Difference between revisions of "Chondrosarcoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Chondrosarcoma_(3).jpg
| Width      =
| Caption    = Chondrosarcoma. [[H&E stain]].
| Synonyms  =
| Micro      =
| Subtypes  = chondrosarcoma not otherwise specified (NOS), juxtacortical chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma, clear cell chondrosarcoma, dedifferentiated chondrosarcoma
| LMDDx      = [[chondroblastic osteosarcoma]], [[enchondroma]] (esp. for low-grade chondrosarcoma), [[chordoma]], others
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      = cartilaginous appearance
| Grossing  =
| Site      = hip, shoulder, soft tissue, others
| Assdx      =
| Syndromes  = Olier disease, Maffucci syndrome
| Clinicalhx = adults
| Signs      = mass lesion
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good ~75% five year survival
| Other      =
| ClinDDx    = [[enchondroma]], [[bone tumours]], [[soft tissue lesions]]
| Tx        = excision
}}
[[Chondrosarcoma]] is a malignant tumour of [[cartilage]]. It is in the [[Chondro-osseous_tumours|chondro-osseous grouping of tumours]] and can be lumped into the much large category of the [[soft tissue lesions]].
[[Chondrosarcoma]] is a malignant tumour of [[cartilage]]. It is in the [[Chondro-osseous_tumours|chondro-osseous grouping of tumours]] and can be lumped into the much large category of the [[soft tissue lesions]].


Revision as of 04:42, 31 December 2013

Chondrosarcoma
Diagnosis in short

Chondrosarcoma. H&E stain.
Subtypes chondrosarcoma not otherwise specified (NOS), juxtacortical chondrosarcoma, myxoid chondrosarcoma, mesenchymal chondrosarcoma, clear cell chondrosarcoma, dedifferentiated chondrosarcoma
LM DDx chondroblastic osteosarcoma, enchondroma (esp. for low-grade chondrosarcoma), chordoma, others
Gross cartilaginous appearance
Site hip, shoulder, soft tissue, others
Syndromes Olier disease, Maffucci syndrome
Clinical history adults
Signs mass lesion
Prevalence uncommon
Prognosis good ~75% five year survival
Clin. DDx enchondroma, bone tumours, soft tissue lesions
Treatment excision

Chondrosarcoma is a malignant tumour of cartilage. It is in the chondro-osseous grouping of tumours and can be lumped into the much large category of the soft tissue lesions.

General

  • Usually a good prognosis - 75% five year survival in one large data set.[1]
    • Subtypes vary substantially - chondrosarcoma NOS and myxoid chondrosarcoma have a five year survival of ~70%, but mesenchymal chondrosarcoma only ~50%, and dedifferentiated chondrosarcoma an abysmal ~0%![2]
  • Grade and stage are independent predictors of survival.[2]

Clinical/epidemiologic features:[3]

  • Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma).
  • May be associated with a syndrome:

Subtypes

Several subtypes and their relative prevalence:[2]

  • Chondrosarcoma not otherwise specified (NOS) ~83% of cases.
  • Juxtacortical chondrosarcoma <1% of cases.
  • Myxoid chondrosarcoma ~10% of cases.
  • Mesenchymal chondrosarcoma ~4% of cases.
  • Clear cell chondrosarcoma <1% of cases
  • Dedifferentiated chondrosarcoma ~1% of cases.

Gross

  • Appendicular skeleton ~45% of cases.[2]
    • Classically hip.
  • Axial skeleton ~30% of cases.
  • Soft tissue ~10% of cases.

Note:

  • Peripheral chondrosarcoma are very rare.[4]

Microscopic

Features:[5][6]

  • "Abnormal cartilage":
    • +/-Nuclear atypia - high grade lesions.
      • High grade lesions:
        • Nuclear clearing.
        • Nucleoli.
        • Hyperchromasia.
      • Low/intermediate grade lesions:
        • Bi-nucleation.
        • Hypochromatic enlarged nuclei.
        • Infiltration of lamellar bone ("invasion") - not common - diagnostic.
    • Increased cellularity.
      • More cellular than cartilage... but relatively paucicellular compared to other sarcomas.
    • Irregular spacing of chondrocytes.

Notes:

  • Low grade chondrosarcoma are not cytologically malignant; the diagnosis rests mostly on radiologic findings.
    • The exception is infiltration of lamellar bone -- this is diagnostic of chondrosarcoma.[7]

DDx:

Images

  • Chondrosarcoma - low mag. (WC)

  • Chondrosarcoma - high mag. (WC)

  • Chondrosarcoma - high mag. (WC)

www:

Variants

Mesenchymal chondrosarcoma

  • Arise in soft tissue; this is where the name comes from.[9]
  • Rare variant of chondrosarcoma.

Microscopic: Features:

  • "White clouds in a blue sky".

Image:

Myxoid chondrosarcoma

Microscopic: Features:

  • Chordoma-like:
    • Myxoid background.
    • Small cells with eosinophilic cytoplasm.

DDx:

Extraskeletal myxoid chondrosarcoma

  • Originally thought to be a variant of myxoid chondrosarcoma of bone; however, may not be a chondrosarcoma at all.[11]
  • Characteristic chromosomal translocation: t(9;22) CHN-EWS.

DDx:

  • Chordoma.[11]
    • S-100 +ve (strong).
    • EMA +ve.

Image:

Dedifferentiated chondrosarcoma

Clinical:

  • Abysmal to poor prognosis.
    • In one series (22 patients) 5-year survival ~20%.[13]
    • All dead in two years in another series (25 patients).[14]

Features:[14]

  1. Poorly differentiated (mesenchymal) malignancy.
  2. Well-differentiated cartilaginous component.

DDx:

Images:

Grading

Features:[15]

  • Grade I: mild-to-moderate increase of cellularity +/- binucleated cells.
  • Grade II: between Grade I and Grade III.
  • Grade III: nuclear pleomorphism, mitoses common.

IHC

  • S-100 -ve. (???)

See also

References

  1. ↑ Damron, TA.; Ward, WG.; Stewart, A. (Jun 2007). "Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report.". Clin Orthop Relat Res 459: 40-7. doi:10.1097/BLO.0b013e318059b8c9. PMID 17414166.
  2. ↑ 2.0 2.1 2.2 2.3 Giuffrida, AY.; Burgueno, JE.; Koniaris, LG.; Gutierrez, JC.; Duncan, R.; Scully, SP. (May 2009). "Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database.". J Bone Joint Surg Am 91 (5): 1063-72. doi:10.2106/JBJS.H.00416. PMID 19411454.
  3. ↑ Skubitz KM, D'Adamo DR (November 2007). "Sarcoma". Mayo Clin. Proc. 82 (11): 1409–32. PMID 17976362. http://www.mayoclinicproceedings.com/content/82/11/1409.long.
  4. ↑ Henderson, ER.; Pala, E.; Angelini, A.; Rimondi, E.; Ruggieri, P. (2013). "Dedifferentiated peripheral chondrosarcoma: a review of radiologic characteristics.". Sarcoma 2013: 505321. doi:10.1155/2013/505321. PMID 23589702.
  5. ↑ IAV. 26 February 2009.
  6. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 417. ISBN 978-1416002741.
  7. ↑ Dickson, B. 28 April 2011.
  8. ↑ URL: http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm. Accessed on: 29 December 2013.
  9. ↑ Dowling EA (June 1964). "Mesenchymal chondrosarcoma". J Bone Joint Surg Am 46: 747–54. PMID 14161087. http://www.ejbjs.org/cgi/reprint/46/4/747.pdf.
  10. ↑ Fisher C (May 2000). "Parachordoma exists--but what is it?". Adv Anat Pathol 7 (3): 141–8. PMID 10809219.
  11. ↑ 11.0 11.1 Aigner, T.; Oliveira, AM.; Nascimento, AG. (Feb 2004). "Extraskeletal myxoid chondrosarcomas do not show a chondrocytic phenotype.". Mod Pathol 17 (2): 214-21. doi:10.1038/modpathol.3800036. PMID 14657948.
  12. ↑ URL: http://www.cttr.org/cms/?p=736. Accessed on: 1 May 2011.
  13. ↑ Mitchell, AD.; Ayoub, K.; Mangham, DC.; Grimer, RJ.; Carter, SR.; Tillman, RM. (Jan 2000). "Experience in the treatment of dedifferentiated chondrosarcoma.". J Bone Joint Surg Br 82 (1): 55-61. PMID 10697315.
  14. ↑ 14.0 14.1 Sopta, J.; Dordević, A.; Tulić, G.; Mijucić, V. (Feb 2008). "Dedifferentiated chondrosarcoma: our clinico-pathological experience and dilemmas in 25 cases.". J Cancer Res Clin Oncol 134 (2): 147-52. doi:10.1007/s00432-007-0262-5. PMID 17653766.
  15. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 643. ISBN 978-0781765275.
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