Other tumors of bone
Benign notochordal cell tumors

Author: Nat Pernick, M.D. (see Authors page)

Revised: 16 August 2018, last major update June 2005

Copyright: (c) 2003-2018,, Inc.

PubMed search: benign notochordal cell tumor

Cite this page: Pernick, N. Benign notochordal cell tumors. website. Accessed December 16th, 2018.
Definition / general
  • Intraosseous tumors - not notochordal rest or hamartoma
  • At autopsy, found in 14% of spinal columns and 11.5% of clivi, usually ages 40+ years, often multiple
  • Followup but no surgery
  • May undergo malignant transformation to classic chordomas
Gross description
  • Usually small within axial bones, rarely involve entire vertebrae
Microscopic (histologic) description
  • Well demarcated but unencapsulated
  • Sheets of adipocyte-like vacuolated or eosinophilic cells with fewer vacuoles
  • Often cytoplasmic eosinophilic hyaline globules
  • Bland round nuclei with mild pleomorphism
  • May contain colloid-like material
  • Bone trabeculae often sclerotic but no bony destruction
  • No intercellular myxoid matrix, no necrosis, no mitotic figures
Positive stains
  • PAS+ diastase resistant eosinophilic hyaline globules
Differential diagnosis
  • Chordoma: osteolytic, lobules are separate by thin fibrous septa, lobules contain cords, strands or sheets of physaliphorous cells with myxoid matrix, cells have mild to marked nuclear atypia
  • Notochordal vestiges: cords or strands of notochordal cells within myxoid background, cells have eosinophilic cytoplasm with small vacuoles, pyknotic round nuclei, CK18 negative, usually replaced by fibrocartilage by age 1 - 3 years
Additional references