Current Management of Sacral Chordoma

Daryl R. Fourney M.D., F.R.C.S.C., Ziya L. Gokaslan, M.D.

Disclosures

Neurosurg Focus. 2003;15(2) 

In This Article

Abstract and Introduction

Abstract

Sacralchordomas are relatively rare, locally invasive, malignant neoplasms. Although metastasis is infrequent at presentation, the prognosis for patients with chordoma of the sacrum is reported to be poor and attributable in most cases to intralesional resection. The value of adjuvant treatment is uncertain, and resection remains the primary mode of treatment. Chordomas are difficult to excise completely, but recent improvements in imaging and surgical techniques have allowed surgeons to perform more frequently en bloc sacral resections with wide surgical margins. The technical challenges of such operations, and the functional costs for the patient (with respect to anorectal and urogenital dysfunction) are significantly increased when the tumor involves high sacral levels. The authors review the clinical presentation and natural history of sacral chordoma and discuss the current treatment techniques and outcomes.

Introduction

Chordoma is a rare malignant bone tumor that arises from ectopic rests of notochordal tissue.[43] Restricted to the axial skeleton, it has a predilection for the sacrum (50%), skull base (35%), and the mobile segments of the spine (15%).[4,24,33,39] Sacral chordoma occurs almost twice as frequently in men compared with women and is uncommon in individuals younger than 40 years of age.[2,37]

Chordomas are typically slow growing but locally aggressive. Significant extracompartmental growth is often seen by the time of diagnosis. Most sacral chordomas present as surgical Stage IB[14] with anterior extension into the pelvis.[5,11,35] The tumor often displaces but does not invade the rectum, because the tough periosteum and presacral fascia resist the transgression of disease.[35] Metastasis is usually a late event.[9,24]

The relative rarity and slow growth of these tumors account for why most large clinical studies over many years have been based on cases collected involving variable treatments.[24,33,45] Authors of early studies[9,45] reported very high local recurrence rates for sacral chordoma treated with traditional surgical debulking and radiotherapy. These patients were condemned to progressive deterioration and pain from continued tumor growth, deteriorating neurological function, pressure sores, and infection. Local recurrence of sacral chordoma is associated with a 21-fold increased risk of tumor-related death.[4]

Techniques to allow the en bloc resection of sacral tumors, along with a wide margin of uninvolved tissue, were pioneered in the 1960s and 1970s.[27,38] Local control of disease has significantly improved with the advent of more aggressive surgery;[3,4,24,38] however, major sacral resections are associated with high morbidity rates. Sacrifice of the sacral nerve roots produces varying degrees of bladder, bowel, and sexual dysfunction.[6,31,40] High sacral amputation may jeopardize lumbopelvic stability,[23] and total sacrectomy requires stabilization of the dissociated lumbar spine and pelvis.[17,23]

The purpose of this article is to review the clinicopathological manifestations of sacral chordoma and to discuss the current surgical and adjuvant treatment options for this condition.

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