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Metastasizing chondroblastoma: a rare bone tumor no longer supported by the WHO classification

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Abstract

According to the World Health Organization (WHO) classification, tumors showing hematogenous spread in less than 2% of cases are categorized as “rarely metastasizing” and constitute a group of neoplasms of intermediate malignancy. Since its introduction in 2002, chondroblastoma has been considered one of the prototypic examples of this category of lesions. In the fifth and only recently published edition of the WHO classification of bone and soft tissue tumors, however, chondroblastoma was re-classified from rarely metastasizing to benign due to the rarity of cases with systemic spread. Here, we present a remarkable case of a 54-year-old male who presented with an expansile tumor in his left acromion that was diagnosed as chondroblastoma following biopsy. Three years later a local recurrence was noted during routine follow-up and a bone scan detected hypermetabolic lesions in a rib and the thoracic spine. Over time, he developed multifocal skeletal spread as well as soft tissue and pulmonary metastases, which histologically all revealed conventional chondroblastoma morphology and the highly specific H3-3B point mutation (p.Lys36Met). Thirteen years after initial diagnosis he is currently treated with experimental immunotherapy and shows stable but disseminated disease. Our case highlights that although metastasizing chondroblastoma is rare, potential systemic spread should be kept in mind in patients with chondroblastoma despite the new WHO classification.

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Funding

DB and BA were supported by the Swiss National Science Foundation, the Foundation of the Basel Bone Tumour Reference Centre, the Gertrude von Meissner Stiftung, and the Stiftung für krebskranke Kinder, Regio Basiliensis.

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Author notes

  1. Daniel Baumhoer and Dorothee Harder contributed equally to this work.

Authors and Affiliations

  1. Bone Tumor Reference Center at the Institute of Pathology, University Hospital Basel, University of Basel, Schoenbeinstrasse 40, 4031, Basel, Switzerland

    Daniel Baumhoer & Baptiste Ameline

  2. Department of Radiology, University Hospital Basel, University of Basel, Basel, Switzerland

    Dorothee Harder

  3. Institute of Pathology, University of Bern, Bern, Switzerland

    Heather Dawson

  4. Department of Medical Oncology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland

    Attila Kollar

Authors
  1. Daniel Baumhoer
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  2. Dorothee Harder
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  3. Baptiste Ameline
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  4. Heather Dawson
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  5. Attila Kollar
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Correspondence to Daniel Baumhoer.

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Baumhoer, D., Harder, D., Ameline, B. et al. Metastasizing chondroblastoma: a rare bone tumor no longer supported by the WHO classification. Skeletal Radiol 50, 255–260 (2021). https://doi.org/10.1007/s00256-020-03525-6

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  • DOI: https://doi.org/10.1007/s00256-020-03525-6

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