin
Alcian blue
Images hosted on other servers:
Incipient chordoma
Benign notocordal cell tumor
Neural type cadherin (fig 2D)
Cytology images
Images hosted on other servers:
Diff Quik
Positive stains
- S100, keratin (CK8 / 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
Electron microscopy description
- Mitochondria - endoplasmic reticulum complexes, parallel bundles of crisscrossing tubules and desmosomes (Arch Pathol Lab Med 1993;117:1055)
Molecular / cytogenetics description
- Aneuploid
Differential diagnosis
- Chondrosarcoma: not midline, no fibrous septa, EMA and keratin negative
- Metastatic renal cell carcinoma: prominent vascularity, not lobulated, S100 negative or other carcinoma
- Myxopapillary ependymoma: negative for epithelial markers
- Parachordoma: soft tissue tumor composed of epithelioid cells, smaller "glomoid" cells and spindle cells and negative for CK7, CK19, CK20, CEA
- Signet cell adenocarcinoma of rectum
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