Metastatic Chondroblastoma with Elevated Creatine Kinase and Paraneoplastic Neurologic Autoimmunity

Divya Devi Joshi, Peter M. Anderson, Jane Matsumoto, Christopher Moir, Thomas Shives, Krishnan Unni, Vanda A. Lennon

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

A 17-year-old girl presented with chondroblastoma of the fibula and pulmonary metastases. Serum creatine kinase (CK) was elevated for a decade preoperatively and fell to near normal after tumor excision. An episode of aseptic meningitis 3 months later led to serologic detection of three antibody markers of paraneoplastic neurologic autoimmunity that in adult patients are characteristic of thymoma and lung carcinoma: muscle-type acetylcholine receptor (AChR) autoantibody (Ab), type 2 Purkinje cell cytoplasmic Ab (PCA-2), and collapsin response-mediator protein-5-IgG (CRMP-5-IgG). Locally recurrent chondroblastoma was excised 1 year later. Serum CK was elevated, CRMP-5-IgG was positive, PCA-2 was lower, and AChR Ab was undetectable. Three years after diagnosis she has stable pulmonary metastases, with elevated CK, higher PCA-2, positive CRMP-5-IgG, and undetectable AChR Ab. The association of metastatic chondroblastoma, elevated CK, and an evolving profile of paraneoplastic autoantibodies reveals a previously unrecognized immunobiologic dimension of chondroblastoma.

Original languageEnglish (US)
Pages (from-to)900-904
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume25
Issue number11
DOIs
StatePublished - Nov 1 2003

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Keywords

  • Acetylcholine receptor antibody
  • CRMP-5
  • Pediatric
  • Pulmonary metastases
  • Tumor immunity

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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