Surgical Consideration for Adolescents and Young Adults With Cervical Chordoma.
METHODS
Retrospective study.


OBJECTIVE
The aim of this study was to compare the clinical outcomes between adolescent and young adult (AYA) patients and old adult patients with cervical chordoma who were treated surgically and present the surgical consideration for adolescents and young adults with cervical chordoma.


BACKGROUND
With predominance in senior patients, chordoma is distinctively rare in AYAs. Because of the rarity of AYA chordoma, individual case report represents most of the literature on this disease entity on mobile spine and lack of long-term follow up, which leads to the paucity of clinical evidence for treatment planning and prognosis prediction.


METHODS
A retrospective study was conducted to investigate the prognosis of AYA patients with cervical chordoma who were treated surgically. We collected the clinical data of these patients and their older counterparts, and further compared the prognosis of the patients in different age groups. To estimate survival curves, Kaplan-Meier method was used, and significance was assessed using a log-rank test.


RESULTS
Forty consecutive patients with chordoma of the cervical spine treated in our institution were included in the study. Two groups were identified according to age. Group 1 comprised children and adolescents (age ≤ 25 yrs; n = 9) and Group 2 comprised adults (age > 25 years; n = 31). In comparison, Group 1 was featured by significantly higher rate of recurrence and shorter overall survival, although no difference found in the surgical modality between two groups.


CONCLUSIONS
There is a dismal prognosis in young patients with chordoma, and thus support the notion that as radical a total en bloc spondylectomy (TES) of the lesions as possible may benefit the overall survival of these young patients. Although the ensuing neurological deficits may be devastating, it will be worth sacrificing if the life expectancy of these young patients is prolonged.


METHODS
4.
Department of Orthopedic Oncology, Changzheng Hospital, Second Military Medical University, Shanghai, China.
Nanzhe
N
Zhong
Xinghai
X
Yang
Jian
J
Yang
Tong
T
Meng
Cheng
C
Yang
Wangjun
W
Yan
Jianru
J
Xiao
D000293:Adolescent; D000328:Adult; D002648:Child; D002817:Chordoma / Q000453:epidemiology* / Q000601:surgery*; D005260:Female; D006801:Humans; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D009333:Neck / Q000473:pathology; D009364:Neoplasm Recurrence, Local / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D013125:Spinal Neoplasms / Q000473:pathology / Q000601:surgery*; D013131:Spine / Q000473:pathology; D055815:Young Adult
McMaster ML, Goldstein AM, Bromley CM, et al. Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 2001; 12:1–11.
Bas T, Bas P, Prieto M, et al. A lumbar chordoma treated with a wide resection. Eur Spine J 1994; 3:115–117.
Carpentier A, Polivka M, Blanquet A, et al. Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease. J Neurosurg 2002; 97:1070–1077.
Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973; 32:410–420.
Borba LA, Al-Mefty O, Mrak RE, et al. Cranial chordomas in children and adolescents. J Neurosurg 1996; 84:584–591.
Lee J, Bhatia NN, Hoang BH, et al. Analysis of prognostic factors for patients with chordoma with use of the California Cancer Registry. J Bone Joint Surg Am 2012; 94:356–363.
Hoch BL, Nielsen GP, Liebsch NJ, et al. Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 2006; 30:811–818.
Zou MX, Huang W, Wang XB, et al. Prognostic factors in spinal chordoma: a systematic review. Clin Neurol Neurosurg 2015; 139:110–118.
Habrand JL, Datchary J, Bolle S, et al. Chordoma in children: case-report and review of literature. Rep Pract Oncol Radiother 2016; 21:1–7.
Tomita K, Kawahara N, Kobayashi T, et al. Surgical strategy for spinal metastases. Spine (Phila Pa 1976) 2001; 26:298–306.
Ridenour RV 3rd, Ahrens WA, Folpe AL, et al. Clinical and histopathologic features of chordomas in children and young adults. Pediatr Dev Pathol 2010; 13:9–17.
Coffin CM, Swanson PE, Wick MR, et al. Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 1993; 117:927–933.
Yasuda M, Bresson D, Chibbaro S, et al. Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 2012; 35:171–182. discussion 182–3.
Yadav YR, Kak VK, Khosla VK, et al. Cranial chordoma in the first decade. Clin Neurol Neurosurg 1992; 94:241–246.
Iwasa Y, Nakashima Y, Okajima H, et al. Sacral chordoma in early childhood: clinicopathological and immunohistochemical study. Pediatr Dev Pathol 1998; 1:420–426.
Sibley RK, Day DL, Dehner LP, et al. Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature. Pediatr Pathol 1987; 7:287–301.
Wold LE, Laws ER Jr. Cranial chordomas in children and young adults. J Neurosurg 1983; 59:1043–1047.
Wang Y, Xiao J, Wu Z, et al. Primary chordomas of the cervical spine: a consecutive series of 14 surgically managed cases. J Neurosurg Spine 2012; 17:292–299.
Molina CA, Ames CP, Chou D, et al. Outcomes following attempted en bloc resection of cervical chordomas in the C-1 and C-2 region versus the subaxial region: a multiinstitutional experience. J Neurosurg Spine 2014; 21:348–356.
Zhou H, Jiang L, Wei F, et al. Chordomas of the upper cervical spine: clinical characteristics and surgical management of a series of 21 patients. Chin Med J (Engl) 2014; 127:2759–2764.
Rutz HP, Weber DC, Goitein G, et al. Postoperative spot-scanning proton radiation therapy for chordoma and chondrosarcoma in children and adolescents: initial experience at Paul Scherrer Institute. Int J Radiat Oncol Biol Phys 2008; 71:220–225.
Yamazaki T, McLoughlin GS, Patel S, et al. Feasibility and safety of en bloc resection for primary spine tumors: a systematic review by the Spine Oncology Study Group. Spine (Phila Pa 1976) 2009; 34 (22 Suppl):S31–S38.
Hsieh PC, Gallia GL, Sciubba DM, et al. En bloc excisions of chordomas in the cervical spine: review of five consecutive cases with more than 4-year follow-up. Spine (Phila Pa 1976) 2011; 36:E1581–E1587.
Bergh P, Kindblom LG, Gunterberg B, et al. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 2000; 88:2122–2134.
Oppenlander, Maulucci CM, Ghobrial GMME, et al. En bloc resection of upper thoracic chordoma via a combined simultaneous anterolateral thoracoscopic and posterior approach. Neurosurgery 2014; 10:380–386. discussion 386.
Bosma JJ, Pigott TJ, Pennie BH, et al. En bloc removal of the lower lumbar vertebral body for chordoma. Report of two cases. J Neurosurg 2001; 94:284–291.
Meng T, Yin H, Li B, et al. Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center. Neuro Oncol 2014; 17:725–732.
Boriani S, Bandiera S, Biagini R, et al. Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976) 2006; 31:493–503.
Eid AS, Chang UK, Lee SY, et al. The treatment outcome depending on the extent of resection in skull base and spinal chordomas. Acta Neurochir (Wien) 2011; 153:509–516.