Chondroblastoma: An Update

Arch Pathol Lab Med. 2017 Jun;141(6):867-871. doi: 10.5858/arpa.2016-0281-RS.

Abstract

Chondroblastoma is a rare primary bone tumor of young people that typically arises in the ends of the long bones. Radiologic investigations show a small, circumscribed, lytic lesion. The tumor is characterized histologically by the proliferation of chondroblasts along with areas of mature cartilage, giant cells, and occasionally, secondary aneurysmal bone cyst formation. Chondroblastoma, however, may also present with atypical features, such as prominent hemosiderin deposition, numerous giant cells, or the presence of a large aneurysmal bone cyst component. Malignant entities such as clear cell chondrosarcoma and chondroblastic osteosarcoma must also be considered. Recently, immunohistochemical stains such as DOG1 and SOX9 have been described in chondroblastoma, and K36M mutations in either the H3F3A or H3F3B genes have also been identified. While generally regarded as a benign entity, chondroblastoma manifests an intermediate type of behavior, given its ability to recur locally, and rarely, metastasize.

Publication types

  • Review

MeSH terms

  • Amino Acid Substitution
  • Anoctamin-1
  • Bone Neoplasms / diagnosis*
  • Bone Neoplasms / genetics
  • Bone Neoplasms / metabolism
  • Bone Neoplasms / pathology
  • Bone and Bones / metabolism
  • Bone and Bones / pathology
  • Chloride Channels / metabolism
  • Chondroblastoma / diagnosis*
  • Chondroblastoma / genetics
  • Chondroblastoma / metabolism
  • Chondroblastoma / pathology
  • Chondrosarcoma / diagnosis*
  • Chondrosarcoma / genetics
  • Chondrosarcoma / metabolism
  • Chondrosarcoma / pathology
  • Diagnosis, Differential
  • Histones / genetics
  • Humans
  • Mutation
  • Neoplasm Proteins / metabolism
  • Neoplasm Recurrence, Local
  • Osteosarcoma / diagnosis*
  • Osteosarcoma / genetics
  • Osteosarcoma / metabolism
  • Osteosarcoma / pathology
  • SOX9 Transcription Factor / metabolism

Substances

  • ANO1 protein, human
  • Anoctamin-1
  • Chloride Channels
  • H3-3A protein, human
  • Histones
  • Neoplasm Proteins
  • SOX9 Transcription Factor
  • SOX9 protein, human