title: In chordoma, metastasis, recurrences, Ki-67 index, and a matrix-poor phenotype are associated with patients' shorter overall survival.
abstract: To establish a chordoma tissue cohort (n = 43) and to correlate localization, size, metastasis, residual disease (R-status), recurrences, histological subtype, matrix content, and Ki-67 proliferation index with patients' overall survival (OS).
  
  
  
  We used routine histopathology supplemented by immunohistochemistry. In our patient cohort (median age 69 years, range 17 to 84 years) the median OS was 8.25 years. 24 chordomas were localized in the sacrum, 6 in lumbar vertebrae, 7 in thoracic and cervical vertebrae, 5 were limited to the clivus, and one was localized in the nasal septum. Ten patients had metastases, with pulmonary, nodal, and hepatic involvement. 23 patients had recurrent disease. 23 chordomas were classified as 'not otherwise specified' (NOS). Besides NOS, we found the following differentiation patterns: renal cell cancer like in six cases, chondroid in four cases, hepatoid differentiation in three cases, and anaplastic morphology in six cases. Ki-67 index of ≥10 %, presence of metastasis, and the low content of extracellular matrix were statistically linked to poor OS (p < 0.05). The matrix-poor phenotype had a higher Ki-67 index (p < 0.05). Furthermore, presence of metastasis was associated with a higher Ki-67 index in the primary lesion, a positive resection margin, and multiple recurrences (p < 0.05 each).
  
  
  
  We propose to include these parameters in the final pathologic report of the resected chordoma.
authors.affiliation: Institute of Pathology, M23, University Ulm, Albert-Einstein-Allee 11, 89081, Ulm, Germany.
authors.forename: Adrian
authors.initials: A
authors.lastname: von Witzleben
authors.affiliation: Institute of Pathology, M23, University Ulm, Albert-Einstein-Allee 11, 89081, Ulm, Germany.
authors.forename: Lukas T
authors.initials: LT
authors.lastname: Goerttler
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: Jochen
authors.initials: J
authors.lastname: Lennerz
authors.affiliation: Institute of Pathology, M23, University Ulm, Albert-Einstein-Allee 11, 89081, Ulm, Germany.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Weissinger
authors.affiliation: Department of General and Visceral Surgery, University Ulm, Ulm, Germany.
authors.forename: Marko
authors.initials: M
authors.lastname: Kornmann
authors.affiliation: Department of Internal Medicine III, University Ulm, Ulm, Germany.
authors.forename: Regine
authors.initials: R
authors.lastname: Mayer-Steinacker
authors.affiliation: Department of Trauma Surgery, University Ulm, Ulm, Germany.
authors.forename: Alexandra
authors.initials: A
authors.lastname: von Baer
authors.affiliation: Department of Trauma Surgery, University Ulm, Ulm, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Schultheiss
authors.affiliation: Institute of Pathology, M23, University Ulm, Albert-Einstein-Allee 11, 89081, Ulm, Germany. peter.moeller@uniklinik-ulm.de.
authors.forename: Peter
authors.initials: P
authors.lastname: Möller
authors.affiliation: Institute of Pathology, M23, University Ulm, Albert-Einstein-Allee 11, 89081, Ulm, Germany.
authors.forename: Thomas F E
authors.initials: TF
authors.lastname: Barth
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002817:Chordoma / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000401:mortality / Q000473:pathology; D006801:Humans; D019394:Ki-67 Antigen / Q000097:blood; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010641:Phenotype; D013125:Spinal Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000401:mortality / Q000473:pathology; D013131:Spine / Q000000981:diagnostic imaging; D055815:Young Adult
chemical_list: D019394:Ki-67 Antigen
keywords: Chordoma; Histological parameters and subtyping; Overall survival