Chordoma of the mobile spine and sacrum: clinical management and prognosis

Abstract

Background: Chordomas are rare malignant tumors of the spinal column often afflicting the upper cervical spine and sacrum. There are few large single center series on chordomas due to its rarity. The purpose of this study is to report a single center's experience with the operative and non-operative management of chordomas. Methods: We evaluated our institution's pathology database from 1994 to 2016 to identify patients diagnosed with chordomas. Inclusion criteria were chordomas of the mobile spine and sacrum. Exclusion criteria were chordomas of the clivus and resection performed at another institution. We collected patient demographics as well as the type of resection performed, intra-operative complications, wound complications and recurrence/prognosis. Results: We identified 18 patients diagnosed with chordomas at our institution, and 12 met our inclusion criteria. There were four females and eight males with an average age of 64 [32-87] years. All patients were Caucasian and 10 of the 12 had surgery. Of the two patients with sacral chordomas that did not have surgery, one received chemotherapy and the other did not elect for any treatment. One is alive 161 months following diagnosis and the second died 96 months following diagnosis. Five of the lesions were in the mobile spine (one cervical, two thoracic, two lumbar) and seven were in the sacrum. Six patients underwent an en bloc resection [two via total en bloc spondylectomy (TES)]. Average length of follow up is 60 [3-161] months and eight of 12 patients are alive at latest follow up. Intraoperative complications included cardiac arrest, pleural tear, and excessive blood loss (8 L). Two patients, with sacral chordomas, had wound complications. Recurrence occurred in one patient with piecemeal resection and one patient with incomplete resection had postoperative metastatic lesions to the liver and lung. Conclusions: We found less recurrence in patients managed with an en bloc resection as opposed to piece meal or intralesional resections. Sacral chordoma patients had higher wound complication rates as compared to chordomas of the mobile spine. The long life expectancy of non-surgically managed patients underscores the indolent nature of chordomas.

Surgical treatment of primary mobile spine chordoma

“…Several studies have examined the outcomes of mobile spine chordomas, but other series mixed recurrent and primary tumors as well as those in the sacrum and skull base 6,8,14‐24 . We sought to report a focused series of chordomas treated surgically at index presentation in the mobile spine with a minimum 5‐year follow‐up.…”

Sacral and thoracic chordoma with pulmonary metastases: A case report and review of the literature

“…Therefore, chordoma resection is associated with a high risk of morbidity and mortality. In addition, the epidemiology of chordoma is unclear ( 9 ), particularly as regards its incidence rates and the most frequently affected parts of the axial skeleton ( 10 , 11 ). This is clinically significant, since the symptoms and treatment strategies of chordoma largely depend on its anatomical location ( 12 , 13 ).…”

Surgical treatment of giant chordoma in the thoracic spine combining thoracoscopic and posterior spinal surgery

“…[1] Clinically, the tumor may be asymptomatic but usually presents with symptoms related to compression or involvement of adjacent structures and organs, including the trachea, esophagus, pleura cavity and spinal cord. [1,2,9–12] The characteristics of thoracic chordomas have not yet been fully clarified. Moreover, there are still no defined treatment protocols for thoracic chordomas due to the paucity of these patients.…”

“…[1,19] Clinical studies looking at chordomas to the mobile spine is lacking due to the extremely low incidence rate. Based on our review of the case reports on PubMed, [1–5,9–24] chordomas of the thoracic spine is slightly more common during the fourth and fifth decades of life for the sporadic form. [1–5,9–24]…”