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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Chordoma_-_high_mag.jpg | |||
| Width = | |||
| Caption = Chordoma. [[HPS stain]]. | |||
| Micro = ''physaliphorous cells'' (also ''bubble cells'') - very large clear bubble with a sharp border, bubble does not compress nucleus; islands of cells surrounded by fibrous tissue; myxoid background | |||
| Subtypes = | |||
| LMDDx = [[chondrosarcoma]], [[myxoid lesions]], [[parachordoma]], chordoid lesions (e.g. chordoid glioma, [[chordoid meningioma]]), [[metastasis]] (e.g. [[clear cell renal cell carcinoma]]) | |||
| Stains = | |||
| IHC = S-100 +ve, [[AE1/AE3]] +ve, Brachyury +ve, [[EMA]] +ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = myxoid | |||
| Grossing = | |||
| Site = sacrum or clivus | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Chordoma''' is an uncommon tumour in [[neuropathology]]. | '''Chordoma''' is an uncommon tumour in [[neuropathology]]. | ||
==General== | ==General== | ||
*Location: usually sacrum or clivus. | *Location: usually sacrum or clivus. | ||
*It is a [[bone tumour]]. | *It is a malignant [[bone tumour]] (1-4% of all primary bone tumors). | ||
*Usually after age 30. | |||
===Classification=== | |||
* Chordoma, NOS (ICD-O: 9370/3). | |||
* Chondroid chordoma (ICD-O: 9371/3). | |||
* Dedifferentiated chordoma (ICD-O: 9372/3). | |||
==Gross== | ==Gross== | ||
| Line 25: | Line 60: | ||
DDx: | DDx: | ||
*[[Chondrosarcoma]]. | *[[Chondrosarcoma]] - negative for EMA and cytokeratins. Beware of 'chondroid' chordoma. | ||
*[[Myxoid lesions]]. | |||
**[[Myxopapillary ependymoma]]. | |||
**[[Myxoid liposarcoma]] - negative for EMA and cytokeratins. | |||
*Chordoid lesions. | |||
**[[Chordoid meningioma]]. | |||
**Chordoid glioma<ref>{{Cite journal | last1 = Zarghouni | first1 = M. | last2 = Vandergriff | first2 = C. | last3 = Layton | first3 = KF. | last4 = McGowan | first4 = JB. | last5 = Coimbra | first5 = C. | last6 = Bhakti | first6 = A. | last7 = Opatowsky | first7 = MJ. | title = Chordoid glioma of the third ventricle. | journal = Proc (Bayl Univ Med Cent) | volume = 25 | issue = 3 | pages = 285-6 | month = Jul | year = 2012 | doi = | PMID = 22754136 }}</ref> - location, location, location. | |||
**Large notochordal rest - only evidence of destructive growth can identify a chordoma. | |||
*[[Metastasis]]. | |||
**Metastatic [[signet ring cell carcinoma]] - negative for S100 and brachyury, [[clinical history]] (important). | |||
**Metastatic [[clear cell renal cell carcinoma]] - negative for S100 and brachyury, clinical history (important). | |||
*[[Parachordoma]] - extremely rare. | |||
===Images=== | ===Images=== | ||
====Case 1==== | |||
<gallery> | <gallery> | ||
Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC) | Image:Chordoma_-_low_mag.jpg | Chordoma - low mag. (WC) | ||
Image:Chordoma - intermed mag.jpg| Chordoma - intermed. mag. (WC) | |||
Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC) | Image:Chordoma_-_high_mag.jpg | Chordoma - high mag. (WC) | ||
Image:Chordoma - very high mag.jpg| Chordoma - very high mag. (WC) | |||
</gallery> | |||
====Case 2==== | |||
<gallery> | |||
Image: Chordoma -- low mag.jpg | Chordoma - low mag. (WC) | |||
Image: Chordoma -- intermed mag.jpg | Chordoma - intermed. mag. (WC) | |||
Image: Chordoma - alt -- intermed mag.jpg | Chordoma - intermed. mag. (WC) | |||
Image: Chordoma -- high mag.jpg | Chordoma - high mag. (WC) | |||
</gallery> | </gallery> | ||
www | ====Case 3==== | ||
<gallery> | |||
Image:Bone Chordoma 2 HP2.JPG|Physaliphorous cells. (SKB) | |||
Image:Bone Chordoma 2 LP PA.JPG|Low power view. Somewhat lobulated tumour with loose areas, cellular areas and fibrous septa. (SKB) | |||
Image:Bone Chordoma PA.jpg|Physaliphorous cells. (SKB) | |||
Image:Bone Chordoma 2 HP PA.JPG|Physaliphorous cells. (SKB) | |||
Image:Bone Chordoma 2 MP PA.JPG|Physaliphorous cells.(SKB) | |||
Image:Bone Chordoma HP PA.JPG|Chordoma cells may form sheets of cells with eosinophilic cytoplasm. (SKB) | |||
</gallery> | |||
====www==== | |||
*[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)]. | *[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)]. | ||
*[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)]. | *[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)]. | ||
| Line 39: | Line 104: | ||
==IHC== | ==IHC== | ||
Features:<ref>URL: [http://path.upmc.edu/cases/case312/micro.html http://path.upmc.edu/cases/case312/micro.html]. Accessed on: 14 January 2012.</ref><ref name=pmid2431128>{{Cite journal | last1 = Coindre | first1 = JM. | last2 = Rivel | first2 = J. | last3 = Trojani | first3 = M. | last4 = De Mascarel | first4 = I. | last5 = De Mascarel | first5 = A. | title = Immunohistological study in chordomas. | journal = J Pathol | volume = 150 | issue = 1 | pages = 61-3 | month = Sep | year = 1986 | doi = 10.1002/path.1711500110 | PMID = 2431128 }}</ref> | Features:<ref>URL: [http://path.upmc.edu/cases/case312/micro.html http://path.upmc.edu/cases/case312/micro.html]. Accessed on: 14 January 2012.</ref><ref name=pmid2431128>{{Cite journal | last1 = Coindre | first1 = JM. | last2 = Rivel | first2 = J. | last3 = Trojani | first3 = M. | last4 = De Mascarel | first4 = I. | last5 = De Mascarel | first5 = A. | title = Immunohistological study in chordomas. | journal = J Pathol | volume = 150 | issue = 1 | pages = 61-3 | month = Sep | year = 1986 | doi = 10.1002/path.1711500110 | PMID = 2431128 }}</ref> | ||
* | *S-100 +ve - '''important'''. | ||
*AE1/AE3 +ve. | *[[AE1/AE3]] +ve - '''important'''. | ||
*Brachyury +ve | *Brachyury +ve - '''key stain'''. | ||
**Protein important for axial development, affects notochord development.<ref name=omim601397>{{OMIM|601397}}</ref> | **Protein important for axial development, affects notochord development.<ref name=omim601397>{{OMIM|601397}}</ref> | ||
**''Brachyury'' literally means ''short tail''.<ref>URL: [http://www.jstor.org/pss/86845 http://www.jstor.org/pss/86845]. Accessed on: 18 May 2010.</ref> | **''Brachyury'' literally means ''short tail''.<ref>URL: [http://www.jstor.org/pss/86845 http://www.jstor.org/pss/86845]. Accessed on: 18 May 2010.</ref> | ||
*EMA +ve. | *[[EMA]] +ve. | ||
*[[PAX8]] -ve (1 +ve (weak) of 12.<ref name=pmid21102418>{{Cite journal | last1 = Sangoi | first1 = AR. | last2 = Karamchandani | first2 = J. | last3 = Lane | first3 = B. | last4 = Higgins | first4 = JP. | last5 = Rouse | first5 = RV. | last6 = Brooks | first6 = JD. | last7 = McKenney | first7 = JK. | title = Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases. | journal = Mod Pathol | volume = 24 | issue = 3 | pages = 425-9 | month = Mar | year = 2011 | doi = 10.1038/modpathol.2010.196 | PMID = 21102418 }}</ref>). | |||
*Other keratins:<ref name=pmid9195570>{{Cite journal | last1 = Naka | first1 = T. | last2 = Iwamoto | first2 = Y. | last3 = Shinohara | first3 = N. | last4 = Chuman | first4 = H. | last5 = Fukui | first5 = M. | last6 = Tsuneyoshi | first6 = M. | title = Cytokeratin subtyping in chordomas and the fetal notochord: an immunohistochemical analysis of aberrant expression. | journal = Mod Pathol | volume = 10 | issue = 6 | pages = 545-51 | month = Jun | year = 1997 | doi = | PMID = 9195570 }}</ref> | |||
**CK8 +ve (16/16). | |||
**CK19 +ve (16/16). | |||
**CK18 +ve/-ve (9 +ve/16). | |||
**[[CK7]] -ve (1 +ve/16). | |||
**[[CK20]] -ve (0 +ve/16). | |||
Classic chordoma panel:<ref name=pmid24296480>{{Cite journal | last1 = Shen | first1 = J. | last2 = Shi | first2 = Q. | last3 = Lu | first3 = J. | last4 = Wang | first4 = DL. | last5 = Zou | first5 = TM. | last6 = Yang | first6 = HL. | last7 = Zhu | first7 = GQ. | title = Histological study of chordoma origin from fetal notochordal cell rests. | journal = Spine (Phila Pa 1976) | volume = 38 | issue = 25 | pages = 2165-70 | month = Dec | year = 2013 | doi = 10.1097/BRS.0000000000000010 | PMID = 24296480 }}</ref> | |||
*EMA, AE1/AE3, CAM5.2, vimentin, S-100. | |||
Key points: | |||
*Brachyury is not a commonly stocked antibody. | |||
*Chordoma will be S100 '''''and''''' epithelial marker positive. | |||
*Many other items in the DDx will be either S100 '''''or''''' epithelial marker positive. | |||
==See also== | ==See also== | ||
*[[CNS tumours]]. | *[[CNS tumours]]. | ||
*[[Parachordoma]]. | |||
==References== | ==References== | ||