title: Metastatic chondroblastoma with elevated creatine kinase and paraneoplastic neurologic autoimmunity.
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.
authors: Division of Pediatric Hematology/Oncology, Mayo Clinic Rochester, Minnesota 55905, USA.
authors: Divya-Devi
authors: DD
authors: Joshi
authors: Peter M
authors: PM
authors: Anderson
authors: Jane
authors: J
authors: Matsumoto
authors: Christopher
authors: C
authors: Moir
authors: Thomas
authors: T
authors: Shives
authors: Krishnan
authors: K
authors: Unni
authors: Vanda A
authors: VA
authors: Lennon
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D015551:Autoimmunity; D002804:Chondroblastoma / Q000201:enzymology / Q000276:immunology* / Q000556:secondary*; D003402:Creatine Kinase / Q000097:blood*; D005260:Female; D006801:Humans; D006867:Hydrolases; D008175:Lung Neoplasms / Q000556:secondary; D008581:Meningitis / Q000209:etiology; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins / Q000276:immunology; D010257:Paraneoplastic Syndromes / Q000276:immunology*; D011689:Purkinje Cells / Q000276:immunology; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D001323:Autoantibodies; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins; D011950:Receptors, Cholinergic; D003402:Creatine Kinase; C415087:DPYSL5 protein, human; D006867:Hydrolases