En bloc resection of primary sacral tumours β margins determine survival
- Chordoma is radioresistant and chemoresistant at conventional doses β wide en bloc resection is the only reliably curative treatment
- Biopsy must be posterior midline transtumoural so the tract can be excised en bloc β NEVER transrectal or transperitoneal
- Neurological deficit is dictated by which roots are sacrificed, not by the bony level per se β preserve bilateral S2 and at least one S3 where oncologically safe
- Total sacrectomy disconnects spine from pelvis: reconstruct with lumbopelvic screw-rod fixation plus anterior column strut (fibula or cage) in a triangulated construct
- Unresectable or high sacral chordoma: definitive carbon-ion or high-dose proton radiotherapy is an established alternative
- βUnilateral root sacrifice at any sacral level generally preserves bladder and bowel through contralateral compensation
- βBilateral S2-S5 loss produces a flaccid, insensate bladder, incontinent bowel and sexual dysfunction β mandatory pre-operative counselling
- βSilastic sheet placed anterior to the sacrum at the first (anterior) stage protects rectum and vessels during posterior osteotomies
In sacral chordoma, an intralesional or contaminated margin roughly doubles local recurrence versus wide/R0 resection, and local recurrence is the strongest predictor of disease-specific death. Never debulk a resectable chordoma.
Posterior midline, transtumoural, image-guided core biopsy with the tract tattooed or marked for en bloc excision. Transrectal biopsy contaminates the rectum and may convert a curable tumour into one requiring abdominoperineal resection β or an incurable one.
Consent must state expected bladder, bowel and sexual outcome for the planned root sacrifice. Trading margin for function is an oncologic error; trading function without consent is a medicolegal one.
Massive haemorrhage (frequently several litres) is the leading intraoperative threat. Anterior-stage ligation of internal iliac and median sacral vessels, cell salvage, staged surgery and a prepared transfusion protocol are mandatory for high resections.
Oncologic Principles and Tumour Biology
Primary sacral tumours amenable to sacrectomy are dominated by three diagnoses:
- Chordoma β the commonest primary malignant sacral tumour; arises from notochordal remnants in the midline, typically in patients in the sixth and seventh decades. Slow-growing, locally destructive, radioresistant and chemoresistant at conventional doses. Physaliphorous cells, brachyury-positive on immunohistochemistry.
- Chondrosarcoma β eccentric rather than midline; also radio- and chemoresistant, so surgical margin is again the decisive variable.
- Giant cell tumour (GCT) β younger patients (third and fourth decades), eccentric, lytic and expansile. Benign but locally aggressive; denosumab and serial embolisation have shifted many sacral GCTs away from sacrectomy toward intralesional surgery or non-operative control, because sacrificing continence for a benign tumour is rarely justified.
Core principle: for chordoma and chondrosarcoma the operation is planned backwards from the margin. The bony osteotomy level, the roots sacrificed, the amount of gluteus maximus and pelvic floor taken, and whether rectum must be resected are all dictated by achieving a wide margin β never by the desire to preserve function.
Biopsy
- CT-guided core needle biopsy through a posterior midline transtumoural tract, placed in the line of the future incision.
- The tract is considered contaminated and is excised en bloc with the specimen.
- Never transrectal, transvaginal or transperitoneal β contamination of these routes commits the patient to visceral resection or renders the tumour effectively unresectable with margin.
- Presacral masses discovered on rectal examination should NOT be biopsied through the rectum by non-oncology teams; refer intact to a sarcoma centre.
Anatomy and Neurological Consequences of Root Sacrifice
The sacral roots exit their foramina and merge to form the sacral plexus on pelvic surface of piriformis. The pelvic splanchnic nerves (S2-S4) carry parasympathetic supply to bladder, rectum and erectile tissue; the pudendal nerve (S2-S4) supplies the external sphincters and perineal sensation.
- Bladder
- Normal or near-normal
- Bowel
- Normal or near-normal
- Sexual function / motor
- Preserved
- Overall pattern
- Minimal deficit
- Bladder
- Usually preserved
- Bowel
- Usually preserved
- Sexual function / motor
- Usually preserved; possible ipsilateral motor/sensory loss
- Overall pattern
- Contralateral roots compensate
- Bladder
- Retained in the majority
- Bowel
- Retained in the majority
- Sexual function / motor
- Largely preserved
- Overall pattern
- Best compromise for mid sacrectomy
- Bladder
- Impaired in a substantial minority; many recover useful voiding
- Bowel
- Constipation and impaired evacuation common
- Sexual function / motor
- Variable dysfunction
- Overall pattern
- Counsel for partial deficit
- Bladder
- Flaccid, areflexic, insensate bladder β intermittent self-catheterisation
- Bowel
- Incontinent, insensate bowel β regimented bowel programme or colostomy
- Sexual function / motor
- Erectile and ejaculatory failure; loss of perineal sensation
- Overall pattern
- Complete sacral deficit
- Bladder
- As above
- Bowel
- As above
- Sexual function / motor
- Foot drop, plantarflexion weakness, gait deficit added
- Overall pattern
- Motor deficit superimposed on sphincter loss
Preserve both S2 roots and at least one S3 root and the majority of patients retain socially acceptable bladder and bowel function. Bilateral S2 sacrifice is the functional watershed: below it, continence is lost. Unilateral sacrifice at any level is generally well compensated.
Classification by Level and Approach Selection
Osteotomy below S3 (distal S3 body or S3-S4 junction; both S3 roots or lower may be sacrificed depending on margin).
- Posterior-only, prone, single stage.
- Rectum mobilised bluntly off the anterior sacrum from below via the retrorectal plane after dividing anococcygeal ligament.
- Expect little or no sphincter deficit if S3 is preserved bilaterally or sacrificed unilaterally.
- No spinopelvic reconstruction required β sacroiliac joint intact.
Approach selection summary: posterior-only is feasible when the anterior tumour extent is modest, the rectal plane is free and vascular control from behind is realistic (generally low and mid lesions). Combined staged anterior-posterior surgery is chosen for high/total sacrectomy, bulky presacral disease, rectal involvement (en bloc rectal resection with colostomy), re-resection after contamination, or post-radiotherapy tissue.
Decision Thresholds in Management
- Sacral GCT: first-line is usually denosumab with or without serial embolisation and, if surgery is needed, nerve-sparing intralesional excision; sacrectomy with sphincter sacrifice for a benign tumour only in exceptional, refractory, destructive disease.
- Chordoma/chondrosarcoma, resectable with acceptable morbidity: en bloc wide resection is the standard of care.
- Chordoma requiring bilateral S2 sacrifice or S1 body resection: discuss in a multidisciplinary sarcoma meeting; options are total sacrectomy accepting complete deficit versus definitive carbon-ion or high-dose proton radiotherapy, which achieves durable local control in a meaningful proportion of patients and is favoured for surgically unfit patients, patients declining sphincter loss, and some high sacral tumours.
- Adjuvant radiotherapy: high-dose photon/proton therapy is commonly added for close margins or after any contamination; pre-operative radiotherapy protocols exist in some centres but raise wound risk.
- Recurrent chordoma: re-resection is rarely curative once the field is contaminated; particle therapy and systemic options (brachyury-targeted trials, imatinib historically with modest activity) are considered.
Operative Technique
supine, midline laparotomy (or laparoscopic/robotic in selected centres).
- Mobilise sigmoid colon and rectum; develop the presacral (retrorectal) plane sharply off the tumour pseudocapsule β if rectum is adherent or involved, resect en bloc with the specimen and form a colostomy.
- Vascular control: ligate the median sacral artery and, for high resections, ligate or temporarily control both internal iliac arteries (and lateral/median sacral branches); preserve superior gluteal arteries where possible to protect gluteal flap options. Ligate internal iliac veins with care β presacral venous plexus injury bleeds torrentially.
- Anterior discectomy or partial corpectomy at the planned proximal level (L5-S1 for total sacrectomy); score or start the anterior osteotomies and lateral SI joint cuts.
- Place a silastic sheet (or mesh/spacer) anterior to the sacrum, separating rectum and iliac vessels from the posterior osteotomy plane β the single most protective manoeuvre for the second stage.
- Consider harvesting a pedicled vertical rectus abdominis myocutaneous (VRAM) flap, tucked into the pelvis for retrieval at the posterior stage.
- Close; stage two after physiological recovery.
Complications: Rates, Prevention, Management
- Typical burden
- Blood loss frequently several litres in total sacrectomy
- Prevention
- Anterior vascular ligation, staged surgery, silastic sheet, hypotensive anaesthesia, transfusion protocol
- Management
- Packing, rapid completion of resection (bleeding often stops when specimen is out), interventional radiology
- Typical burden
- 25-40 percent, higher after radiotherapy
- Prevention
- VRAM or gluteal flap, dead-space obliteration, drains, offloading, nutrition
- Management
- Debridement, negative-pressure therapy, secondary flap
- Typical burden
- Common, frequently linked to wound failure and rectal proximity
- Prevention
- Flap cover, consider diverting colostomy in total sacrectomy, antibiotic protocol
- Management
- Debridement, hardware retention early / removal after fusion, prolonged antibiotics
- Typical burden
- Follows thecal sac ligation
- Prevention
- Double-ligature or watertight oversew of sac, fibrin sealant, layered closure
- Management
- Lumbar drain, re-exploration and repair if persistent
- Typical burden
- Substantial in long-term total sacrectomy survivors
- Prevention
- Triangulated anterior strut plus lumbopelvic rods, biological fusion mass
- Management
- Observe if fused and asymptomatic; revise if painful instability
- Typical burden
- Deficit is planned by root sacrifice; unplanned injury to preserved roots also occurs
- Prevention
- Neuromonitoring, sharp division only of intended roots
- Management
- Intermittent catheterisation, bowel programme, sexual health input, ankle-foot orthosis for L5/S1 loss
Injury to the presacral venous plexus during blunt rectal mobilisation causes bleeding that is diffuse, valveless and cannot be suture-ligated. Control with packing, thumbtack occlusion or muscle-fragment welding β and prevent it by staying in the correct retrorectal fascial plane and by anterior-stage vascular control.
BLOCKOrder of the posterior stage
Hook:You BLOCK the tumour's escape β every step protects the en bloc margin.
2-3-4 ruleRoot sacrifice outcomes
Hook:Count the bilateral roots you keep: two is the watershed for continence.
Guidelines, Registries & Global Practice
- Epidemiology: chordoma incidence is roughly one per million per year worldwide; sacrococcygeal disease accounts for about half of chordomas, with male predominance and peak in the sixth to seventh decades. Sacral chondrosarcoma and GCT are rarer at this site.
- Society guidance: ESMO/EURACAN and Chordoma Global Consensus Group guidance emphasise referral of presacral masses to sarcoma centres before biopsy, trocar/core biopsy through a resectable posterior tract, en bloc resection with wide margins as standard for chordoma, and high-dose particle therapy (proton or carbon-ion) as definitive treatment where resection is not feasible or declined. NCCN bone tumour guidance mirrors these principles. BOA/BSG sarcoma pathways in the UK mandate management within designated sarcoma networks.
- Registries and multicentre series: there is no arthroplasty-style implant registry for sacrectomy; evidence rests on multicentre collaborations β AOSpine Knowledge Forum Tumour primary spinal tumour cohorts, Rizzoli Institute and Memorial Sloan Kettering chordoma series, and Japanese and Italian carbon-ion cohorts (NIRS/QST Chiba, CNAO Pavia).
- Practice variation by resource setting: carbon-ion facilities exist in only a handful of countries (Japan, Germany, Italy, Austria, China), so surgical resection remains the only curative option in most of the world; in low-resource settings, staged surgery, flap availability and intensive-care capacity constrain total sacrectomy, and non-operative palliation or referral abroad is common for S1-level disease. Denosumab access similarly determines whether sacral GCT is managed medically or surgically.
Controversies & Areas of Uncertainty
- Surgery versus particle therapy for high sacral chordoma: no randomised comparison exists; carbon-ion cohorts show competitive control without sphincter sacrifice, but long-term insufficiency fractures, nerve toxicity and salvage difficulty after radiotherapy failure remain concerns.
- Rigid spinopelvic reconstruction versus no reconstruction after total sacrectomy: rigid constructs restore load transfer but carry high hardware failure and infection rates; some series report acceptable ambulation without reconstruction.
- Extent of nerve sacrifice for margin: whether a planned marginal (rather than wide) plane over preserved S2/S3 roots is oncologically acceptable in selected chordomas is debated.
- Cell salvage and tumour surgery: traditionally avoided; leucocyte-depletion filters and irradiation of salvaged blood are used in some centres.
- Denosumab in sacral GCT: optimal duration, rebound after cessation, and whether it should replace surgery entirely in the sacrum remain unresolved.
- Prophylactic colostomy in total sacrectomy: reduces perineal wound soiling but adds morbidity; practice varies widely.