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Five years following his initial presentation, surveillance imaging revealed a new asymptomatic lung mass for which the patient underwent thoracotomy and resection of the mass. Histological examination of the lung mass revealed findings consistent with a de-differentiated chordoma, confirming extraneural metastasis from the original tumor without evidence of local recurrence. Chest wall and scalp metastases subsequently developed, and the patient was started on an adjuvant chemotherapy regimen that included imatinib and rapamycin followed by subsequent nivolumab and an EZH2 inhibitor for recurrent, disseminated disease. Despite this patient's remote and distant metastases, primary gross-total resection for chordoma remains a critical treatment objective, followed by proton beam radiotherapy. This case illustrates the importance of interval posttreatment imaging and the emerging potential to treat chordoma with molecularly targeted therapies.<\/jats:p>","DOI":"10.3171\/2017.1.peds16549","type":"journal-article","created":{"date-parts":[[2017,3,17]],"date-time":"2017-03-17T13:59:17Z","timestamp":1489759157000},"page":"531-537","source":"Crossref","is-referenced-by-count":6,"title":["Metastatic clival chordoma: a case report of multiple extraneural metastases following resection and proton beam radiotherapy in a 5-year old boy"],"prefix":"10.3171","volume":"19","author":[{"given":"Martin J.","family":"Rutkowski","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harjus S.","family":"Birk","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew D.","family":"Wood","sequence":"additional","affiliation":[{"name":"Pathology, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arie","family":"Perry","sequence":"additional","affiliation":[{"name":"Pathology, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Theodore","family":"Nicolaides","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"},{"name":"Pediatrics, University of California, San Francisco, California"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher P.","family":"Ames","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nalin","family":"Gupta","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery,"},{"name":"Pediatrics, University of California, San Francisco, California"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-peds16549","series-title":"Sao Paulo Med J","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1590\/1516-3180.2014.1325628","article-title":"Natural history and surgical treatment of chordoma: a retrospective cohort study","volume":"132","author":"Aguiar J\u00fanior S","year":"2014","unstructured":"Aguiar J\u00fanior S, Andrade WP, Baiocchi G, Guimar\u00e3es GC, Cunha IW, Estrada DA, : Natural history and surgical treatment of chordoma: a retrospective cohort study. 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