Chordoma Arising in Benign Notochordal Cell Tumor: A Detailed Radiological, Gross and Microscopic Description of Three Cases Involving the Lumbar Spine
Shady Elia A Ishak, G Petur Nielsen, Andrew E Rosenberg. Cairo University, Cairo, Egypt; Massachusetts General Hospital, Boston, MA; University of Miami, Miami, FL
Background: Chordoma is a rare primary malignant bone neoplasm that recapitulates the emberyonic notochord. It arises almost exclusively in the axial skeleton with predilection for its proximal and distal segments. The pathogenesis of chordoma is still unknown, however, some investigators have suggested that it develops from benign notochordal cell tumor (BNCT) based on demonstrating both lesions in the same patient or by illustrating that they have the same anatomical distribution.
Design: We identified from the surgical pathology files of the department of pathology of Massachussettss General Hospital and the consultation files of one of the authors three cases of chordoma arising directly adjacent to BNCT. The radiological, gross and microscopic features were carefully analyzed.
Results: The three tumors arose in two women and one man and their ages were 42,17 and 31 respectively. On imaging studies the BNCT manifested as an irregular region of sclerosis located within the center of the vertebral body and the chordoma appeared as an oval area of lytic destruction that was centered posterior to the BNCT andtransgressed the cortex and extended into the spinal canal.
Microscopically, both lesions demonstrated characteristic histologic features and were juxtaposed to one another.
Conclusions: Hypotheses regarding the etiology of chordoma have largely been speculative with very few published examples of both lesions intimately related to one another. Our study clearly demonstrates the anatomic proximity of the two neoplasms provides further evidence that BNCT is the precursor lesion for chordoma.
Category: Bone & Soft Tissue
Tuesday, March 20, 2012 9:30 AM
Poster Session III # 3, Tuesday Morning