Chondrosarcoma

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Chondrosarcoma
Diagnosis in short

Chondrosarcoma. H&E stain.
LM "abnormal cartilage": +/-high grade changes - nuclear atypia (nuclear clearing, nucleoli, hyperchromasia), low/intermediate grade changes - bi-nucleation, hypochromatic enlarged nuclei, infiltration of lamellar bone ("invasion"), increased cellularity, irregular spacing of chondrocytes
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
Molecular t(9;22) for extraskeletal myxoid chondrosarcoma
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]
  • Chondrosarcoma is the most common primary malignant chest wall lesion.[5]
    • The classical location is anterior (costochondral arches or sternum), where it is more common than chondroma.

Microscopic

Features:[6][7]

  • "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.[8]

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.[10]
  • Rare variant of chondrosarcoma.

Microscopic: Features:

  • "White clouds in a blue sky".

Image:

General

  • Rare variant of chondrosarcoma.
  • 2–10% of primary chondrosarcomas
  • Adolescents and young adults
  • Female predilection
  • Most commonly intraosseous but can occur in extraskeletal sites especially the central nervous system (from the meminges)
  • Conceptualized as originating from a pleuripotential mesenchymal cell with foci recapitulating enchondral ossification.
  • The small cells appear to be an undifferentiated cartilage stem cell which “differentiate” into benign cartilage [11].
  • This is where the name comes from.[10]

Gross

Pink and fleshy with foci of calcification.

Microscopic

  • "White clouds in a blue sky".
  • Malignant tumor with a characteristic biphasic pattern
    • Cellular poorly differentiated small round blue cells
    • Islands of well-differentiated hyaline cartilage
      • Progressive maturation of cartilage towards the center
      • Central calcification or bone formation

IHC

  • SOX9 (positive in small cells and chondrocytes)
  • S100 (positive in chondrocytes)
  • Osteocalcin (negative in small cells)

Molecular

t(8;8)(q21.1;q13.3) HEY1-NCOA2

Photos


See also

Image:

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. ↑ Somers, J.; Faber, LP. (Jul 1999). "Chondroma and chondrosarcoma.". Semin Thorac Cardiovasc Surg 11 (3): 270-7. PMID 10451259.
  6. ↑ IAV. 26 February 2009.
  7. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 417. ISBN 978-1416002741.
  8. ↑ Dickson, B. 28 April 2011.
  9. ↑ URL: http://www.path.utah.edu/casepath/ms%20cases/MSCase6/MSCase6Part3.htm. Accessed on: 29 December 2013.
  10. ↑ 10.0 10.1 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.
  11. ↑ Fanburg-Smith, JC.; Auerbach, A.; Marwaha, JS.; Wang, Z.; Rushing, EJ. (May 2010). "Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases.". Hum Pathol 41 (5): 653-62. doi:10.1016/j.humpath.2009.11.006. PMID 20138330.

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.[2]
  • Characteristic chromosomal translocation: t(9;22) CHN-EWS.

DDx:

  • Chordoma.[2]
    • S-100 +ve (strong).
    • EMA +ve.

Image:

Dedifferentiated chondrosarcoma

Clinical:

  • Abysmal to poor prognosis.
    • In one series (22 patients) 5-year survival ~20%.[4]
    • All dead in two years in another series (25 patients).[5]

Features:[5]

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

DDx:

Images:

Grading

Features:[6]

  • 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. ↑ Fisher C (May 2000). "Parachordoma exists--but what is it?". Adv Anat Pathol 7 (3): 141–8. PMID 10809219.
  2. ↑ 2.0 2.1 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.
  3. ↑ URL: http://www.cttr.org/cms/?p=736. Accessed on: 1 May 2011.
  4. ↑ 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.
  5. ↑ 5.0 5.1 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.
  6. ↑ 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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