Vertebral intra-osseous chordoma or giant notochordal rest?

Abstract

Chordomas of the lumbar vertebral bodies are rare. We report an unusual case of an entirely intraosseous chordoma of the fifth lumbar vertebra treated by vertebrectomy. Conventional radiographs and scintigraphy were normal. The lesion was well visualised by MR imaging, but showed only slight sclerosis on CT. We give our reasons for making a diagnosis of chordoma rather than giant notochordal rest and discuss the problems of management resulting from this diagnostic dilemma.

Entrapped intralesional marrow: a hitherto undescribed imaging feature of benign notochordal cell tumour

“…[4]. Since 1996, the relatively consistent pathological and imaging features of this entity have been mainly described in a number of case reports.…”

“…This case had previously been presented at the International Skeletal Society meeting in 1996 and subsequently published in 1999 [3,4]. The original histology report at the time of the vertebrectomy noted "The tumour is seen replacing the marrow, except for a few small foci of marrow which survive in the midst of the tumour.…”

Cervical Chordoma in Childhood Without Typical Vertebral Bony Destruction

“…Giant vertebral notochordal rest is a new, benign entity, which is easily confused with vertebral chordoma. [12][13][14] It also shows a gray-white lesion, involving about half of the vertebral body without visible bone destruction. 13 Histologically, the trabecular bone was preserved.…”

Notochordal Inclusions in the Vertebral Bone Marrow

“…Pain can be associated with notochordal inclusions. (1,(6)(7)(8) To what extent the inclusions contributed to pain in our case cannot be stated, because Schmorl's nodes are also a source of back pain. (21) Notably, Schmorl's nodes are common and can arise not only from defects of developmental process (vertebral end plate defects through the vascular channels or through ossification gaps) but also from trauma or degenerative In the developing vertebra, disappearance of the notochord occurs early in development (around the fifth week) (B).…”

“…(2)(3)(4) More recently, however, several reports have suggested that the detection of notochordal cells in the vertebrae may not be diagnostic of chordoma and may in fact not be infrequent. (5) The finding of notochordal tissue without diagnostic features of chordoma has been interpreted as evidence of a notochordal "rest," (1,6,7) "hamartoma," (8) or "benign tumor." (5,(9)(10)(11) Criteria for dispelling a diagnosis of chordoma in these cases include the absence of osteolysis and extension into the soft tissues and the absence of histological hallmarks of chordoma such as nuclear atypia, mitotic activity, necrosis, and extensive production of a mucoid matrix.…”