Difference between revisions of "Chondro-osseous tumours"

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'''Bone''' occasionally crosses the desk of the pathologist.  Primary bone tumours are rare; the most common bone tumour is metastases.<ref>WMSP P.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]].  


==Normal==
Primary bone tumours are rare; the most common bone tumour is [[metastases]].<ref name=Ref_WMSP632>{{Ref WMSP|632}}</ref> 
*Normal bone has osteocytes.
**If the osteocytes are missing... the bone is dead.


*Osteoblasts - make bone.
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.
*Osteoclasts - destroy bone.


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


===Bone marrow===
===Common malignant===
*Fat content (%) ~= age (in years)<ref>IAV 26 Feb 09</ref>
*[[Osteosarcoma]].
**e.g. 60 year old will have 60% fatty replacement.
*[[Chondrosarcoma]].
*[[Ewing's sarcoma]].
*[[Multiple myeloma]].
*[[Metastases]].
**Most common tumours metastatic to bone (mnemonic: ''BLT with Ketchup & Pickles''):
***[[Breast]].
***[[Liver tumours|Liver]].
***[[Thyroid gland|Thyroid gland]].
***[[Kidney tumours|Kidney]].
***[[Prostate gland]].
 
Epidemiology:<ref name=Ref_TN2005_OR42>{{Ref TN2005 |OR42}}</ref>
*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.


*Should see three cell lines.
===Malignant bone tumours by age===
**The cell lines:<ref>[http://emedicine.medscape.com/article/199003-overview http://emedicine.medscape.com/article/199003-overview]</ref>
Most common by age:<ref name=Ref_TN2005_OR42>{{Ref TN2005 |OR42}}</ref>
***Erythroid (red cells),  
*<1 year old - [[neuroblastoma]].
***Myeloid (white blood cells),  
*1-10 years old - [[Ewing sarcoma|Ewing's]] of tubular bones.
***Megakaryocytic (platelets).
*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]].
*>40 years old - mets, [[multiple myeloma]], [[chondrosarcoma]].


Note: Lymphocytes are considered separately and typically spared in bone marrow failure.<ref>[http://emedicine.medscape.com/article/199003-overview http://emedicine.medscape.com/article/199003-overview]</ref>
===Benign aggressive bone tumours===
*[[Giant cell tumour of bone]].
*[[Osteoblastoma]].
**Thought to be related to [[osteoid osteoma]].
**If in long bones often diaphyseal.


Identifying the lines:<ref>[http://upload.wikimedia.org/wikipedia/commons/6/69/Hematopoiesis_%28human%29_diagram.png http://upload.wikimedia.org/wikipedia/commons/6/69/Hematopoiesis_%28human%29_diagram.png]</ref>
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>
#Megakaryocytes:
#*Big cells ~ 3x the size of a RBC.
===Summary tables===
#Normoblasts (RBC precursors):
====Bone tumours====
#*Hyperchromatic, i.e. blue, nucleus.
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
#Myeloid line:
! Entity
#*Granules.
! Key feature
#*Reniform nucleus, i.e. kidney bean shaped nucleus.
! 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)]]
|-
|}


Images:
====Cartilage tumours====
*[http://commons.wikimedia.org/wiki/File:Bone_marrow_WBC.JPG Myeloid line (WC)].
{| 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)]]
|-
|}


====Organization====
====Other====
*Mature hematopoeitic cells at the centre (distant from bone).
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
*Immature hematopoeitic cells adjacent to the bone.
! 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)]]
|-
|}


==Infectious==
====Radiology====
===Osteomyelitis===
=====Radiologic features=====
General
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
*Hematogenous - often in children.
! Features
*Direct entry (skin defect) - adults with diabetes.
! 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=====
*PMNs.
{| class="wikitable sortable" style="margin-left:auto;margin-right:auto"
! 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]]
|}


===Chronic osteomyelitis===
How to remember the primary bone lesions:
*Plasma cells.
#''Ewing sarcoma'' is the only malignant primary bone tumour of the diaphysis.
**May be sterile, i.e. no organisms.
#''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.


==Bone tumours==
How to remember the primary cartilaginous lesions:
General:
#''Chondroblastoma'' is epiphyseal. The chicken wire goes around the chicken coop.
*Metastasis:primary bone tumours = >20:1.<ref>WMSP P.632.</ref>
#The others are diaphyseal.


==Giant cell tumour==
=Cartilage=
General<ref>WMSP P.648.</ref>
==Chondroma==
*Approx. 5% of primary bone tumours.
{{Main|Chondroma}}
*Age 20-45 years.


Clinical
==Chondroblastoma==
*May present with joint pain, immobility.
{{Main|Chondroblastoma}}


Microscopic:
==Chondromyxoid fibroma==
*Mononuclear cells '''key feature'''.
{{Main|Chondromyxoid fibroma}}
*Giant cells.


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


==Ewing sarcoma==
=Bone=
===General===
==Osteoma==
*AKA EWS/PNET:
{{Main|Osteoma}}
**EWS = Ewing sarcoma.
**PNET = Primative neuroectodermal tumour.
*EWS and PNET were once thought to be different tumours.


Clinical
==Osteoid osteoma==
*Painful.
{{Main|Osteoid osteoma}}
*Usually younger than 20 years.


===Radiology===
==Osteoblastoma==
Features:<ref>WMSP P.650.</ref>
{{Main|Osteoblastoma}}
*Long bones, diaphyses.
*Destructive.
*"Onion-skin" periosteal reaction.


===Microscopic===
==Ewing sarcoma==
Classification:
{{Main|Ewing sarcoma}}
*Small blue cell tumour.


Features:<ref>PST. 22 February 2010.</ref>
==Osteosarcoma==
*Scant clear cytoplasm (contain glycogen - PAS +ve, PAS-D -ve).
{{Main|Osteosarcoma}}
*Lack nucleoli.
*Round small nucleus.


===[[IHC]]===
==Giant cell tumour of bone==
Features:<ref>WMSP P.651.</ref>
{{Main|Giant cell tumour of bone}}
*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>
=Other=
*CD99 +ve (plasma membrane) tumours:
This section collects stuff that doesn't neatly fit into the ''bone'' or ''cartilage'' category.
**Lymphoblastic lymphoma/leukemia.
**Angiomatoid fibrous histiocytoma.
**Desmoplastic small round cell tumour.


===Molecular diagnostics===
==Notochordal tumors==
Common features:
Notochordal tumors arise from notochordal remnants and thus are seen in the clivus or sacrum.
*''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>
{{Main|Chordoma}}
**Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers).


Notes:
==Osteochondroma==
*The ''t(11;22)(q24;q12)'' is seen in ~90% of EWS/PNET... but also in:
{{Main|Osteochondroma}}
**Olfactory neuroblastoma.
**Small cell osteogenic sarcoma.
**Polyphenotypic tumours.
**Rhbdomyosarcoma.
**Neuroblastoma (possibly).
*Several other translocations exist.


==Pigmented villonodular synovitis==
==Diffuse tenosynovial giant-cell tumour==  
*Commonly abbreviated: ''PVNS''.
*[[AKA]] ''tenosynovial giant-cell tumour, diffuse type''.
*Course: benign.
*Previously known as ''pigmented villonodular synovitis'' (PVNS).<ref>{{Ref PBoD8|1247}}</ref>
{{Main|Diffuse tenosynovial giant-cell tumour}}


===Microscopy===
==Giant cell tumour of tendon sheath==
Features:<ref>[http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis http://www.wheelessonline.com/ortho/pigmented_villonodular_synovitis]</ref>
*Abbreviated ''GCT of tendon sheath''.
*Subsynovial nodules composed of cells with:
{{Main|Giant cell tumour of tendon sheath}}
**Abundant cytoplasm.
**Pale nuclei.
*Multinucleated giant cells.
*Hemosiderin-laden macrophages.
*Foam cells.


==Osteosarcoma==
==Adamantinoma==
===General===
{{Main|Adamantinoma}}
*Terry Fox was afflicited by this tumour.
 
===Definition===
*Tumour that makes osteoid.
**Osteoid = (extracellular) organic component of bone, normally produced by osteoblasts (cells which make bone matrix).


===Histology===
==Brown tumour==
*Spindle cells with malignant features (e.g. nuclear membrane irregularies, 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>
 
==Adamantinoma==
===General===
===General===
Features:<ref>WMSP P.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]].<ref>NEED REF.</ref>


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


===Microscopic===
===Microscopic===
Features:
Features:
*Fibrous tumour.
*Fibrosis.
 
*+/-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>
==Common malignant==
*Bone unaffected by tumour - increased numbers of the following:
*Osteosarcoma.
**Multinucleated cells (osteoclasts).
*Chondrosarcoma.
**Mononuclear cells around the bony trabeculae (osteoblasts).
*Ewing's sarcoma.
*Multiple myeloma.
*Metastases.
**Most common tumours metastatic to bone (mnemonic: ''BLT with Ketchup & Pickles''):
***Breast.
***Liver.  
***Thyroid.
***Kidney.  
***Prostate.  
 
Epi.<ref>TN05 OR42.</ref>
*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==
DDx:
Most common by age:<ref>TN05 OR42.</ref>
*[[Giant cell tumour of bone]] and other [[giant cell lesions]].
*<1 year old - neuroblastoma.
*1-10 years old - Ewing's of tubular 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.
*>40 years old - mets, multiple myeloma, chondrosarcoma.


==Benign aggressive bone tumours==
====Images====
*Giant cell tumours.
<gallery>
*Osteoblastoma.
Image:Brown_tumour_-_low_mag.jpg | Brown tumour - low mag. (WC)
**Thought to be related to osteoid osteoma.
Image:Brown_tumour_-_intermed_mag.jpg | Brown tumour - intermed. mag. (WC)
**If in long bones often diaphyseal.
Image:Brown_tumour_-_high_mag.jpg | Brown tumour - high mag. (WC)
Ref.:<ref>TN05 OR41.</ref><ref>URL: [http://www.emedicine.com/RADIO/topic494.htm http://www.emedicine.com/RADIO/topic494.htm].</ref>
</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)].


==Brown cell tumour==
=See also=
===Etiology===
*Due to hyperparathyroidism - usually parathyroid adenoma.
 
===Microscopy===
Features:
*Fibrosis.
 
===Hypercalcemia DDx===
Mnemonic ''GRIMED'':<ref>TN06 Emerg</ref>
*Granulomatous disease (tuberculosis, sarcoidosis).
*Renal disease.
*Immobility.
*Malignancy (esp. squamous cell carcinoma, [[plasmacytoma]]).
*Endocrine (primary hyperparathyroidism - leads to brown cell tumour).
*Drugs (thiazides ... others).
 
==See also==
*[[Hematopathology]].
*[[Hematopathology]].
*[[Soft tissue lesions]].
*[[Small round cell tumours]].


==References==
=References=
{{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

Main article: Chondroma

Chondroblastoma

Main article: Chondroblastoma

Chondromyxoid fibroma

Main article: Chondromyxoid fibroma

Chondrosarcoma

Main article: Chondrosarcoma

Bone

Osteoma

Main article: Osteoma

Osteoid osteoma

Main article: Osteoid osteoma

Osteoblastoma

Main article: Osteoblastoma

Ewing sarcoma

Main article: Ewing sarcoma

Osteosarcoma

Main article: Osteosarcoma

Giant cell tumour of bone

Main article: 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.

Main article: Chordoma

Osteochondroma

Main article: Osteochondroma

Diffuse tenosynovial giant-cell tumour

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

Giant cell tumour of tendon sheath

  • Abbreviated GCT of tendon sheath.
Main article: Giant cell tumour of tendon sheath

Adamantinoma

Main article: 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

  • Brown tumour - low mag. (WC)

  • Brown tumour - intermed. mag. (WC)

  • Brown tumour - high mag. (WC)

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

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