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)

  • Grade 2 chondrosarcoma, low power showing bone permeation (SKB)

  • Chondrocytic hyperchromasia and atypicality in this photo of a Grade 2 chondrosarcoma. (SKB)

  • Lobule of neoplastic Grade 2 cartilage with some bone permeation and calcification at the top of the photo.(SKB)

  • Lobule of neoplastic Grade 2 cartilage with atypia and bone permeation. (SKB)

  • Grade 3 - very cellular neoplastic cartilage with high grade nuclear atypia. (SKB)

www:

Variants

Mesenchymal chondrosarcoma

Main article: Mesenchymal chondrosarcoma

Myxoid chondrosarcoma

Microscopic: Features:

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

DDx:

Images:

  • Anastomizing chords of small neoplastic cells surround mucin pools.(SKB)

  • Chords of neoplastic cells surround mucin pools. (SKB)

  • Pathology outlines [1]
  • Pathology outlines [2]

Clear cell chondrosarcoma

  • Rare variant of chondrosarcoma (1.6%–5.4% of all chondrosarcomas)
  • Usually a low-grade malignant tumour[11]
  • Younger age than conventional chondrosarcoma
  • Teens to 40s; more common in males
  • Epiphyses of long tubular bones; proximal femur or humerus
  • Rarely head and neck [12]
  • Malignant counterpart of chondroblastoma?

Microscopic findings

  • Lobules of uniform to polymorphic densely-packed large cells
  • Well defined pushing borders
  • Clear to intensively acidophilic granular cytoplasm cytoplasm with vacuoles
  • Central nuclei with occasional prominent nucleoli
  • Low mitotic rate
  • Clear cell areas lack production of hyaline chondroid matrix
  • Areas with osteoclast-type giant cells mixed with small trabeculae of reactive bone
  • May contain conventional low-grade chondrosarcoma
  • May have secondary aneurysmal bone cyst changes

  • High grade round cells with cytoplasmic clearing. (SKB)

  • High grade round cells with cytoplasmic clearing. (SKB)

  • Clear cells, giant cells and bone spicules. (SKB)

  • Somewhat hemangiopericytomatous vascular pattern with giant cells. (SKB)

  • Clear cells, giant cells and bone spicules. (SKB)

  • The lesion also had areas of more conventional chondrosarcoma. (SKB)

  • Pathology outlines [3]
  • Pathology outlines [4]
  • Pathology outlines [5]
  • Tumor library [6]
  • Tumor library [7]

DDX:

  • Chondroblastoma
  • Giant cell tumour of bone
  • Osteoblastic tumours
  • Metastatic clear cell renal cell carcinoma

Outside sources: E-immunohistochemistry[8]

Extraskeletal myxoid chondrosarcoma

  • Originally thought to be a variant of myxoid chondrosarcoma of bone; however, may not be a chondrosarcoma at all.[13]
  • Characteristic chromosomal translocation: t(9;22) CHN-EWS.

DDx:

  • Chordoma.[13]
    • S-100 +ve (strong).
    • EMA +ve.
  • Myxoid liposarcoma.

Image:

  • Strands of atypical cells suspended in mucin. (SKB)

  • Strands of atypical cells suspended in mucin. (SKB)

Dedifferentiated chondrosarcoma

Clinical:

  • Abysmal to poor prognosis.
    • In one series (22 patients) 5-year survival ~20%.[15]
    • All dead in two years in another series (25 patients).[16]

Features:[16]

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

DDx:

Images:

  • A lobule of cartilagenous chondrosarcoma on the left; high grade sarcoma on the right. (SKB)

  • High grade sarcoma on the left and upper; malignant cartilage right and lower. (SKB)

Grading

Features:[17]

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

  • Grade 1 - Somewhat cellular cartilage with binucleation.(SKB)

  • Grade 2 - Very cellular cartilage with obvious hyperchromasia and nuclear atypia. (SKB)

  • Grade 3 - very cellular neoplastic cartilage with high grade nuclear atypia. (SKB)

IHC

  • S-100 positive
  • Keratin negative
  • Collagen II positive

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. ↑ 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. ↑ Fisher C (May 2000). "Parachordoma exists--but what is it?". Adv Anat Pathol 7 (3): 141–8. PMID 10809219.
  11. ↑ Corradi, D.; Bacchini, P.; Campanini, N.; Bertoni, F. (Nov 2006). "Aggressive clear cell chondrosarcomas: do distinctive characteristics exist?: a report of 4 cases.". Arch Pathol Lab Med 130 (11): 1673-9. doi:10.1043/1543-2165(2006)130[1673:ACCCDD]2.0.CO;2. PMID 17076530.
  12. ↑ Mokhtari, S.; Mirafsharieh, A. (2012). "Clear cell chondrosarcoma of the head and neck.". Head Neck Oncol 4: 13. doi:10.1186/1758-3284-4-13. PMID 22520362.
  13. ↑ 13.0 13.1 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.
  14. ↑ URL: http://www.cttr.org/cms/?p=736. Accessed on: 1 May 2011.
  15. ↑ 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.
  16. ↑ 16.0 16.1 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.
  17. ↑ 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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