Sacral chordoma - Clinical Tree

Introduction

Chordoma is a malignant tumor of the bone and should be always considered when a midline tumor of the axial skeleton is found. The majority of chordomas affect the sacral region, representing 49% of all the cases. There is a modest male prevalence and a peak incidence between the fifth and seventh decades of life. Chordomas follow a slow progressive course, with aggressive local invasion and metastasis. Patients usually present with an advanced disease, and the symptoms usually result from neurological compression or invasion to adjacent organs. Perineal pain and neurological deficits are often reported. Other symptoms include constipation, urinary incontinence, and rectal bleeding. The best strategy to reduce recurrence and improve long-term prognosis is total resection with wide margins spanning surrounding healthy tissue. However, the close proximity to neural and pelvic structures decreases the feasibility for obtaining negative margins without serious morbidity, such as sexual dysfunction and bowel incontinence. High-dose radiotherapy (60–70 Gy) can be used as either adjuvant therapy or main treatment when operative management is not possible. Despite this, chordomas exhibit significant resistance to radiotherapy and chemotherapy, and recurrence occurs in virtually all cases. Complete surgical resection for local sacral recurrences is recommended in the literature. In this chapter, we discuss the case of a middle aged man with a history of bilateral posterior thigh pain whose symptoms originated from a large anterior sacral space occupying lesion.

Example case

Chief complaint: leg pain and urinary incontinence
History of present illness: This is a 57-year-old male patient with a history of hypertension and coronary artery disease who presented with bilateral posterior thigh pain for 3 to 4 months and new urinary incontinence. The patient underwent a magnetic resonance image of the lumbosacral spine that showed a large mass occupying the upper sacral segment with soft tissue involvement. ( Fig. 60.1 ).
Medications: amlodipine, aspirin
Allergies: no known drug allergies
Past medical and surgical history: hypertension, coronary artery disease
Family history: noncontributory
Social history: no smoking or alcohol
Physical examination: awake, alert, and oriented to person, place, and time; cranial nerves II–XII intact; bilateral deltoids/triceps/biceps 5/5; interossei 5/5; iliopsoas/knee flexion/knee extension/dorsi, and plantar flexion 5/5
Reflexes: 2+ in bilateral biceps/triceps/brachioradialis with negative Hoffman; 2+ in bilateral patella/ankle; no clonus or Babinski; sensation diminished in perianal area

	Stefano Boriani, MD Orthopaedic Surgery IRCCS Istituto Ortopedico Galeazzi, Milan, Italy	Ziya L. Gokaslan, MD Tianyi Niu, MD Neurosurgery Brown University Providence, Rhode Island, United States	Sheng-Fu Lo, MD Neurosurgery Johns Hopkins Baltimore, Maryland, United States	Claudio Yampolsky, MD Neurosurgery Hospital Italiano de Buenos Aires Buenos Aires, Argentina
Preoperative
Additional tests requested	CT-guided biopsy confirming chordoma	CT sacrum and pelvis	CT chest/abdomen/pelvis CTA abdomen/pelvis Upright scoliosis x-rays CT-guided biopsy with guarded needle	CT L-spine MRI L-spine STIR CTA abdomen/pelvis Angiogram and potential embolization CT-guided biopsy
Surgical approach selected	En bloc resection or carbon ion therapy; for surgery: Stage 1: anterior midline laparotomy and L5 transverse osteotomy Stage 2: posterior L2-sacrum fusion	Stage 1: anterior L5-S1 discectomy with colostomy Stage 2: L2-pelvic instrumentation, L5-S1 complete facetectomy, sacrectomy, hemipelvectomy	Stage 1: anterior midline laparotomy, presacral tumor dissection, L5 transverse osteotomy, VRAM flap elevation Stage 2: posterior L4-5 laminectomy with thecal sac ligation, L5 transverse vertebral body and lateral sagittal osteotomy, L5 hemivertebrectomy and total sacrectomy for en bloc removal of tumor, L3-pelvis fusion, pelvic ring reconstruction, VRAM flap closure	Transabdominal L5 and sacral transverse osteotomy and sacrectomy with L3-iliac fusion
Goal of surgery	En bloc resection with negative margins	En bloc resection, reconstruction/stabilization of spine	En bloc resection with negative margins	En bloc resection with negative margins, fixation
Perioperative
Positioning	Stage 1: supine Stage 2: prone	Stage 1: supine Stage 2: prone on Jackson table	Stage 1: supine Stage 2: prone on Jackson table, with pins	Stage 1: supine Stage 2: prone
Surgical equipment	Stage 1: vascular and abdominal surgeon	Stage 1: IOM (MEP/SSEP/EMG), surgical navigation, silastic sheath Stage 2: IOM (including sphincter), navigation, ultrasonic bone scalpel	IOM (EMG) Ultrasonic bone scalpel Surgical navigation	IOM Fluoroscopy Surgical navigation
Medications	None	None	Tranexamic acid	None
Anatomical considerations	Dural sac, aorta and inferior vena cava bifurcation, hypogastric arteries and veins, piriformis, rectum	Stage 1: great vessels, peritoneal contents, iliac vessels, lumbosacral plexusStage 2: thecal sac, bilateral L5 nerve roots	Iliac vessels, middle sacral vessels, superior gluteal vessels, rectum, descending colon, ureters, L5 nerve roots	Sacrum, pelvis, pars lateralis, ventral ligamentous complex, aorta, iliac arteries, sacral middle artery, lateral sacral arteries, sacral plexus
Complications feared with approach chosen	CSF leak, arterial or venous bleeding, tumor breach, rectal perforation	Tumor breach, injury to vascular structures, unintended injury to nerves, CSF leak	Catastrophic bleeding, colonic perforation, ureteral injury	Vascular injury, nerve root injury
Intraoperative
Anesthesia	General	General	General	General
Exposure	Stage 1: L5/iliac Stage 2: L2-sacrum	Stage 1: L5-S1 Stage 2: L2-sacrum	Stage 1: L5-sacrum Stage 2: L3-pelvis	L5-sacrum
Levels decompressed	Stage 1: L5 Stage 2: L4-5	Stage 1: L5-S1 Stage 2: L2-L5	Stage 1: L5 Stage 2: L4-5	L5-S1
Levels fused	Stage 1: none Stage 2: L2-sacrum	Stage 1: none Stage 2: L2-sacrum	Stage 1: none Stage 2: L3-pelvis	L3-iliac
Surgical narrative	Stage 1: vascular surgeon and abdominal surgeon, midline laparotomy, vascular surgery to free great vessels/iliac vessels, ligate both hypogastric arteries and veins, anterior L5 osteotomy and start bilateral iliac osteotomiesStage 2 (same day): prone position, Mercedes-like incision, subperiosteal dissection, L4–5 laminectomy, ligate thecal sac at L4–5 level and transect above L5 nerve roots, full tumor release making sure to leave healthy tissue all around, cut piriformis muscle bilaterally as far as possible, cut the ligament from the coccyx and the sacrospinous and sacrotuberous ligaments, complete osteotomies of L5 and iliac wings, en bloc removal, Varga-type	Stage 1: position supine, IOM baseline, midline laparotomy, vascular surgery to free great vessels/iliac vessels and expose lower lumbar spine and anterior sacral pelvis, plastics to harvest VRAM, colorectal surgery performs colectomy, attach surgical navigation and perform intraoperative CT, dissect bilateral L5 nerve roots to lumbosacral plexus, dissect medial to psoas muscles bilaterally and free up L4 and L5 contribution to plexus, L5-S1 anterior annulotomy and discectomy making sure to disconnect lateral annula, score medial pelvis just lateral to SI joints, place silastic sheath to protect vessels and nerves from tumor, VRAM placed into pelvis and incision closedStage 2 (2 days	Preoperative placement of ureteral stents, bowel prep, IVC filterStage 1: position supine, anterior midline laparotomy by vascular surgery down to presacral space and lumbosacral junction, mobilize aortic and IVC bifurcation / middle sacral vessels/bilateral internal iliac vessels/external iliac vessel down to pelvis, identify L5 nerve roots and dissect off of tumor, dissect mesorectum and descending colon off of tumor with colorectal surgery with possible diverting colostomy if tumor adherent/infiltrative or cannot obtain adequate exposure, transverse cut into L5 vertebral body above tumor through ALL and down to PLL, place silastic sheath between tumor and external iliac	Stage 1: GI or general surgeon to perform transabdominal incision via an infraumbilical incision, mobilize aorta and IVC as well as middle and lateral sacral vessels, expose L5 and sacrum, osteotomy guided by surgical navigation through L5 and sacrum, complete radical resection, placement of pedicle screws and L3–5 with iliac screws, place rods for L3-iliac fusion
	reconstruction, place pedicle screws at L2-4, connect pedicle screws to U-bend rod fixed by double screw in each iliac wing, femoral shaft allograft combined with titanium cage interposed between iliac wings and L5 or possible 3D printed titanium prosthesis, layered closure	later): position prone, baseline IOM, expose L2- sacrum, intraoperative navigation and CT scan that is fused to preoperative MRI, place bilateral L2-4 pedicle screws and pelvis screws, cannulate and tap L5 pedicle screws, L5 laminectomy and facetectomy, complete posterior L5-S1 discectomy, thecal sac tied off at L5-S1 with silk ties and divided, drill the pelvis just lateral to the SI joint using navigation, complete bony disconnection with osteotome, dissect sacrum down to the most inferior aspect and disconnect muscle attachments, rotate specimen to deliver it, protect L4 and L5 contribution to the plexus, remove specimen en bloc, inspect the specimen to ensure there is no breach, remove silastic sheath, place L5 pedicle screws, femur shaft sized in placed with ilium-femur-ilium screw at normal sacral promontory position, fibula grafts are sized and placed in effect and anchored between inferior aspect of L5 vertebral body and medial remaining pelvis and secure in place with screws in a V-shape, four-rod construct is placed with each of the rods secured using pelvic screws and multiple side-to-side connectors, place horizontal cross rod between left and right pelvic screws, VRAM is pulled out to fill defect, decorticate and place allograft on all bony surfaces, plastics surgery to close in anatomical layers with drains	vessels and L5 nerve roots, elevation of epithelialized VRAM by plastic surgery to close off dead space, plastic surgery closure of wound Stage 2 (next day): position prone, midline posterior incision, subperiosteal dissection L3-5 and sacrum and leave gluteal muscles attached to sacrum, expose PSIS and ilium, L4-5 laminectomy and L5 inferior facetectomy, skeletonize L5 nerve roots proximally, ligate thecal sac below L5, attach navigation array and intraoperative CT scan, CT-guided sagittal cuts lateral to SI joints and tumor bilaterally through ilium using bone scalpel, identify transverse osteotomy across L5 from stage 1, complete osteotomy through PLL and lateral vertebral body to disconnect L5, detach pyriformis muscle lateral to tumor and ligaments while avoiding injury to underlying viscera/gluteal vessels/sciatic nerve, rotate tumor specimen with laminar spreader, bluntly dissect mesorectum and colon away from tumor making sure to preserve tumor capsule, detach coccygeus muscle inferior to tumor and anococcygeal ligament, deliver tumor en bloc, examination of specimen and resection cavity to ensure R0 resection, floor surgical field with sterile water to lyse tumor, lateral x-ray to confirm detachment and alignment, reposition pelvis using bolsters to match preoperative lumbosacral pelvic parameters on scoliosis x-rays, confirm with x-ray, place bilateral L3-5 pelvic screws and
			double iliac screws, fashion structural femoral allograft to wedge against lateral osteotomies for pelvic ring reconstruction, horizontal rods across iliac screws with double vertical rods up to L3 with cross connectors for four-rod reconstruction, cable femoral structural allograft to horizontal rods across ilium, examine for CSF leak, decorticate exposed bone surfaces and place morselized allograft, identify VRAM from stage 1 and obliterate sacral dead space to prevent bowel herniation, plastic surgery closure
Complication avoidance	Multidisciplinary team approach, free anterior border to tumor before posterior, ligate thecal sac above L5 nerve roots, en bloc resection, Varga-type reconstruction, femoral shaft allograft with titanium cage to reconstruct defect	Multidisciplinary team approach, silastic sheath to protect nerves and vessels, VRAM to fill dead space, surgical navigation tow staged approach, en bloc resection, four-rod construct, plastics surgery closure	Preoperative placement of ureteral stents/bowel prep/IVC filter, staged procedure, multidisciplinary care, preserve L5 nerve roots, silastic sheath to protect vessels for posterior portion of surgery, VRAM flap to close dead space, surgical navigation, flood field with water to lyse microtumor cells,	Preoperative angiogram and possible embolization, general surgery to perform the approach, surgical navigation
Postoperative
Admission	ICU	ICU	ICU	ICU
Postoperative complications feared	CSF leak, wound dehiscence, hematoma, wound infection	CSF leak, wound dehiscence, wound infection, hardware failure	CSF leak, wound dehiscence, L5 nerve root injury, medical complications, lower extremity vascular insufficiency	Vascular injury, nerve root injury
Anticipated length of stay	12–14 days	7 days	5–7 days	4–5 days
Follow-up testing	MRI every 6 months for 5 years	Non-weight bearing for 6 weeks MRI sacrum/pelvis prior to discharge Standing x-rays 3 months after surgery	CT L-spine within 48 hours of surgery MRI L-spine within 48 hours of surgery Bilateral lower extremity Dopplers	CT L-spine within 48 hours of surgery MRI L-spine within 48 hours of surgery
Bracing	None	None	None	None
Follow-up visits	2 weeks and 6 months after surgery	2 weeks and 3 months after surgery	2 weeks after surgery	7 days and 2 weeks after surgery
Radiation therapy for STR	Carbon ion	Proton beam	Proton beam or stereotactic radiosurgery	External beam radiation
Radiation therapy for GTR	Observation	Observation	Proton beam or stereotactic radiosurgery	Observation

CSF , Cerebrospinal fluid; CT , computed tomography; CTA , computed tomography angiography; EMG , electromyography; GTR , gross total resection; ICU , intensive care unit; IOM , intraoperative monitoring; IVC , inferior vena cava; MEP , motor evoked potential; MRI , magnetic resonance imaging; PSIS , psoterior superior iliac spine; SI , sacroiliac; SSEP , somatosensory evoked potential; STIR , short tau inversion recovery; STR , subtotal resection; VRAM , vertical rectus abdominis myocutaneous.

Differential diagnosis

▪
Chordoma
▪
Chondrosarcoma
▪
Giant cell tumor
▪
Plasmacytoma
▪
Glioma
▪
Metastatic tumor
▪
Ewing sarcoma
▪
Chronic infections (tuberculosis, fungus)

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Introduction

Differential diagnosis

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