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Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets.

ABSTRACT: BACKGROUND:Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures. METHODS:A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospective trials. RESULTS:A synthesis of the literature is presented, including sections on pathology, treatment, molecular genetics, challenges, and future directions. CONCLUSION:Beyond an understanding of the current treatment paradigms for skull base chordomas, the reader gains insight into the collaborative approach applied to orphan diseases, of which chordomas is a prime exemplar.

SUBMITTER: Di Maio S

PROVIDER: S-EPMC3683175 | biostudies-literature | 2013

REPOSITORIES: biostudies-literature

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Converging paths to progress for skull base chordoma: Review of current therapy and future molecular targets.

Di Maio Salvatore S Kong Esther E Yip Stephen S Rostomily Robert R

Surgical neurology international 20130601

<h4>Background</h4>Chordomas of the skull base are rare locally aggressive neoplasms with a predilection for encapsulating critical neurovascular structures, bony destruction and irregular growth patterns, and from which patients succumb to recurrence and treatment failures.<h4>Methods</h4>A review of the medical literature is performed, using standard search engines and identifying articles related to skull base chordomas, surgery, radiation therapy, chemotherapy, molecular genetics, and prospe ...[more]

PMID: 23776758

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