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| '''Chondro-osseous tumours''' occasionally cross the desk of the pathologist. They are grouped together as [[bone]] may develop from [[cartilage]]. | | [[Image:Osteosarcoma - very high mag.jpg|thumb|250px|right|A chondro-osseous tumour ([[osteosarcoma]]). [[H&E stain]].]] |
| | '''Chondro-osseous tumours''' occasionally cross the desk of the pathologist. They are grouped together as [[bone]] may develop from [[cartilage]]. |
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| Primary bone tumours are rare; the most common bone tumour is [[metastases]].<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref> | | Primary bone tumours are rare; the most common bone tumour is [[metastases]].<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref> |
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| | ? | | | ? |
| | no stains / may be assoc. with [[FAP]] | | | no stains / may be assoc. with [[FAP]] |
| | Image (???) | | | [[Image:Osteoma -- intermed mag.jpg |thumb|center|150px| Osteoma. (WC)]] |
| |- | | |- |
| | [[Osteoid osteoma]] | | | [[Osteoid osteoma]] |
| | osteoblastic rimming | | | osteoblastic rimming |
| | anastomosing bony trabeculae | | | anastomosing bony trabeculae |
| | <= 1.5 cm, metaphysis | | | must be <2 cm,<ref name=pmid25224389>{{Cite journal | last1 = Yalcinkaya | first1 = U. | last2 = Doganavsargil | first2 = B. | last3 = Sezak | first3 = M. | last4 = Kececi | first4 = B. | last5 = Argin | first5 = M. | last6 = Basdemir | first6 = G. | last7 = Oztop | first7 = F. | title = Clinical and morphological characteristics of osteoid osteoma and osteoblastoma: a retrospective single-center analysis of 204 patients. | journal = Ann Diagn Pathol | volume = 18 | issue = 6 | pages = 319-25 | month = Dec | year = 2014 | doi = 10.1016/j.anndiagpath.2014.08.006 | PMID = 25224389 }}</ref> metaphysis |
| | painful, NSAIDs remove pain, young | | | painful, NSAIDs remove pain, young |
| | IHC / other | | | IHC / other |
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| | osteoblastic rimming | | | osteoblastic rimming |
| | anastomosing bony trabeculae | | | anastomosing bony trabeculae |
| | > 1.5 cm, metaphysis | | | must be >1 cm,<ref name=pmid25224389/> often >=2 cm, metaphysis |
| | not painful | | | not painful |
| | IHC / other | | | IHC / other |
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| =Cartilage= | | =Cartilage= |
| ==Chondroma== | | ==Chondroma== |
| ===General===
| | {{Main|Chondroma}} |
| *Benign thingy.
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| *Usual legs and feet.
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| *May be difficult to separate from [[chondrosarcoma]].
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| *Multiple chondromas = ''enchondromatosis''; three distinct syndromes:<ref name=emed_enchondroma>URL: [http://emedicine.medscape.com/article/389224-overview http://emedicine.medscape.com/article/389224-overview]. Accessed on: 25 December 2010.</ref>
| |
| **Ollier disease.
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| **Maffucci syndrome - with [[hemangioma]]s, increased risk of [[chondrosarcoma]].<ref name=omim166000>{{OMIM|166000}}</ref>
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| **Metachondromatosis - autosomal dominant.
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| *''Enchondroma'' = chondroma in the marrow space.
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| | |
| Clinical:<ref name=emed_enchondroma>URL: [http://emedicine.medscape.com/article/389224-overview http://emedicine.medscape.com/article/389224-overview]. Accessed on: 25 December 2010.</ref>
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| *Pain.
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| | |
| ===Radiology===
| |
| Features:<ref name=emed_enchondroma>URL: [http://emedicine.medscape.com/article/389224-overview http://emedicine.medscape.com/article/389224-overview]. Accessed on: 25 December 2010.</ref>
| |
| *Lytic lesion.
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| *Usual close to a growth plate.
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|
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|
| ===Microscopic===
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| Features:
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| *Ctyologically benign cells is spaced nests.
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|
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| ====Images====
| |
| <gallery>
| |
| Image:Enchondroma_-_intermed_mag.jpg | Enchondroma - intermed mag. (WC)
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| Image:Enchondroma_-_very_high_mag.jpg | Enchondroma - very high mag. (WC)
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| </gallery>
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| ==Chondroblastoma== | | ==Chondroblastoma== |
| ===General===
| | {{Main|Chondroblastoma}} |
| *Growth plate lesion.
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| *Sclerotic margin.
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| *"Young" = growth plates open.
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| *Typically painful.<ref name=Ref_PCPBoD8_625>{{Ref PCPBoD8|625}}</ref>
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| | |
| ===Gross===
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| *Well-defined lesion.
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| | |
| Image:
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| *[http://www.flickr.com/photos/35441329@N05/4052875138/in/set-72157622681280610 Chondroblastoma (flickr.com/humpath)].
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| | |
| ===Microscopic===
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| Features:<ref name=Ref_WMSP_642>{{Ref WMSP|642}}</ref>
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| *Abundant extracellular material - pink on [[H&E stain]] - looks vaguely like cartilage.
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| *Chondroblasts:
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| **Nuclear morphology variable: ovoid, folded or grooved.
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| **Moderate-abundant eosinophilic cytoplasm.
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| *+/-Calcification surrounds the cell nests ("chickenwire" appearance) - '''classic feature'''.
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| **Cell nests have a thin pale blue rimming.
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| *+/-[[Giant cells]].
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| **May lead to confusion with ''[[giant cell tumour of bone]]''.
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| | |
| DDx:
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| *[[Giant cell tumour of bone]].
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| *[[Chondroma]].
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| *Well-differentiated [[chondrosarcoma]].
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| | |
| ====Images====
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| <gallery>
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| Image:Chondroblastoma_-_intermed_mag.jpg | Chondroblastoma - intermed. mag. (WC)
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| Image:Chondroblastoma_-_very_high_mag.jpg | Chondroblastoma - very high mag. (WC)
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| </gallery>
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| www:
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| *[http://img.medscape.com/pi/emed/ckb/orthopedic_surgery/1230552-1254949-996.jpg Chondroblastoma (medscape.com)].<ref name=emed_chondroblastoma>URL: [http://emedicine.medscape.com/article/1254949-diagnosis http://emedicine.medscape.com/article/1254949-diagnosis]. Accessed on: 31 December 2010.</ref>
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| *[http://img.medscape.com/pi/emed/ckb/orthopedic_surgery/1230552-1254949-997.jpg Chondroblastoma with "chickenwire" appearance (medscape.com)].<ref name=emed_chondroblastoma>URL: [http://emedicine.medscape.com/article/1254949-diagnosis http://emedicine.medscape.com/article/1254949-diagnosis]. Accessed on: 31 December 2010.</ref>
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| *[http://path.upmc.edu/cases/case494/images/fig2a.jpg Chondroblastoma (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case494.html http://path.upmc.edu/cases/case494.html]. Accessed on: 24 January 2012.</ref>
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| | |
| ===IHC===
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| Features:<ref name=Ref_WMSP_642>{{Ref WMSP|642}}</ref>
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| *S100 +ve.
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| *Vimentin +ve.<ref name=emed_chondroblastoma>URL: [http://emedicine.medscape.com/article/1254949-diagnosis http://emedicine.medscape.com/article/1254949-diagnosis]. Accessed on: 31 December 2010.</ref>
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| ==Chondromyxoid fibroma== | | ==Chondromyxoid fibroma== |
| ===General===
| | {{Main|Chondromyxoid fibroma}} |
| *Benign.
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| *Teenagers or young adults.
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| | |
| ===Gross===
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| *Metaphyseal lesion.
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| *Well-circumscribed.
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| ===Microscopic===
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| Features:<ref name=Ref_WMSP642>{{Ref WMSP|642}}</ref>
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| *Spindle cells or stellate cells in a myxoid or chondroid stroma.
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| Images:
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| *[http://www.webpathology.com/image.asp?n=7&Case=331 Chondromyxoid fibroma - low mag. (webpathology.com)].
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| *[http://www.webpathology.com/image.asp?case=331&n=8 Chondromyxoid fibroma - high mag. (webpathology.com)].
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| ==Chondrosarcoma== | | ==Chondrosarcoma== |
| ===General===
| | {{Main|Chondrosarcoma}} |
| *Usually a good prognosis.
| |
| | |
| Clinical/epidemiologic features:<ref name=pmid17976362>{{cite journal |author=Skubitz KM, D'Adamo DR |title=Sarcoma |journal=Mayo Clin. Proc. |volume=82 |issue=11 |pages=1409–32 |year=2007 |month=November |pmid=17976362 |doi= |url=http://www.mayoclinicproceedings.com/content/82/11/1409.long}}</ref>
| |
| *Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma).
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| *May be associated with a syndrome:
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| **Olier disease (multiple enchondromatosis).
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| **Maffucci syndrome (multiple enchondromas and hemangiomas).
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| | |
| Notes:
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| *Review article (from oncology perspective): PMID 17545802.
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| | |
| ====Subtypes====
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| Several subtypes exist:
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| *Chondrosarcoma not otherwise specified (NOS).
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| *Juxtacortical chondrosarcoma.
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| *Myxoid chondrosarcoma.
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| *Mesenchymal chondrosarcoma.
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| *Clear cell chondrosarcoma.
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| *Dedifferentiated chondrosarcoma.
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| | |
| ===Microscopic===
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| Features:<ref>IAV. 26 February 2009.</ref><ref name=Ref_Klatt417>{{Ref Klatt|417}}</ref>
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| *"Abnormal cartilage":
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| **+/-Nuclear atypia - high grade lesions.
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| ***High grade lesions:
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| ****Nuclear clearing.
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| ****Nucleoli.
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| ****Hyperchromasia.
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| ***Low/intermediate grade lesions:
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| ****Bi-nucleation.
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| ****Hypochromatic enlarged nuclei.
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| ****Infiltration of lamellar bone ("invasion") - not common - '''diagnostic'''.
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| **Increased cellularity.
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| ***More cellular than cartilage... but relatively paucicellular compared to other sarcomas.
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| **Irregular spacing of chondrocytes.
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| | |
| Notes:
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| *Low grade chondrosarcoma are not cytologically malignant; the diagnosis rests mostly on radiologic findings.
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| **The exception is ''infiltration of lamellar bone'' -- this is diagnostic of chondrosarcoma.<ref>Dickson, B. 28 April 2011.</ref>
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| DDx:
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| *[[Chordoma]].
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| *[[Enchondroma]].
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| *[[Synovial chondromatosis]].
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| *[[Osteosarcoma]] - esp. [[chondroblastic osteosarcoma]] - has osteoid, may be focal.
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| | |
| =====Images=====
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| <gallery>
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| Image:Chondrosarcoma_(1).jpg | Chondrosarcoma - low mag. (WC)
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| Image:Chondrosarcoma_(2).jpg | Chondrosarcoma - high mag. (WC)
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| Image:Chondrosarcoma_(3).jpg | Chondrosarcoma - high mag. (WC)
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| </gallery>
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| *www:
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| **[http://path.upmc.edu/cases/case168.html Chondrosarcoma (upmc.edu)].
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| | |
| ====Variants====
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| =====Mesenchymal chondrosarcoma=====
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| *Arise in soft tissue; this is where the name comes from.<ref name=pmid14161087>{{cite journal |author=Dowling EA |title=Mesenchymal chondrosarcoma |journal=J Bone Joint Surg Am |volume=46 |issue= |pages=747–54 |year=1964 |month=June |pmid=14161087 |doi= |url=http://www.ejbjs.org/cgi/reprint/46/4/747.pdf}}</ref>
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| *Rare variant of chondrosarcoma.
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| | |
| Microscopic:
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| Features:
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| *"White clouds in a blue sky".
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| | |
| Image:
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| *[http://moon.ouhsc.edu/kfung/jty1/opaq/PathQuiz/S0A001-PQ01-M.htm Mesenchymal chondrosarcoma (ouhsc.edu)].
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| | |
| =====Myxoid chondrosarcoma=====
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| Microscopic:
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| Features:
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| *[[Chordoma]]-like:
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| **[[Myxoid]] background.
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| **Small cells with eosinophilic cytoplasm.
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| | |
| DDx:
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| *Chondroid [[syringoma]].
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| *Parachordoma.<ref name=pmid10809219>{{cite journal |author=Fisher C |title=Parachordoma exists--but what is it? |journal=Adv Anat Pathol |volume=7 |issue=3 |pages=141–8 |year=2000 |month=May |pmid=10809219 |doi= |url=}}</ref>
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| *[[Chordoma]]. (???)
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| | |
| =====Extraskeletal myxoid chondrosarcoma=====
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| *Originally thought to be a variant of ''myxoid chondrosarcoma of bone''; however, may not be a chondrosarcoma at all.<ref name=pmid14657948>{{Cite journal | last1 = Aigner | first1 = T. | last2 = Oliveira | first2 = AM. | last3 = Nascimento | first3 = AG. | title = Extraskeletal myxoid chondrosarcomas do not show a chondrocytic phenotype. | journal = Mod Pathol | volume = 17 | issue = 2 | pages = 214-21 | month = Feb | year = 2004 | doi = 10.1038/modpathol.3800036 | PMID = 14657948 | URL = http://www.nature.com/modpathol/journal/v17/n2/full/3800036a.html }}</ref>
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| *Characteristic [[chromosomal translocation]]: t(9;22) CHN-EWS.
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| DDx:
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| *Chordoma.<ref name=pmid14657948/>
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| **S-100 +ve (strong).
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| **EMA +ve.
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| Image:
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| *[http://www.cttr.org/large/03113.jpg Extraskeletal myxoid chondrosarcoma (cttr.org)].<ref>URL: [http://www.cttr.org/cms/?p=736 http://www.cttr.org/cms/?p=736]. Accessed on: 1 May 2011.</ref>
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| | |
| =====Dedifferentiated chondrosarcoma=====
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| Clinical:
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| *Abysmal to poor prognosis.
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| **In one series (22 patients) 5-year survival ~20%.<ref>{{Cite journal | last1 = Mitchell | first1 = AD. | last2 = Ayoub | first2 = K. | last3 = Mangham | first3 = DC. | last4 = Grimer | first4 = RJ. | last5 = Carter | first5 = SR. | last6 = Tillman | first6 = RM. | title = Experience in the treatment of dedifferentiated chondrosarcoma. | journal = J Bone Joint Surg Br | volume = 82 | issue = 1 | pages = 55-61 | month = Jan | year = 2000 | doi = | PMID = 10697315 | URL = http://www.jbjs.org.uk/cgi/pmidlookup?view=long&pmid=10697315 }}</ref>
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| **All dead in two years in another series (25 patients).<ref name=pmid17653766/>
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| Features:<ref name=pmid17653766>{{Cite journal | last1 = Sopta | first1 = J. | last2 = Dordević | first2 = A. | last3 = Tulić | first3 = G. | last4 = Mijucić | first4 = V. | title = Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases. | journal = J Cancer Res Clin Oncol | volume = 134 | issue = 2 | pages = 147-52 | month = Feb | year = 2008 | doi = 10.1007/s00432-007-0262-5 | PMID = 17653766 }}</ref>
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| #Poorly differentiated (mesenchymal) malignancy.
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| #Well-differentiated cartilaginous component.
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| Images:
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| *[http://path.upmc.edu/cases/case118/micro.html Dedifferentiated chondrosarcoma (upmc.edu)].
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| | |
| ====Grading====
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| Features:<ref name=Ref_WMSP643>{{Ref WMSP|643}}</ref>
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| *Grade I: mild-to-moderate increase of cellularity +/- binucleated cells.
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| *Grade II: between Grade I and Grade III.
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| *Grade III: nuclear pleomorphism, mitoses common.
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| ===IHC===
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| *S-100 -ve. (???)
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|
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| =Bone= | | =Bone= |
| ==Osteoma== | | ==Osteoma== |
| ===General===
| | {{Main|Osteoma}} |
| *Benign.
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| *May be associated with [[familial adenomatous polyposis]].
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| *Normal bone - unusual location/outgrowth.
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| ===Microscopic===
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| Features:
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| *Normal bone.
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|
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|
| ==Osteoid osteoma== | | ==Osteoid osteoma== |
| *Abbreviated ''OO''.
| | {{Main|Osteoid osteoma}} |
| ===General===
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| *Benign bone lesion.
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| Clinical:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
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| *Extremely painful.
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| **Relieved by [[NSAIDs]].
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| | |
| ===Gross===
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| *Bone: femur > tibia > spine > elsewhere.<ref name=uthscsa>URL: http://radiology.uthscsa.edu/CME/ELTXT/OOT/skeletallocation.html http://radiology.uthscsa.edu/CME/ELTXT/OOT/skeletallocation.html]. Accessed on: 7 May 2012.</ref><ref name=radiologyassistant>URL: [http://www.radiologyassistant.nl/en/494e15cbf0d8d http://www.radiologyassistant.nl/en/494e15cbf0d8d]. Accessed on: 7 May 2012.</ref>
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| *Most common location (in bone): diaphysis.<ref name=uthscsa>URL: http://radiology.uthscsa.edu/CME/ELTXT/OOT/skeletallocation.html http://radiology.uthscsa.edu/CME/ELTXT/OOT/skeletallocation.html]. Accessed on: 7 May 2012.</ref>
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| Images:
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| *[http://njms2.umdnj.edu/tutorweb/casegifs/ostostgross.jpg Osteoid osteoma - gross (umdnj.edu)].<ref>URL: [http://njms2.umdnj.edu/tutorweb/gross.htm http://njms2.umdnj.edu/tutorweb/gross.htm]. Accessed on: 7 May 2012.</ref>
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| *[http://radiology.uthscsa.edu/CME/ELTXT/OOT/treatment.html Osteoid osteoma (uthscsa.edu)].
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| | |
| ===Microscopic===
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| Features:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
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| *Anastomosing bony [[trabeculae]] with:
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| **Variable mineralization.
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| ***Mineralization (calcium '''p'''hosphate) = '''p'''urple on [[H&E stain]].
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| **Osteoblasts rimming.
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| ***Cells line-up at edge of bone.
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| | |
| Note:
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| *Histomorphologically near identical/indistinguishable from ''[[osteoblastoma]]'';<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref> one needs some history to make the diagnosis.
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| | |
| ====Images====
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| <gallery>
| |
| Image:Osteoid_osteoma_-_low_mag.jpg | Osteoid osteoma - low mag. (WC)
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| Image:Osteoid osteoma - intermed mag.jpg | Osteoid osteoma - intermed. mag. (WC)
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| Image:Osteoid_osteoma_-_high_mag.jpg | Osteoid osteoma - high mag. (WC)
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| Image:Osteoid osteoma - very high mag.jpg | Osteoid osteoma - very high mag. (WC)
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| </gallery>
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| www:
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| *[http://library.med.utah.edu/WebPath/COW/COW211.html Osteoid osteoma - CT scan (med.utah.edu)].
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| *[http://www.sciencephoto.com/images/imagePopUpDetails.html?pop=1&id=700030210&pviewid=&country=67&search=gschmeissners&matchtype=FUZZY Osteoid osteoma (sciencephoto.com)].
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| | |
| ===Sign out===
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| <pre>
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| BONE, RIGHT FEMUR, EXCISION:
| |
| - OSTEOID OSTEOMA.
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| </pre>
| |
| | |
| ====Micro====
| |
| The sections show anastomosing bony trabeculae with variable mineralization and osteoblastic rimming. Multinucleated osteoclasts are scattered through the lesion. Hemosiderin-laden macrophages are present. No osteocyte nuclear atypia is apparent. Mitotic activity is not apparent. The osteoid is not lace-like.
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|
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|
| ==Osteoblastoma== | | ==Osteoblastoma== |
| ===General===
| | {{Main|Osteoblastoma}} |
| *Benign bone tumour.
| |
| | |
| ===Microscopic===
| |
| Features:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
| |
| *Anastomosing bony [[trabeculae]] with:
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| **Osteoblasts rimming.
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| ***Cells line-up at edge of bone.
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|
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|
| Notes:
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| *Histomorphologically near identical/indistinguishable from ''[[osteoid osteoma]]''.<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref>
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| *'''Must''' be greater 1.5 cm by definition.<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref>
| |
|
| |
| ====Images====
| |
| <gallery>
| |
| Image:Osteoblastoma_-_high_mag.jpg | Osteoblastoma - high mag. (WC)
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| Image:Osteoblastoma_-_low_mag.jpg | Osteoblastoma - low mag. (WC)
| |
| </gallery>
| |
| ==Ewing sarcoma== | | ==Ewing sarcoma== |
| *[[AKA]] EWS/pPNET, AKA (confusingly) EWS/PNET:
| | {{Main|Ewing sarcoma}} |
| **EWS = Ewing sarcoma.
| |
| **pPNET = peripheral primitive neuroectodermal tumour.
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| *EWS and pPNET were once thought to be different tumours.
| |
| | |
| Note:
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| *''Peripheral primitive neuroectodermal tumour'' should not be confused with ''[[primitive neuroectodermal tumour]]'', commonly abbreviated ''PNET'', a (supertentorial) brain tumour with similarities to [[medulloblastoma]].
| |
| | |
| ===General===
| |
| Clinical:
| |
| *Painful.
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| *Usually younger than 20 years.
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| *Second most common malignant bone tumour in children.
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| **Most common malignant bone tumour = osteosarcoma (AKA osteogenic sarcoma).
| |
| | |
| Poor prognostic factors:<ref>PST. 14 February 2011.</ref>
| |
| *Age (18 years-old+).
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| *Pelvis (extremity = good).
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| *>8 cm.
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| *Metastases.
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| *EWS-FL1 fusion type 2.
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| *>90% necrosis.
| |
| | |
| Etiology:
| |
| *Unknown origin; hypothesis: Ewing sarcoma arises from mesenchymal stem cell.<ref name=pmid20953407>{{cite journal |author=Lin PP, Wang Y, Lozano G |title=Mesenchymal Stem Cells and the Origin of Ewing's Sarcoma |journal=Sarcoma |volume=2011 |issue= |pages= |year=2011 |pmid=20953407 |pmc=2952797 |doi=10.1155/2011/276463 |url=}}</ref>
| |
| | |
| ====Radiology====
| |
| Features:<ref name=Ref_WMSP650>{{Ref WMSP|650}}</ref>
| |
| *Long bones, diaphyses.
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| *Destructive.
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| *"Onion-skin" periosteal reaction.
| |
| | |
| ===Microscopic===
| |
| Features:<ref>PST. 22 February 2010.</ref>
| |
| *Scant clear cytoplasm (contain glycogen -- PAS +ve, PAS-D -ve) - '''key feature'''.
| |
| *Round small nucleus.
| |
| **Usu. lack nucleoli.
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| **Usu. minimal-moderate size variation.
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| *Mitoses (common).
| |
| | |
| Notes:
| |
| *It is a [[small round cell tumour]].
| |
| *[[Rhabdomyosarcoma]] occasionally has cytoplasmic clearing (due to glycogen).<ref name=PST14feb11>PST. 14 February 2011.</ref>
| |
| | |
| ====Images====
| |
| <gallery>
| |
| Image:Ewing_sarcoma_-_very_high_mag.jpg | ES - very high mag. (WC)
| |
| Image:Ewing_sarcoma_-_PAS_-_very_high_mag.jpg | ES - PAS - very high mag. (WC)
| |
| </gallery>
| |
| www:
| |
| *[http://path.upmc.edu/cases/case103.html Ewing sarcoma - case 1 - several images (upmc.edu)].
| |
| *[http://path.upmc.edu/cases/case420.html Ewing sarcoma - case 2 - several images (upmc.edu)].
| |
| | |
| ===[[IHC]]===
| |
| Features:<ref name=Ref_WMSP651>{{Ref WMSP|651}}</ref>
| |
| *CD99 +ve -- 1. diffuse, 2. plasma membrane staining; both required -- most specific.
| |
| *FLI-1 +ve.<ref name=pmid15001993>{{cite journal |author=Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos AP |title=Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody |journal=Mod. Pathol. |volume=17 |issue=5 |pages=547–52 |year=2004 |month=May |pmid=15001993 |doi=10.1038/modpathol.3800065 |url=http://www.nature.com/modpathol/journal/v17/n5/full/3800065a.html}}</ref>
| |
| *CD45 -ve.
| |
| **Done to r/o [[lymphoma]].
| |
| *+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S100).
| |
| *+/-Cytokeratins.
| |
| *Caveolin-1 +ve in ~ 85% of EWS.<ref>PST. 14 February 2011.</ref>
| |
| *WT-1 -ve.<ref name=Ref_WMSP286>{{Ref WMSP|286}}</ref>
| |
| | |
| Notes:<ref>PST. 22 February 2010.</ref>
| |
| *CD99 +ve
| |
| **Plasma membrane staining tumours:
| |
| ***[[Lymphoblastic lymphoma]]/leukemia.
| |
| ***[[Angiomatoid fibrous histiocytoma]].
| |
| ***[[Desmoplastic small round cell tumour]].
| |
| **Weak/cytoplasmic staining:
| |
| ***[[Synovial sarcoma]].
| |
| ***[[Rhabdomyosarcoma]].
| |
| ***[[Rhabdoid tumour]].
| |
| *FLI-1 +ve:<ref name=pmid15001993/>
| |
| **[[Vascular neoplasms]].
| |
| **-/+[[Merkel cell carcinoma]].
| |
| **-/+[[Melanoma]].
| |
| | |
| ===Molecular diagnostics===
| |
| Common features:
| |
| *''EWS/FLI-1 fusion gene'' formation due to [[translocation]]: ''t(11;22)(q24;q12)''.<ref>URL: [http://atlasgeneticsoncology.org/Tumors/Ewing5010.html http://atlasgeneticsoncology.org/Tumors/Ewing5010.html]. Accessed on: 23 February 2010.</ref><ref name=pmid3163261>{{cite journal |author=Turc-Carel C, Aurias A, Mugneret F, ''et al.'' |title=Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12) |journal=Cancer Genet. Cytogenet. |volume=32 |issue=2 |pages=229–38 |year=1988 |month=June |pmid=3163261 |doi= |url=}}</ref>
| |
| **Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers).
| |
| **Type 1 = EWS exon 7 + FLI-1 exon 6; good prognosis.
| |
| **Type 2 = others; poor prognosis.
| |
| | |
| Notes:
| |
| *The ''t(11;22)(q24;q12)'' is seen in ~90% of EWS/PNET... but also in:
| |
| **[[Olfactory neuroblastoma]].
| |
| **Small cell osteogenic sarcoma (small cell variant of [[osteosarcoma]]).
| |
| **Polyphenotypic tumours.
| |
| **[[Rhabdomyosarcoma]].
| |
| **[[Neuroblastoma]] (possibly).
| |
| *Several other EWS translocations exist.<ref>URL: [http://www.cancerindex.org/geneweb/EWSR1.htm http://www.cancerindex.org/geneweb/EWSR1.htm]. Accessed on: 20 November 2011.</ref>
| |
| **ERG,<ref name=omim165080>{{OMIM|165080}}</ref> ETV1, E1AF and FEV.
| |
| *Lack of molecular findings does ''not'' exclude Ewing sarcoma.
| |
| *Testing:
| |
| **A break apart probe for EWS is a common way to look for pathologic change, as it covers almost all variants.
| |
| | |
| ===Electron microscopy===
| |
| *Primitive cell junctions.
| |
| *Clear zone (glycogen lakes).
| |
|
| |
|
| ==Osteosarcoma== | | ==Osteosarcoma== |
Line 697: |
Line 337: |
|
| |
|
| ==Giant cell tumour of bone== | | ==Giant cell tumour of bone== |
| ===General===
| | {{Main|Giant cell tumour of bone}} |
| Features:<ref name=Ref_WMSP648>{{Ref WMSP|648}}</ref>
| |
| *Approximately 5% of primary bone tumours.
| |
| *Typical age: 20-45 years.
| |
| | |
| ====Clinical====
| |
| *Location: growth plate of long bones.<ref name=pmid11501745>{{Cite journal | last1 = Wülling | first1 = M. | last2 = Engels | first2 = C. | last3 = Jesse | first3 = N. | last4 = Werner | first4 = M. | last5 = Delling | first5 = G. | last6 = Kaiser | first6 = E. | title = The nature of giant cell tumor of bone. | journal = J Cancer Res Clin Oncol | volume = 127 | issue = 8 | pages = 467-74 | month = Aug | year = 2001 | doi = | PMID = 11501745 }}</ref>
| |
| **May present with joint pain, immobility.
| |
| | |
| Note:
| |
| *Several types of [[giant cell lesions|giant cell tumours]] exist.
| |
| | |
| ===Microscopic===
| |
| Features:<ref name=Ref_Klatt420>{{Ref Klatt|420}}</ref>
| |
| *Giant cells with a large number of nuclei (usu. >10 in the plane of section).
| |
| **Usu. have prominent nucleoli.
| |
| *Mononuclear cells and small multinucleated cells with nuclei similar to those in the giant cells - '''key feature'''.
| |
| *+/-Hemosiderin deposition - not common.<ref name=pmid1939753>{{Cite journal | last1 = Aoki | first1 = J. | last2 = Moriya | first2 = K. | last3 = Yamashita | first3 = K. | last4 = Fujioka | first4 = F. | last5 = Ishii | first5 = K. | last6 = Karakida | first6 = O. | last7 = Imai | first7 = S. | last8 = Sakai | first8 = F. | last9 = Imai | first9 = Y. | title = Giant cell tumors of bone containing large amounts of hemosiderin: MR-pathologic correlation. | journal = J Comput Assist Tomogr | volume = 15 | issue = 6 | pages = 1024-7 | month = | year = | doi = | PMID = 1939753 }}</ref><ref name=pmid18554912>{{Cite journal | last1 = Matsushige | first1 = T. | last2 = Nakaoka | first2 = M. | last3 = Yahara | first3 = K. | last4 = Kagawa | first4 = K. | last5 = Miura | first5 = H. | last6 = Ohnuma | first6 = H. | last7 = Kurisu | first7 = K. | title = Giant cell tumor of the temporal bone with intratumoral hemorrhage. | journal = J Clin Neurosci | volume = 15 | issue = 8 | pages = 923-7 | month = Aug | year = 2008 | doi = 10.1016/j.jocn.2007.03.013 | PMID = 18554912 }}</ref>
| |
| | |
| Notes:
| |
| *Giant cells typically present in abundance.
| |
| | |
| DDx:
| |
| *[[Giant cell lesions]].
| |
| **[[Aneurysmal bone cyst]] - typically has spindle cells around the giant cells.
| |
| | |
| ====Images====
| |
| <gallery>
| |
| Image:Giant_cell_tumour_of_bone_-_high_mag.jpg | GCT of bone - high mag. (WC)
| |
| Image:Giant_cell_tumour_of_bone_-_low_mag.jpg | GCT of bone - low mag. (WC)
| |
| </gallery>
| |
| ===IHC===
| |
| *p63 +ve in scattered mononuclear cells.<ref name=pmid18311114>{{cite journal |author=Dickson BC, Li SQ, Wunder JS, ''et al.'' |title=Giant cell tumor of bone express p63 |journal=Mod. Pathol. |volume=21 |issue=4 |pages=369–75 |year=2008 |month=April |pmid=18311114 |doi=10.1038/modpathol.2008.29 |url=}}</ref>
| |
| **This seems to be contradicted by another paper.<ref name=pmid20012988>{{cite journal |author=Alberghini M, Kliskey K, Krenacs T, ''et al.'' |title=Morphological and immunophenotypic features of primary and metastatic giant cell tumour of bone |journal=Virchows Arch. |volume=456 |issue=1 |pages=97–103 |year=2010 |month=January |pmid=20012988 |doi=10.1007/s00428-009-0863-2 |url=}}</ref>
| |
|
| |
|
| =Other= | | =Other= |
| This section collects stuff that doesn't neatly fit into the ''bone'' or ''cartilage'' category. | | This section collects stuff that doesn't neatly fit into the ''bone'' or ''cartilage'' category. |
| ==Osteochondroma==
| |
| ===General===
| |
| *Benign.
| |
| *Very common.
| |
| *Abnormal outgrowth of bone and cartilage - associated with growth plate.
| |
| *Usually present before age 20.<ref name=pmid12873205>{{Cite journal | last1 = Parkin | first1 = B. | last2 = Bennett | first2 = JC. | last3 = Zaher | first3 = A. | title = Pathologic quiz case: an iliac mass in a 25-year-old woman. Osteochondroma. | journal = Arch Pathol Lab Med | volume = 127 | issue = 8 | pages = e355-6 | month = Aug | year = 2003 | doi = 10.1043/1543-2165(2003)127e355:PQCAIM2.0.CO;2 | PMID = 12873205 | URL = http://www.archivesofpathology.org/doi/full/10.1043/1543-2165%282003%29127%3Ce355:PQCAIM%3E2.0.CO;2}}</ref>
| |
|
| |
| ===Gross===
| |
| Features:
| |
| *Metasphyseal lesions.<ref name=medpath>URL: [http://www.medpath.info/MainContent/Skeletal/Bone_07.html http://www.medpath.info/MainContent/Skeletal/Bone_07.html]. Accessed on: 18 September 2012.</ref>
| |
| **Often distal femur, proximal humerus or proximal tibia.<ref name=pmid12873205/>
| |
| **Occasionally iliac mass.<ref name=pmid22217414>{{Cite journal | last1 = Kokavec | first1 = M. | last2 = Gajdoš | first2 = M. | last3 = Džupa | first3 = V. | title = [Osteochondroma of the iliac crest: case report]. | journal = Acta Chir Orthop Traumatol Cech | volume = 78 | issue = 6 | pages = 583-5 | month = | year = 2011 | doi = | PMID = 22217414 }}
| |
| </ref>
| |
| *Mushroom-like shape - with broad or narrow base.
| |
|
| |
|
| Note:
| | ==Notochordal tumors== |
| *Very unusual in craniofacial bones.<ref name=pmid12873205/>
| | Notochordal tumors arise from notochordal remnants and thus are seen in the clivus or sacrum. |
| | {{Main|Chordoma}} |
|
| |
|
| ===Microscopic=== | | ==Osteochondroma== |
| Features:<ref name=pmid12873205/>
| | {{Main|Osteochondroma}} |
| *Bone - forms base of "mushroom".
| |
| *Cartilage - layer usu. thinner in older individuals.
| |
| **Lobular arrangement<ref name=upmc_case341>URL: [http://path.upmc.edu/cases/case341/dx.html http://path.upmc.edu/cases/case341/dx.html]. Accessed on: 19 September 2012.</ref> - clusters of 3-12 lacunae (separated by stroma).
| |
| **May have mild atypia.
| |
| **Nuclear enlargement - up to 5x normal.
| |
| *[[Perichondrium]] - covers the cartilage.
| |
| **Thin layer of cells - higher cellular density than cartilage.
| |
| ***Bland spindle cells in a fibrous (eosinophilic) stroma.
| |
| | |
| Notes:
| |
| *Benign cartilage - one chondrocyte per lacuna, small and round nucleus, no binucleation.
| |
| | |
| DDx:
| |
| *[[Chondrosarcoma]] - esp. in older individuals.
| |
| | |
| Images:
| |
| *[http://path.upmc.edu/cases/case341.html Super rare intracranial mesenchymal osteochondroma - several images (upmc.edu)].<ref name=upmc_case341/>
| |
|
| |
|
| ==Diffuse tenosynovial giant-cell tumour== | | ==Diffuse tenosynovial giant-cell tumour== |
| *[[AKA]] ''tenosynovial giant-cell tumour, diffuse type''. | | *[[AKA]] ''tenosynovial giant-cell tumour, diffuse type''. |
| *Previously known as ''pigmented villonodular synovitis'' (PVNS).<ref>{{Ref PBoD8|1247}}</ref> | | *Previously known as ''pigmented villonodular synovitis'' (PVNS).<ref>{{Ref PBoD8|1247}}</ref> |
| | | {{Main|Diffuse tenosynovial giant-cell tumour}} |
| ===General===
| |
| *Course: benign.
| |
| *''Giant cell tumor of the tendon sheath'' is considered to be the soft-tissue counterpart of PVNS.<ref>URL: [http://emedicine.medscape.com/article/1253223-overview http://emedicine.medscape.com/article/1253223-overview]. Accessed on: 6 January 2011.</ref>
| |
| *Typically knee or hip.<ref name=pmid10524485>{{Cite journal | last1 = Frassica | first1 = FJ. | last2 = Bhimani | first2 = MA. | last3 = McCarthy | first3 = EF. | last4 = Wenz | first4 = J. | title = Pigmented villonodular synovitis of the hip and knee. | journal = Am Fam Physician | volume = 60 | issue = 5 | pages = 1404-10; discussion 1415 | month = Oct | year = 1999 | doi = | PMID = 10524485 | URL = http://www.aafp.org/afp/1999/1001/p1404.html }}</ref>
| |
| | |
| ===Microscopic===
| |
| Features:<ref>URL: [http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis].</ref>
| |
| *Subsynovial nodules composed of cells with:
| |
| **Abundant cytoplasm.
| |
| **Pale nuclei.
| |
| *Multinucleated giant cells.
| |
| *Hemosiderin-laden macrophages.
| |
| *Foam cells.
| |
| | |
| ====Images====
| |
| <gallery>
| |
| Image:Pigmented_villonodular_synovitis_low_mag.jpg | PVNS - low mag. (WC)
| |
| Image:Pigmented_villonodular_synovitis_high_mag.jpg | PVNS - high mag. (WC)
| |
| </gallery>
| |
| www:
| |
| *[http://path.upmc.edu/cases/case251/micro.html PVNS - several images (upmc.edu)].
| |
| *[http://www.webpathology.com/image.asp?case=357&n=2 PVNS (webpathology.com)].
| |
| ===Sign out===
| |
| <pre>
| |
| RIGHT FEMORAL HEAD AND JOINT CAPSULE, EXCISION:
| |
| - DEGENERATIVE JOINT DISEASE.
| |
| - DIFFUSE TENOSYNOVIAL GIANT-CELL TUMOUR (PIGMENTED VILLONODULAR SYNOVITIS).
| |
| </pre>
| |
| | |
| ====Micro====
| |
| The soft tissue sections show nodules with abundant hemosiderin-laden macrophages and multinucleated giant cells. Nuclear atypia is not identified. Mitotic activity is not apparent.
| |
|
| |
|
| ==Giant cell tumour of tendon sheath== | | ==Giant cell tumour of tendon sheath== |
| *Abbreviated ''GCT of tendon sheath''. | | *Abbreviated ''GCT of tendon sheath''. |
| ===General===
| | {{Main|Giant cell tumour of tendon sheath}} |
| *Can be thought of as the small joint version of [[diffuse tenosynovial giant-cell tumour]] ([[AKA]] ''PVNS'').<ref name=Ref_DCHH341>{{Ref DCHH|341}}</ref>
| |
| *Rarely recur.
| |
| *Classically afflicts the hand.<ref name=Ref_WMSP612>{{Ref WMSP|612}}</ref>
| |
| *Rarely malignant.<ref name=pmid19094761>{{Cite journal | last1 = Pan | first1 = YW. | last2 = Huang | first2 = XY. | last3 = You | first3 = JF. | last4 = Tian | first4 = GL. | last5 = Li | first5 = C. | title = [Malignant giant cell tumor of the tendon sheaths in the hand]. | journal = Zhonghua Wai Ke Za Zhi | volume = 46 | issue = 21 | pages = 1645-8 | month = Nov | year = 2008 | doi = | PMID = 19094761 }}</ref><ref name=pmid8230758>{{Cite journal | last1 = Shinjo | first1 = K. | last2 = Miyake | first2 = N. | last3 = Takahashi | first3 = Y. | title = Malignant giant cell tumor of the tendon sheath: an autopsy report and review of the literature. | journal = Jpn J Clin Oncol | volume = 23 | issue = 5 | pages = 317-24 | month = Oct | year = 1993 | doi = | PMID = 8230758 }}</ref>
| |
| | |
| ===Gross===
| |
| Features:<ref name=Ref_WMSP612>{{Ref WMSP|612}}</ref>
| |
| *Circumscribed mass - yellow-brown to tan.
| |
| | |
| Note:
| |
| *May be associated with bony erosions in larger lesions.<ref name=Ref_WMSP612>{{Ref WMSP|612}}</ref>
| |
| | |
| Image:
| |
| *[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3122708/figure/Fig2/ GCT of tendon sheath (nih.gov)].<ref name=pmid22282671>{{Cite journal | last1 = Suresh | first1 = SS. | last2 = Zaki | first2 = H. | title = Giant cell tumor of tendon sheath: case series and review of literature. | journal = J Hand Microsurg | volume = 2 | issue = 2 | pages = 67-71 | month = Dec | year = 2010 | doi = 10.1007/s12593-010-0020-9 | PMID = 22282671 }}</ref>
| |
| | |
| ===Microscopic===
| |
| Features:<ref name=Ref_DCHH341>{{Ref DCHH|341-2}}</ref>
| |
| *Foam cells.
| |
| **Cells with moderate to abundant foamy-appearing cytoplasm.
| |
| *Multinucleated giant cells - may be scarce.
| |
| *+/-Tendon.
| |
| **Dense connective tissue.
| |
| *+/-Hemosiderin-laden macrophages.
| |
| | |
| Note:
| |
| *Features of malignancy: nuclear pleomorphism,<ref name=pmid8230758>{{Cite journal | last1 = Shinjo | first1 = K. | last2 = Miyake | first2 = N. | last3 = Takahashi | first3 = Y. | title = Malignant giant cell tumor of the tendon sheath: an autopsy report and review of the literature. | journal = Jpn J Clin Oncol | volume = 23 | issue = 5 | pages = 317-24 | month = Oct | year = 1993 | doi = | PMID = 8230758 }}</ref> abnormal mitoses, >10 mitoses/[[HPF]], tumour necrosis lack of maturation to superficial part (nuclei shrink, cytoplasm lipid-ified).<ref name=Ref_DCHH341>{{Ref DCHH|341-2}}</ref>
| |
| | |
| DDx:
| |
| *[[Giant cell lesions]].
| |
| | |
| ====Images====
| |
| <gallery>
| |
| Image:Giant_cell_tumor_of_tendon_sheath_histopathology%281%29.jpg | GCT of tendon sheath. (WC/KGH)
| |
| </gallery>
| |
| www:
| |
| *[http://www.webpathology.com/image.asp?n=1&Case=484 GCT of tendon sheath - very low mag. (webpathology.com)]
| |
| *[http://www.webpathology.com/image.asp?case=484&n=5 GCT of tendon sheath - low mag. (webpathology.com)].
| |
| *[http://www.webpathology.com/image.asp?n=4&Case=484 GCT of tendon sheath - high mag. (webpathology.com)].
| |
| | |
| ===Sign out===
| |
| <pre>
| |
| LESION, RIGHT INDEX FINGER, EXCISION:
| |
| - GIANT CELL TUMOUR OF THE TENDON SHEATH.
| |
| </pre>
| |
| | |
| ====Micro====
| |
| The sections show histiocytes and rare multinucleated giant cells on a background of dense connective tissue compatible with tendon. No nuclear atypia is apparent. Rare mitotic activity is identified. No atypical mitoses are apparent.
| |
|
| |
|
| ==Adamantinoma== | | ==Adamantinoma== |
| :Should '''not''' be confused with ''[[adenomatoid tumour]]''.
| | {{Main|Adamantinoma}} |
| ===General===
| |
| Features:<ref name=Ref_WMSP650>{{Ref WMSP|650}}</ref>
| |
| *Rare: < 1% of bone tumours.
| |
| *25-35 years old.
| |
| *Tibia, fibula.
| |
| *Benign, may be locally aggressive.
| |
| *Cousin of [[ameloblastoma]]. (???)
| |
| | |
| ===Radiology===
| |
| *Intracortical, radiolucent.
| |
| | |
| ===Microscopic===
| |
| Features:
| |
| *Biphasic tumour:
| |
| *#Fibrous/spindle cell component.
| |
| *#Epithelial component.
| |
| | |
| DDx:<ref name=pathcon_adam>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970057-2 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970057-2]. Accessed on: 28 April 2011.</ref>
| |
| *Vascular tumours ([[Epithelioid hemangioendothelioma]]).
| |
| *Metastatic carcinoma.
| |
| | |
| ====Images====
| |
| <gallery>
| |
| Image:Adamantinoma_-_intermed_mag.jpg | Adamantinoma - intermed. mag. (WC)
| |
| </gallery>
| |
| www:
| |
| *[http://southbaypath.org/CaseImages/sb5260/AdamantinomaBiopsy3.jpg Adamantinoma (southbaypath.org)].<ref>URL: [http://southbaypath.org/CaseImages/sb5260/sb5260.htm http://southbaypath.org/CaseImages/sb5260/sb5260.htm]. Accessed on: 7 December 2010.</ref>
| |
| | |
| ===IHC===
| |
| Features:<ref name=pathcon_adam/>
| |
| *CK14 +ve (HMWK).<ref>URL: [http://www.nordiqc.org/Epitopes/Cytokeratins/cytokeratins.htm http://www.nordiqc.org/Epitopes/Cytokeratins/cytokeratins.htm]. Accessed on: 28 April 2011.</ref>
| |
| *CK19 +ve (LMWK).
| |
| *CK8/18 -ve (LMWK).
| |
|
| |
|
| ==Brown tumour== | | ==Brown tumour== |
Line 946: |
Line 412: |
| *[http://www.radiologyassistant.nl/en/494e15cbf0d8d Bone radiology (radiologyassistant.nl)]. | | *[http://www.radiologyassistant.nl/en/494e15cbf0d8d Bone radiology (radiologyassistant.nl)]. |
|
| |
|
| | [[Category:Chondro-osseous tumours]] |
| [[Category:Weird stuff]] | | [[Category:Weird stuff]] |