Difference between revisions of "Chondro-osseous tumours"

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'''Bone tumours''' occasionally cross the desk of the pathologist.  Primary bone tumours are rare; the most common bone tumour is metastases.<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref> 
[[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]].  


Bone tumours occasionally are lumped with soft tissue tumours.  Soft tissue tumours are dealt with in the ''[[soft tissue lesions]]'' article.  An introduction to bone is found in the ''[[bone]]'' article.
Primary bone tumours are rare; the most common bone tumour is [[metastases]].<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref> 
 
Bone tumours occasionally are lumped with soft tissue tumours.  Soft tissue tumours are dealt with in the ''[[soft tissue lesions]]'' article.  An introduction to bone is found in the ''[[bone]]'' article.  An introduction to cartilage is found in the ''[[cartilage]]'' article.


==General==
==General==
*Diagnosis should not be made without radiologic & clinical information.
*Diagnosis of a primary bone tumour should not be made without radiologic & clinical information!
*Metastasis:primary bone tumours = >20:1.<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref>
*Metastasis:primary bone tumours = >20:1.<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref>


===Common malignant===
===Common malignant===
*Osteosarcoma.
*[[Osteosarcoma]].
*Chondrosarcoma.
*[[Chondrosarcoma]].
*Ewing's sarcoma.
*[[Ewing's sarcoma]].
*Multiple myeloma.
*[[Multiple myeloma]].
*Metastases.
*[[Metastases]].
**Most common tumours metastatic to bone (mnemonic: ''BLT with Ketchup & Pickles''):  
**Most common tumours metastatic to bone (mnemonic: ''BLT with Ketchup & Pickles''):  
***[[Breast]].  
***[[Breast]].  
Line 20: Line 23:
***[[Prostate gland]].  
***[[Prostate gland]].  


Epidemiology:<ref>TN05 OR42.</ref>
Epidemiology:<ref name=Ref_TN2005_OR42>{{Ref TN2005 |OR42}}</ref>
*Osteosarcoma -> 2nd decade.  
*Osteosarcoma -> 2nd decade.  
*Ewing's ->5-20 yrs.
*Ewing's ->5-20 yrs.
*Chondrosarcoma -> from enchondroma or osteochrondroma -- patients over 40 yrs.
*Chondrosarcoma -> from enchondroma or osteochrondroma -- patients over 40 yrs.
*Multiple myeloma -> most common primary bone tumour in adults.
*[[Multiple myeloma]] -> most common primary bone tumour in adults.


===Malignant bone tumours by age===
===Malignant bone tumours by age===
Most common by age:<ref>TN05 OR42.</ref>
Most common by age:<ref name=Ref_TN2005_OR42>{{Ref TN2005 |OR42}}</ref>
*<1 year old - neuroblastoma.
*<1 year old - [[neuroblastoma]].
*1-10 years old - Ewing's of tubular bones.
*1-10 years old - [[Ewing sarcoma|Ewing's]] of tubular bones.
*10-30 years old - osteosarcoma, Ewing's of flat bones.  
*10-30 years old - osteosarcoma, Ewing's of flat bones.  
*30-40 years old - reticulum cell sarcoma, fibrosarcoma, parosteal osteosarcoma, malignant giant cell tumour, lymphoma.
*30-40 years old - [[reticulum cell sarcoma]], [[fibrosarcoma]], parosteal osteosarcoma, [[malignant giant cell tumour]], [[lymphoma]].
*>40 years old - mets, multiple myeloma, chondrosarcoma.
*>40 years old - mets, [[multiple myeloma]], [[chondrosarcoma]].


===Benign aggressive bone tumours===
===Benign aggressive bone tumours===
*Giant cell tumours.
*[[Giant cell tumour of bone]].
*Osteoblastoma.
*[[Osteoblastoma]].
**Thought to be related to osteoid osteoma.
**Thought to be related to [[osteoid osteoma]].
**If in long bones often diaphyseal.
**If in long bones often diaphyseal.
Ref.:<ref>TN05 OR41.</ref><ref>URL: [http://www.emedicine.com/RADIO/topic494.htm http://www.emedicine.com/RADIO/topic494.htm].</ref>


=Cartilage=
Ref.:<ref name=Ref_TN2005_OR41>{{Ref TN2005 |OR41}}</ref><ref>URL: [http://www.emedicine.com/RADIO/topic494.htm http://www.emedicine.com/RADIO/topic494.htm].</ref>
==Enchondroma==
===General===
===Summary tables===
*Benign thingy.
====Bone tumours====
*Usu. legs and feet.
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
*May be difficult to separate from chondrosarcoma.
! Entity
*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>
! Key feature
! Other features
! Radiology / gross
! Clinical
! Stains / other
! Image
|-
| [[Osteoma]]
| normal bone (???)
| other features (???)
| radiology / gross (???)
| ?
| no stains / may be assoc. with [[FAP]]
| [[Image:Osteoma -- intermed mag.jpg |thumb|center|150px| Osteoma. (WC)]]
|-
| [[Osteoid osteoma]]
| osteoblastic rimming
| anastomosing bony trabeculae
| 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
| IHC / other
| [[Image:Osteoid_osteoma_-_high_mag.jpg |thumb|center|150px| Osteoid osteoma. (WC)]]
|-
| [[Osteoblastoma]]
| osteoblastic rimming
| anastomosing bony trabeculae
| must be >1 cm,<ref name=pmid25224389/> often >=2 cm, metaphysis
| not painful
| IHC / other
| [[Image:Osteoblastoma_-_high_mag.jpg|thumb|center|150px|Osteoblastoma. (WC)]]
|-
| [[Ewing sarcoma]]
| [[small round blue cell tumour]]
| cytoplasmic clearing (due to glycogen)
| usu. diaphysis
| pediatric, typically 1-10 years
| PAS+, PASD-, [[chromosomal translocations]] (usually t(11;22)(q24;q12)) 
| [[Image:Ewing_sarcoma_-_PAS_-_high_mag.jpg |thumb|center|150px| Ewing sarcoma. [[PAS stain]]. (WC)]]
|-
| [[Osteosarcoma]]
| osteoid
| +/-hemorrhage, +/-cartilage
| distal femur, prox. tibia, prox. humerous
| typically 10-30 years, pain, swelling
| no stains; many subtypes
| [[Image:Osteosarcoma_-_very_high_mag.jpg |thumb|center|150px|Osteosarcoma. (WC)]]
|-
| [[Giant cell tumour of bone]]
| abundant giant cells
| nuclei of surrounding cells similar to those in giant cells
| growth plate of long bones
| 20-45 years old, +/-joint pain, +/-immobility
| IHC / other
| [[Image:Giant_cell_tumour_of_bone_-_high_mag.jpg|thumb|center|150px|Giant cell tumour. (WC)]]
|-
|}
 
====Cartilage tumours====
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Entity
! Key feature
! Other features
! Radiology / gross
! Clinical
! Stains / other
! Image
|-
| [[Chondroma]]
| ctyologically benign cells
| equally spaced nests
| usu. diaphysis
| benign / DDx: chondroma, well-diff. chondrosarcoma
| IHC / bone marrow cavity chondroma = ''enchondroma''
| [[Image:Enchondroma_-_very_high_mag.jpg |thumb|center|150px| Enchondroma. (WC)]]
|-
| [[Chondroblastoma]]
| abundant extracellular material, abundant eosinophilic cytoplasm
| calcifications surround cells nests ("chickenwire" appearance) - '''classic'''
| epiphysis
| DDx: [[giant cell tumour of bone]]  
| S100+ve, vimentin +ve
| [[Image:Chondroblastoma_-_very_high_mag.jpg |thumb|center|150px| Chondroblastoma. (WC)]]
|-
| [[Chondrosarcoma]]
| cartilaginous appearance +/- nuclear atypia
| lack osteoid, if present -> osteosarcoma
| usu. diaphysis, classically hip; almost never distal extremity
| >40 years old
| IHC / may be histologically benign looking
| [[Image:Chondrosarcoma_%282%29.jpg |thumb|center|150px|Chondrosarcoma. (WC)]]
|-
|}


Radiology:<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>
====Other====
*Lytic lesion.
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
*Usu. close to a growth plate.
! Entity
! Key feature
! Other features
! Radiology / gross
! Clinical
! Stains / other
! Image
|-
| [[Osteochondroma]]
| benign bone and cartilage
| Other features
| metaphyseal lesions
| Clinical
| IHC / other
| Image
|-
| [[Adamantinoma]]
| bisphasic - stroma & epithelium
| Other features
| tibia, fibula, intracortical, radiolucent
| Clinical
| IHC / other
| [[Image:Adamantinoma_-_intermed_mag.jpg |thumb|center|150px|Adamantinoma. (WC)]]
|-
| [[Diffuse tenosynovial giant-cell tumour]] ([[AKA]] [[PVNS]])
| pigmented giant cells
| nodules
| Radiology / gross
| Clinical
| IHC / other
| [[Image:Pigmented_villonodular_synovitis_low_mag.jpg |thumb|center|150px| PVNS. (WC)]]
|-
| [[Brown tumour]]
| fibrosis, +/-giant cells
| unaffected bone incr. osteoblasts and osteoclasts
| Radiology / gross
| due to hypercalcemia; not a neoplasm
| IHC / other
| [[Image:Brown_tumour_-_low_mag.jpg |thumb|center|150px| Brown tumour. (WC)]]
|-
|}


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>
====Radiology====
*Pain.
=====Radiologic features=====
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! Features
! Benign
! Malignant
|-
| Bone changes
| sclerotic rim
| tumour perforation
|-
| Circumscription
| pushing margins
| ill-defined/moth-eaten
|-
| Soft tissue involvement
| no
| common
|-
| Periosteal reaction
| no
| "hair-on-end" or "sunburst",<br> "onion skin", Codman's triangle
|}


===Microscopic===
=====Location=====
Features:
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
*Ctyologically benign cells is spaced nests.
! Diagnosis
! [[Epiphysis]]
! [[Metaphysis]]
! [[Diaphysis]]
! Type of lesion
|-
| [[Aneurysmal bone cyst]]
| common
| most common
| rare
| [[bone]]
|-
| [[Chondroblastoma]]
| most common
| rare
| extremely rare
| [[cartilage]]
|-
| [[Chondrosarcoma]]
| uncommon
| common
| most common
| [[cartilage]]
|-
| [[Chondromyxoid fibroma]]
| rare
| most common
| common
| other
|-
| [[Enchondroma]]
| rare
| common
| common
| [[cartilage]]
|-
| [[Ewing sarcoma]]
| rare
| common
| most common
| [[bone]]
|-
| [[Giant cell tumour of bone|Giant cell tumour]]
| most common
| rare
| extremely rare
| [[bone]]
|-
| Metastatic carcinoma
| rare
| common
| most common
| other
|-
| Non-ossifying fibroma
| extremely rare
| most common
| common
| other
|-
| [[Osteoblastoma]]
| rare
| most common
| uncommon
| [[bone]]
|-
| [[Osteochondroma]]
| extremely rare{{fact}} <!-- PMID 12873205 questions this -->
| most common
| common
| [[bone]]/[[cartilage]]
|-
| [[Osteoid osteoma]]
| uncommon
| common
| common<ref name=wheelessonline>URL: [http://www.wheelessonline.com/ortho/osteoid_osteoma http://www.wheelessonline.com/ortho/osteoid_osteoma]. Accessed on: 7 May 2012</ref>
| [[bone]]
|-
| [[Osteosarcoma]]
| rare
| most common
| uncommon
| [[bone]]
|}


Images:
How to remember the primary bone lesions:
*[http://commons.wikimedia.org/wiki/File:Enchondroma_-_intermed_mag.jpg Enchondroma - intermed mag. (WC)].
#''Ewing sarcoma'' is the only malignant primary bone tumour of the diaphysis.
*[http://commons.wikimedia.org/wiki/File:Enchondroma_-_very_high_mag.jpg Enchondroma - very high mag. (WC)].
#''Giant cell tumour of bone'' is the only primary bone lesion of the epiphysis.
#The rest of the primary bone lesions are metaphyseal.
#*''Osteochondroma'' is bone first and cartilage second. It behaves like most primary bone lesions; it is usually metaphyseal.


==Chondrosarcoma==
How to remember the primary cartilaginous lesions:
===General===
#''Chondroblastoma'' is epiphyseal. The chicken wire goes around the chicken coop.
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>
#The others are diaphyseal.
*Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma).
*May be associated with a syndrome:
**Olier disease (multiple enchondromatosis).
**Maffucci syndrome (multiple enchondromas and hemangiomas).


Several subtypes exist:
=Cartilage=
*Chondrosarcoma not otherwise specified (NOS).
==Chondroma==
*Juxtacortical chondrosarcoma.
{{Main|Chondroma}}
*Myxoid chondrosarcoma.
*Mesenchymal chondrosarcoma.
*Clear cell chondrosarcoma.
*Dedifferentiated chondrosarcoma chondrosarcoma.


===Microscopic===
==Chondroblastoma==
Features:<ref>IAV. 26 February 2009.</ref>
{{Main|Chondroblastoma}}
*"Abnormal cartilage":
**Nuclear atypia.
***Nuclear clearing.
***Nucleoli.


Images:
==Chondromyxoid fibroma==
*[http://commons.wikimedia.org/wiki/File:Chondrosarcoma_(2).jpg Chondrosarcoma (WC)].
{{Main|Chondromyxoid fibroma}}


====Mesenchymal chondrosarcoma====
==Chondrosarcoma==
*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>
{{Main|Chondrosarcoma}}
*Rare variant of chondrosarcoma.


=Bone=
=Bone=
==Osteoma==
{{Main|Osteoma}}
==Osteoid osteoma==
==Osteoid osteoma==
===General===
{{Main|Osteoid osteoma}}
*Benign bone lesion.
 
Clinical:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
*Extremely painful.
**Relieved by NSAIDS.
 
===Microscopic===
Features:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
*Anastomosing bony [[trabeculae]] with:
**Variable mineralization.
***Mineralization (calcium '''p'''hosphate) = '''p'''urple on [[H&E stain]].
**Osteoblasts rimming.
***Cells line-up at edge of bone.
 
Images:
*[http://library.med.utah.edu/WebPath/COW/COW211.html Osteoid osteoma - CT scan (med.utah.edu)].
*[http://www.sciencephoto.com/images/imagePopUpDetails.html?pop=1&id=700030210&pviewid=&country=67&search=gschmeissners&matchtype=FUZZY Osteoid osteoma (sciencephoto.com)].
*[http://commons.wikimedia.org/w/index.php?title=File:Osteoblastoma_-_high_mag.jpg Osteoblastoma - high mag. (WC)] - histological similar to OO.
*[http://commons.wikimedia.org/w/index.php?title=File:Osteoblastoma_-_low_mag.jpg Osteoblastoma - low mag. (WC)] - histological similar to OO.
 
Notes:
*Histomorphologically near identical/indistinguishable from ''osteoblastoma''.<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref>


==Osteoblastoma==
==Osteoblastoma==
===General===
{{Main|Osteoblastoma}}
*Benign bone tumour.
 
===Microscopic===
Features:<ref name=Ref_Sternberg4_285>{{Ref Sternberg4|285}}</ref>
*Anastomosing bony [[trabeculae]] with:
**Osteoblasts rimming.
***Cells line-up at edge of bone.
 
Notes:
*Histomorphologically near identical/indistinguishable from ''osteoid osteoma''.<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref>
*'''Must''' be greater 1.5 cm by definition.<ref name=Ref_Sternberg4_286>{{Ref Sternberg4|286}}</ref>
 
Images:
*[http://commons.wikimedia.org/w/index.php?title=File:Osteoblastoma_-_high_mag.jpg Osteoblastoma - high mag. (WC)].
*[http://commons.wikimedia.org/w/index.php?title=File:Osteoblastoma_-_low_mag.jpg Osteoblastoma - low mag. (WC)].


==Ewing sarcoma==
==Ewing sarcoma==
===General===
{{Main|Ewing sarcoma}}
*AKA EWS/PNET:
**EWS = Ewing sarcoma.
**PNET = Primative neuroectodermal tumour.
*EWS and PNET were once thought to be different tumours.
 
Clinical
*Painful.
*Usually younger than 20 years.
 
====Radiology====
Features:<ref name=Ref_WMSP650>{{Ref WMSP|650}}</ref>
*Long bones, diaphyses.
*Destructive.
*"Onion-skin" periosteal reaction.
 
===Microscopic===
Features:<ref>PST. 22 February 2010.</ref>
*Scant clear cytoplasm (contain glycogen - PAS +ve, PAS-D -ve).
*Lack nucleoli.
*Round small nucleus.
 
Notes:
*It is a [[small round cell tumour]].
 
===[[IHC]]===
Features:<ref name=Ref_WMSP651>{{Ref WMSP|651}}</ref>
*CD99 +ve (plasma membrane staining).
*CD45 -ve.
**Done to r/o [[lymphoma]].
*+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S100).
*+/-Cytokeratins.
*Caveolin-1.<ref>PST. 22 February 2010.</ref>
**New kid on the block.
 
Notes:<ref>PST. 22 February 2010.</ref>
*CD99 +ve (plasma membrane) tumours:
**Lymphoblastic lymphoma/leukemia.
**Angiomatoid fibrous histiocytoma.
**Desmoplastic small round cell tumour.
 
===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>PMID: 3163261</ref>
**Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers).
 
Notes:
*The ''t(11;22)(q24;q12)'' is seen in ~90% of EWS/PNET... but also in:
**Olfactory neuroblastoma.
**Small cell osteogenic sarcoma.
**Polyphenotypic tumours.
**Rhbdomyosarcoma.
**Neuroblastoma (possibly).
*Several other translocations exist.


==Osteosarcoma==
==Osteosarcoma==
===General===
{{Main|Osteosarcoma}}
*Terry Fox was afflicited by this tumour.


====Definition====
==Giant cell tumour of bone==
*Tumour that makes osteoid.
{{Main|Giant cell tumour of bone}}
**Osteoid = (extracellular) organic component of bone, normally produced by osteoblasts (cells which make bone matrix).


===Microscopic===
=Other=
Features:
This section collects stuff that doesn't neatly fit into the ''bone'' or ''cartilage'' category.
*Cells with malignant features (e.g. nuclear membrane irregularities, marked nuclear size differences, mitoses) surrounded by delicate strands of osteoid.
**Osteoid on H&E: pink, homogenous, "glassy".
**Tumours typically very cellular - when compared to normal bone.
*Large (multinucleated) osteoclast-like giant cells may be seen.<ref>{{cite journal |author=Papalas JA, Balmer NN, Wallace C, Sangueeza OP |title=Ossifying dermatofibroma with osteoclast-like giant cells: report of a case and literature review |journal=Am J Dermatopathol |volume=31 |issue=4 |pages=379-83 |year=2009 |month=June |pmid=19461244 |doi=10.1097/DAD.0b013e3181966747 |url=}}</ref>


Images:
==Notochordal tumors==
*[http://commons.wikimedia.org/wiki/File:Osteosarcoma_-_intermed_mag.jpg Osteosarcoma - intermed. mag. (WC)].
Notochordal tumors arise from notochordal remnants and thus are seen in the clivus or sacrum.
*[http://commons.wikimedia.org/wiki/File:Osteosarcoma_-_very_high_mag.jpg Osteosarcoma - very high mag. (WC)].
{{Main|Chordoma}}


=Other=
==Osteochondroma==
==Pigmented villonodular synovitis==
{{Main|Osteochondroma}}
*Commonly abbreviated: ''PVNS''.
*Course: benign.


===Microscopic===
==Diffuse tenosynovial giant-cell tumour==  
Features:<ref>URL: [http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis].</ref>
*[[AKA]] ''tenosynovial giant-cell tumour, diffuse type''.
*Subsynovial nodules composed of cells with:
*Previously known as ''pigmented villonodular synovitis'' (PVNS).<ref>{{Ref PBoD8|1247}}</ref>  
**Abundant cytoplasm.
{{Main|Diffuse tenosynovial giant-cell tumour}}
**Pale nuclei.
*Multinucleated giant cells.
*Hemosiderin-laden macrophages.
*Foam cells.


Images:
==Giant cell tumour of tendon sheath==
*[http://commons.wikimedia.org/wiki/File:Pigmented_villonodular_synovitis_low_mag.jpg PVNS - low mag. (WC)].
*Abbreviated ''GCT of tendon sheath''.
*[http://commons.wikimedia.org/wiki/File:Pigmented_villonodular_synovitis_high_mag.jpg PVNS - high mag. (WC)].
{{Main|Giant cell tumour of tendon sheath}}


==Adamantinoma==
==Adamantinoma==
{{Main|Adamantinoma}}
==Brown tumour==
===General===
===General===
Features:<ref name=Ref_WMSP650>{{Ref WMSP|650}}</ref>
*''Not'' a true neoplasm.<ref name=pmid16775919>{{cite journal |author=Meydan N, Barutca S, Guney E, ''et al.'' |title=Brown tumors mimicking bone metastases |journal=J Natl Med Assoc |volume=98 |issue=6 |pages=950–3 |year=2006 |month=June |pmid=16775919 |pmc=2569361 |doi= |url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2569361/?page=1 }}</ref>  
*Rare: < 1% of bone tumours.
**If ''tumour'' is understood as a synonym for ''neoplasm'', the name is a misnomer.
*25-35 years old.
**May (clinically) mimic a true neoplasm.
*Tibia, fibula.
*Due to hyperparathyroidism - usually [[parathyroid adenoma]].
*Benign, may be locally aggressive.
**Usually secondary to chronic renal failure.
*Cousin of [[ameloblastoma]]. (???)
 
===Radiology===
*Intracortical, radiolucent.
 
===Microscopic===
Features:
*Biphasic tumour:
*#Fibrous/spindle cell component.
*#Epithelial component.
 
Images:
*[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>
 
==Brown cell tumour==
===Etiology===
*Due to hyperparathyroidism - usually parathyroid adenoma.
 
===Microscopy===
Features:
*Fibrosis.


===Hypercalcemia DDx===
====Hypercalcemia DDx====
Mnemonic ''GRIMED'':<ref>TN06 Emerg.</ref>
Mnemonic ''GRIMED'':<ref>{{Ref TN2006 |Emerg.}}</ref>
*Granulomatous disease (tuberculosis, [[sarcoidosis]]).
*Granulomatous disease (tuberculosis, [[sarcoidosis]]).
*Renal disease.
*Renal disease.
*Immobility.
*Immobility.
*Malignancy (esp. squamous cell carcinoma, [[plasmacytoma]]).
*Malignancy (esp. [[squamous cell carcinoma]], [[plasmacytoma]]).
*Endocrine (primary hyperparathyroidism - leads to brown cell tumour).
*Endocrine ([[parathyroid gland|primary hyperparathyroidism]] - leads to [[brown tumour]]).
*Drugs (thiazides ... others).
*Drugs (thiazides ... others).


==Giant cell tumour==
===Microscopic===
===General===
Features:
Features:<ref name=Ref_WMSP648>{{Ref WMSP|648}}</ref>
*Fibrosis.
*Approximately 5% of primary bone tumours.
*+/-Giant cells with round to oval nuclei and nucleoli.<ref>URL: [http://path.upmc.edu/cases/case139/micro.html http://path.upmc.edu/cases/case139/micro.html]. Accessed on: 6 January 2012.</ref>
*Typical age: 20-45 years.
*Bone unaffected by tumour - increased numbers of the following:
**Multinucleated cells (osteoclasts).
**Mononuclear cells around the bony trabeculae (osteoblasts).


===Clinical===
DDx:
*May present with joint pain, immobility.
*[[Giant cell tumour of bone]] and other [[giant cell lesions]].


===Microscopic===
====Images====
Features:<ref name=Ref_Klatt420>{{Ref Klatt|420}}</ref>
<gallery>
*Giant cells.
Image:Brown_tumour_-_low_mag.jpg | Brown tumour - low mag. (WC)
*Mononuclear cells, with nuclei similar to those in giant cells - '''key feature'''
Image:Brown_tumour_-_intermed_mag.jpg | Brown tumour - intermed. mag. (WC)
Image:Brown_tumour_-_high_mag.jpg | Brown tumour - high mag. (WC)
</gallery>
www:
*[http://wwwold.path.utah.edu/classes/webpath/bonehtml/bone053.htm Brown tumour (utah.edu)].
*[http://www.mda-sy.com/pathology/BONEHTML/BONE054.HTM Brown tumour (mda-sy.com)].
*[http://path.upmc.edu/cases/case139/micro.html Brown tumour - several images (upmc.edu)].


=See also=
=See also=
Line 291: Line 408:
{{reflist|2}}
{{reflist|2}}


=External links=
*[http://www.medpath.info/MainContent/Skeletal/Bone_07.html Bone lesions (medpath.info)].
*[http://www.radiologyassistant.nl/en/494e15cbf0d8d Bone radiology (radiologyassistant.nl)].
[[Category:Chondro-osseous tumours]]
[[Category:Weird stuff]]
[[Category:Weird stuff]]

Latest revision as of 01:46, 20 June 2016

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.

Primary bone tumours are rare; the most common bone tumour is metastases.[1]

Bone tumours occasionally are lumped with soft tissue tumours. Soft tissue tumours are dealt with in the soft tissue lesions article. An introduction to bone is found in the bone article. An introduction to cartilage is found in the cartilage article.

General

  • Diagnosis of a primary bone tumour should not be made without radiologic & clinical information!
  • Metastasis:primary bone tumours = >20:1.[1]

Common malignant

Epidemiology:[2]

  • Osteosarcoma -> 2nd decade.
  • Ewing's ->5-20 yrs.
  • Chondrosarcoma -> from enchondroma or osteochrondroma -- patients over 40 yrs.
  • Multiple myeloma -> most common primary bone tumour in adults.

Malignant bone tumours by age

Most common by age:[2]

Benign aggressive bone tumours

Ref.:[3][4]

Summary tables

Bone tumours

Entity Key feature Other features Radiology / gross Clinical Stains / other Image
Osteoma normal bone (???) other features (???) radiology / gross (???) ? no stains / may be assoc. with FAP
Osteoma. (WC)
Osteoid osteoma osteoblastic rimming anastomosing bony trabeculae must be <2 cm,[5] metaphysis painful, NSAIDs remove pain, young IHC / other
Osteoid osteoma. (WC)
Osteoblastoma osteoblastic rimming anastomosing bony trabeculae must be >1 cm,[5] often >=2 cm, metaphysis not painful IHC / other
Osteoblastoma. (WC)
Ewing sarcoma small round blue cell tumour cytoplasmic clearing (due to glycogen) usu. diaphysis pediatric, typically 1-10 years PAS+, PASD-, chromosomal translocations (usually t(11;22)(q24;q12))
Ewing sarcoma. PAS stain. (WC)
Osteosarcoma osteoid +/-hemorrhage, +/-cartilage distal femur, prox. tibia, prox. humerous typically 10-30 years, pain, swelling no stains; many subtypes
Osteosarcoma. (WC)
Giant cell tumour of bone abundant giant cells nuclei of surrounding cells similar to those in giant cells growth plate of long bones 20-45 years old, +/-joint pain, +/-immobility IHC / other
Giant cell tumour. (WC)

Cartilage tumours

Entity Key feature Other features Radiology / gross Clinical Stains / other Image
Chondroma ctyologically benign cells equally spaced nests usu. diaphysis benign / DDx: chondroma, well-diff. chondrosarcoma IHC / bone marrow cavity chondroma = enchondroma
Enchondroma. (WC)
Chondroblastoma abundant extracellular material, abundant eosinophilic cytoplasm calcifications surround cells nests ("chickenwire" appearance) - classic epiphysis DDx: giant cell tumour of bone S100+ve, vimentin +ve
Chondroblastoma. (WC)
Chondrosarcoma cartilaginous appearance +/- nuclear atypia lack osteoid, if present -> osteosarcoma usu. diaphysis, classically hip; almost never distal extremity >40 years old IHC / may be histologically benign looking
Chondrosarcoma. (WC)

Other

Entity Key feature Other features Radiology / gross Clinical Stains / other Image
Osteochondroma benign bone and cartilage Other features metaphyseal lesions Clinical IHC / other Image
Adamantinoma bisphasic - stroma & epithelium Other features tibia, fibula, intracortical, radiolucent Clinical IHC / other
Adamantinoma. (WC)
Diffuse tenosynovial giant-cell tumour (AKA PVNS) pigmented giant cells nodules Radiology / gross Clinical IHC / other
PVNS. (WC)
Brown tumour fibrosis, +/-giant cells unaffected bone incr. osteoblasts and osteoclasts Radiology / gross due to hypercalcemia; not a neoplasm IHC / other
Brown tumour. (WC)

Radiology

Radiologic features
Features Benign Malignant
Bone changes sclerotic rim tumour perforation
Circumscription pushing margins ill-defined/moth-eaten
Soft tissue involvement no common
Periosteal reaction no "hair-on-end" or "sunburst",
"onion skin", Codman's triangle
Location
Diagnosis Epiphysis Metaphysis Diaphysis Type of lesion
Aneurysmal bone cyst common most common rare bone
Chondroblastoma most common rare extremely rare cartilage
Chondrosarcoma uncommon common most common cartilage
Chondromyxoid fibroma rare most common common other
Enchondroma rare common common cartilage
Ewing sarcoma rare common most common bone
Giant cell tumour most common rare extremely rare bone
Metastatic carcinoma rare common most common other
Non-ossifying fibroma extremely rare most common common other
Osteoblastoma rare most common uncommon bone
Osteochondroma extremely rare[citation needed] most common common bone/cartilage
Osteoid osteoma uncommon common common[6] bone
Osteosarcoma rare most common uncommon bone

How to remember the primary bone lesions:

  1. Ewing sarcoma is the only malignant primary bone tumour of the diaphysis.
  2. Giant cell tumour of bone is the only primary bone lesion of the epiphysis.
  3. The rest of the primary bone lesions are metaphyseal.
    • Osteochondroma is bone first and cartilage second. It behaves like most primary bone lesions; it is usually metaphyseal.

How to remember the primary cartilaginous lesions:

  1. Chondroblastoma is epiphyseal. The chicken wire goes around the chicken coop.
  2. The others are diaphyseal.

Cartilage

Chondroma

Chondroblastoma

Chondromyxoid fibroma

Chondrosarcoma

Bone

Osteoma

Osteoid osteoma

Osteoblastoma

Ewing sarcoma

Osteosarcoma

Giant cell tumour of bone

Other

This section collects stuff that doesn't neatly fit into the bone or cartilage category.

Notochordal tumors

Notochordal tumors arise from notochordal remnants and thus are seen in the clivus or sacrum.

Osteochondroma

Diffuse tenosynovial giant-cell tumour

  • AKA tenosynovial giant-cell tumour, diffuse type.
  • Previously known as pigmented villonodular synovitis (PVNS).[7]

Giant cell tumour of tendon sheath

  • Abbreviated GCT of tendon sheath.

Adamantinoma

Brown tumour

General

  • Not a true neoplasm.[8]
    • If tumour is understood as a synonym for neoplasm, the name is a misnomer.
    • May (clinically) mimic a true neoplasm.
  • Due to hyperparathyroidism - usually parathyroid adenoma.
    • Usually secondary to chronic renal failure.

Hypercalcemia DDx

Mnemonic GRIMED:[9]

Microscopic

Features:

  • Fibrosis.
  • +/-Giant cells with round to oval nuclei and nucleoli.[10]
  • Bone unaffected by tumour - increased numbers of the following:
    • Multinucleated cells (osteoclasts).
    • Mononuclear cells around the bony trabeculae (osteoblasts).

DDx:

Images

www:

See also

References

  1. 1.0 1.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 632. ISBN 978-0781765275.
  2. 2.0 2.1 Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. OR42. ISBN 978-0968592854.
  3. Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. OR41. ISBN 978-0968592854.
  4. URL: http://www.emedicine.com/RADIO/topic494.htm.
  5. 5.0 5.1 Yalcinkaya, U.; Doganavsargil, B.; Sezak, M.; Kececi, B.; Argin, M.; Basdemir, G.; Oztop, F. (Dec 2014). "Clinical and morphological characteristics of osteoid osteoma and osteoblastoma: a retrospective single-center analysis of 204 patients.". Ann Diagn Pathol 18 (6): 319-25. doi:10.1016/j.anndiagpath.2014.08.006. PMID 25224389.
  6. URL: http://www.wheelessonline.com/ortho/osteoid_osteoma. Accessed on: 7 May 2012
  7. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1247. ISBN 978-1416031215.
  8. Meydan N, Barutca S, Guney E, et al. (June 2006). "Brown tumors mimicking bone metastases". J Natl Med Assoc 98 (6): 950–3. PMC 2569361. PMID 16775919. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2569361/?page=1.
  9. Shiau, Carolyn; Toren, Andrew (2006). Toronto Notes 2006: Comprehensive Medical Reference (Review for MCCQE 1 and USMLE Step 2) (22nd edition (2006) ed.). Toronto Notes for Medical Students, Inc.. pp. Emerg.. ISBN 978-0968592861.
  10. URL: http://path.upmc.edu/cases/case139/micro.html. Accessed on: 6 January 2012.

External links