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Send your answers to: Sushama Patil, MD
Final Diagnosis: Bone, L4 vertebra - Giant notochordal rest
Answers from Participants:
Chordoma / Vertebral Chordoma -
Other differentials considered along with some of the above stated diagnosis were liposarcoma (primary/metstatic) and chondrosarcoma.
Vimentin - strongly positive
Positive staining for mucicarmine and Alcian blue with and without hyaluronidase (pH 2.5)
Microscopy: The lesion showed sheet-like proliferation of notochord-like tissue, which was composed of large cells with cleared cytoplasm. The cells were tightly compacted with well defined borders. In most of the cells the cytoplasm appeared as univacuolated space, while in others there was multivacuolation (physaliferous like). Some cells showed wisps of granular cytoplasm within an otherwise clear background. In some areas the cells fused to form a microcystic space. The nuclei were bland with even finely dispersed chromatin. Most of the cells displayed a small nucleolus. There was no evidence of nuclear atypia or mitosis ( as observed in H&E slides ). There was a conspicuous absence of bone destruction. And the demarcation between the lesion tissue and the uninvolved marrow was sharp, and the lesion had pushing borders. No mucinous matrix with cords or strands of cells was apparent. In our view, there was no evidence of a metastatic process, that may have resulted from an unknown primary.
We believe that this lesion represents a benign notochordal tissue and not a chordoma. Such lesions have been variously termed as Giant notochordal rest, Notochordal hamartomas or Ecchordosis physaliphora ( when present at the base of the skull ). We base this diagnosis on the following facts;
- Absence of bone destruction or soft
tissue extension by radiological studies.
We know of no intraosseous chordoma, which at the time of diagnosis DID NOT demonstrate either bone destruction or gross soft tissue invasion. All chordomas demonstrate radiological evidence of bone destruction either on plain roentgenograms, CT scans or MRI studies.
It should be noted however that only a handful of these "benign notochordal rest" cases have been reported (current case is our 2nd in house case ), and there are those who believe that such cases actually represent early stage chordomas. Only time and the publication of additional cases will confirm or refute these positions. As far as this case was concerned, it was our view that the prognosis in this patient was excellent, since the lesion was completely excised. It is close to two years, and this patient is alive and doing very well when he was seen at last follow up.
- The American Journal of Surg Path 27(3); 396-406, 2003
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