Chondromyxoid fibroma

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Chondromyxoid fibroma
Diagnosis in short

Chondromyxoid fibroma. H&E stain.

LM spindle cells or stellate cells in a myxoid or chondroid stroma, lobules with hypocellular centers and hypercellular peripheries, +/-giant cells in the hypercellular periphery, scattered calcifications, no true hyaline cartilage formation, no mitotic activity
LM DDx chondroblastoma, chondrosarcoma, phosphaturic mesenchymal tumour (case report)
Site bone (metaphysis) - see bone tumours

Clinical history teenager/young adult
Prevalence uncommon
Prognosis benign

Chondromyxoid fibroma is a rare benign chondro-osseous tumour typically found in the metaphysis of teenagers or young adults.

General

  • Uncommon and benign.[1]
  • Teenagers or young adults.

Gross

  • Metaphyseal lesion - classic location.[2]
  • Well-circumscribed.
  • Fragments of white-grey rubbery tissue.

Microscopic

Features:[3]

  • Spindle cells or stellate cells in a myxoid or chondroid stroma.
  • Lobules with hypocellular centers and hypercellular peripheries.
  • Giant cells in the hypercellular periphery.
  • Scattered calcifications.
  • No true hyaline cartilage formation.
  • No mitotic activity.

IHC

SOX9 positive

DDx:

Images

www:

Molecular

  • Activating rearrangements of GRM1 (metabotropic glutamate receptor 1).[5]

See also

References

  1. Bhamra, JS.; Al-Khateeb, H.; Dhinsa, BS.; Gikas, PD.; Tirabosco, R.; Pollock, RC.; Skinner, JA.; Aston, WJ. et al. (2014). "Chondromyxoid fibroma management: a single institution experience of 22 cases.". World J Surg Oncol 12: 283. doi:10.1186/1477-7819-12-283. PMID 25217119.
  2. Budny, AM.; Ismail, A.; Osher, L.. "Chondromyxoid fibroma.". J Foot Ankle Surg 47 (2): 153-9. doi:10.1053/j.jfas.2007.08.013. PMID 18312923.
  3. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 642. ISBN 978-0781765275.
  4. Suryawanshi, P.; Agarwal, M.; Dhake, R.; Desai, S.; Rekhi, B.; Reddy, KB.; Jambhekar, NA. (Nov 2011). "Phosphaturic mesenchymal tumor with chondromyxoid fibroma-like feature: an unusual morphological appearance.". Skeletal Radiol 40 (11): 1481-5. doi:10.1007/s00256-011-1159-6. PMID 21533894.
  5. Nord, KH.; Lilljebjörn, H.; Vezzi, F.; Nilsson, J.; Magnusson, L.; Tayebwa, J.; de Jong, D.; Bovée, JV. et al. (May 2014). "GRM1 is upregulated through gene fusion and promoter swapping in chondromyxoid fibroma.". Nat Genet 46 (5): 474-7. doi:10.1038/ng.2927. PMID 24658000.

External links