Pediatric Metastatic Sacrococcygeal Chordoma Treated with Surgery

Background

Sacrococcygeal chordomas are extremely rare tumors, especially in the pediatric population [1]. These neurogenic tumors arise from the nucleus pulposus (notochord remnant) and account for less than 1% of all primary spinal tumors [2]. The prognosis is poor with death most commonly due to local invasion and recurrence [3] [4]. In the pediatric population, chordomas are believed to behave like a more aggressive variant [2] with shorter survival times and a shorter time to disease metastasis [4]. A review of the literature on pediatric sacrococcygeal chordomas has revealed less than 25 cases, going back to the first case reported in 1910 [5] [6] [7] [8] [9] [10] [11] [12]. [Table1] outlines the important characteristics of each case in the literature for which results were reported. Notably, only 7 patients developed metastatic disease [13] [14] [15] [16] [17] [18] [19], and only 3 patients were found to have metastatic disease at the time of presentation [13] [14] [17].

Table 1 Characteristics of Pediatric Sacrococcygeal Chordomas. Authors Cases Age Metastasis (at presentation Y/N) Treatment Status Argaud, M.R. and Lestrade, A. 18 1 14 mo. liver (Y) surgery dead 82 months Montgomery, A.H. and Wolman, I.J. 19 3 3 yr lungs (Y) supportive dead 9 days after admission 3.5 mo. N surgery non-resectable, dead POD 23 22 mo. N surgery+radiation non-resectable, decrease in size with rad. Rosenqvist, H. and Saltzman, G. 21 1 4 yr lungs (N) rad.+surgery+rad. incomplete resection, local recurrence, repeat resection, dead at 1 year Worthy, T.S. 20 1 5 yr liver (N) surgery ×3+radiation dead 18 months Richards, A.T., Stricke, L. and Spitz, L. 22 2 1 yr3 yr NN radiationsurgery+radiation dead 3 monthsdead 2 months Dutton, R.V. and Singleton, E.B. 23 1 newborn N surgery alive at 17 yrs Prignitz, R. and Tauber, R. 24 1 3 yr lung (Y) none dead 1 month after presentation Garofalo, E., Minerva, A. and Baltieri, G. 25 1 19 mo. inguinal nodes (N) supportive dead 7 days after admission Nix, W.L., Steuber, C.P., Hawkins, E.P. and Stenback, W.A. 26 1 1 day N surgery – Azzarelli, A. 13 1 2 yr – – – Kozlowski, K. 27 1 12 yr lung, bone (N) – – Cable, D.G. and Moir, C. 28 1 12 yr N surgery alive at 6 months

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