Pathology Outlines

CNS tumor
Mesenchymal and other tumors
Chordoma

Topic Completed: 1 January 2006

Revised: 3 January 2019, last major update January 2006

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Chordoma[title] CNS tumor "loattrfree full text"[sb]

See also: Chondroid chordoma

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Table of Contents

Cite this page: Pernick N. Chordoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorchordoma.html. Accessed October 14th, 2019.

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

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Drawing of clival chordoma

Radiology images

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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

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Sacral tumor

Pankeratin

Synaptophysin

Alcian blue

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Neural type cadherin (fig 2D)

Cytology images

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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

CK7, CK20 and chromogranin (Hum Pathol 1998;29:119)

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