Children and Adults With Chordoma

Background:

• Chordomas are a rare tumor with an incidence of 325 new cases per year in the United States. Pediatric chordomas are very rare and comprise only 5% of all chordoma cases.
• The standard therapy for localized primary or recurrent chordomas is surgical resection. However, complete surgical resection is often not possible due to the location of the

chordoma.

-For chordomas that cannot be surgically resected, treatment options are limited. Currently,

no standard therapy approach exists for recurrent chordomas. In addition, there are no FDA approved medical therapies for chordoma.

• The natural history of pediatric and adult chordoma is incompletely understood. Patients with chordoma seek expert advice in the management of their care.
• The NCI has basic and clinical expertise and research interest in chordoma. The planned natural history study as part of the NCI POB Rare Tumor Patient Engagement Network (RTPEN) will allow for comprehensive evaluation and recommendations to these patients while longitudinally collecting clinical, epidemiologic and biological data.

Objective:

-To characterize the natural history of chordoma, including clinical presentation and patterns of disease progression.

Eligibility:

• Subjects with radiographically or histologically documented chordoma.
• Age greater than or equal to 2 years old

Design:

-This protocol is a subprotocol to protocol 19-C-0016: Natural History and Biospecimen

Acquisition Study for Children and Adults with Rare Solid Tumors . After enrollment on the master protocol and undergoing evaluations detailed, participants will be enrolled on this subprotocol specific for chordoma.

-Medical histories will be documented, and participants followed throughout the course of their disease, with particular attention to patterns of disease recurrence and progression, response to therapies, duration of responses and patient reported outcomes. Tumor growth rates will also be calculated throughout the course of the disease.

• INCLUSION CRITERIA:
• Ability of subject or Legally Authorized Representative (LAR) to understand and the willingness to sign a written consent document.
• Subjects with radiographically or histologically documented chordoma
• Age greater than or equal to 2 years old
• Subjects must be enrolled into NCI protocol 19-C-0016: Natural History and Biospecimen Acquisition Study for Children and Adults with Rare Solid Tumors .

Exclusion criteria

-None