
Bone Tumors: Base of Skull Chordoma: The Conundrum of Biology and Morphology
Dr. Benjamin L. Hoch
Assistant Professor of Pathology
Mount Sinai School of Medicine, New York, NY
Tuesday, March 25th, at 4:30 – 5:30 p.m.
Ruhl Student Center, Community Room
ABSTRACT:

Bone tumors in
humans are uncommon neoplasms that often arise in children and adolescents.
Predicting the biological behavior of a given tumor is essential to developing a
definitive treatment plan for the patient which can range from relatively simple
surgical procedures to the amputation of an extremity with combined systemic
chemotherapy. Traditionally, clinical factors, such as the location of a tumor,
and pathologic features, such as the microscopic appearance of the tumor cells
and mitotic activity, have been used to help predict the behavior of a tumor.
Today, understanding the molecular mechanisms of tumorogenesis is essential to
better predicting the behavior of bone tumors and developing customized
treatment strategies including targeted pharmacological therapies. Chordoma is a
malignant bone tumor that accounts for less than 5% of bone neoplasms.
It develops in the sacrum, base of skull, and mobile spine. Chordoma is
believed to arise from embryological remnants of the notochord. As such, this
tumor provides an opportunity to explore the relationship between embryogenesis
and the pathogenesis of bone tumors. In general, chordomas are slow growing
neoplasms that can recur following surgery and result in the death of the
patient by local invasion, or less commonly, by metastatic spread to other organ
systems. Traditional pathological features used to predict tumor behavior have
not been applicable to chordomas.
Therefore, defining the unique relationship between the embryology of the
notochord and chordoma should reveal new molecular markers to be used in
predicting behavior and as potential molecular targets for pharmacological
therapy.

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