Css Meet 2d1s6f

C2 Chordoma a Case Report Dr. M. Ashok Kumar M.S.(Ortho), FSS(CMC Vellore),

Consultant Spine Surgeon, SNHRC, Vellore.

Patient’s information • 27yrs male from Bangladesh, • Manual laborer • Chief complaints: Progressive tightness of both upper and lower limbs- 3weeks. Inability to walk – 2weeks.

H/o presenting illness • • • • • • •

Progressive tightness of all four limbs. Loss of balance on walking. Progressive compromise in hand functions. Dysphagia for solid foods for 4 weeks. Inability to walk for 2weeks. Bowel & Bladder were normal. No constitutional symptoms.

General examination • • • • •

Conscious, oriented Thin built No pressure sores On CBD. Systemic examination – Within normal limits.

Spine examination • Skin- normal • ROM in neck- 15 degrees of flexion, extension, 20 degrees of rotation to either side. • Motor: – Bulk- normal – Tone- increased – Upper limbs- MAS 2 – Lower limbs- MAS 3

Spine examination Sensory examination – Last intact level C3 – C4-C8 reduced by 60% – From T1 reduced by 90% for fine touch.

Deep Tendon reflexes • All DTR were exaggerated, 3+. • B/L plantar- extensor.

Spine examination • • • •

Proprioception, vibration reduced by 50-60%. B/L ankle clonus+. B/L Hoffman’s +. P/R- perianal sensation reduced by 50%. – VAC- intact. – Deep anal sensation- intact.

Radiological examination

Radiological examination

Radiological examination

Radiological examination

Surgical staging Weinstein, Boriani et al

Patient’s image

Spine update Primary Bone Tumors of Spine-Terminology & Surgical Staging, Stefano Boriani et al, Spine Vol 22, N0.9. 1036-1044.

Procedure • Tumour classified as -IB Enneking classification. • Total Enbloc excision not feasible. • Removed by intralesional spondylectomy through 2 stages. • Stage-1: anterior transoral approach • Stage-2 : Posterior approach.

Anterior approach • Transoral, transpalatine approach. • ENT surgeon for surgical access to C2. • Tumor capsule was clearly exposed and incised. • Resected from tip of Dens. • Brownish hard- soft material. • Irrigated, covered with gelfoam.

Anterior approach

Difficulties encountered… • RT feeding- aspiration. • Day-2, sudden dyspnoea with tacchycardia, fever epiodes. • SICU care with repeated suctions. • IV antibiotics for 5 days.

Posterior approach • Midline approach from EOP to C5. • C2 laminectomy and tumour was accessed. • Occipito cervical fixation done - Occipital plate with screws and Cervical lateral mass screws. (JAYON- POCER)

Posterior approach CRANIAL

C1

C3 C4

CAUDAL

Difficulties encountered on postop... • Continuous suction required- tracheostomy. • RT feed was continued. • ICU stay for 3 days and HDU observation 2 days. • Oral feeds patient tolerated 8 days after 2nd surgery.

Post Op X-rays

Post OP CT

Discussion • • • • • • •

Relatively rare, low grade malignancy. Spine- sacro pelvic & skull base. M:F= 1.6:1 Slowly growing, locally invasive. Mets –rare, more common in lungs. Median survival- 6.3yrs. Untreated skull base – survival is 6mon-2yrs.

Atlanto Axial Chordomas • Site, size determines outcome • AA chordomas – anatomical constraints. • Large dural space, tumor enlarges, delayed clinical presentation. • Surgery mainstay of therapy. • Teamwork has a role.

Chemothreapy • Inactive. • Imatinib mesylate- PK inhibitor. • Minimal anti tumor activity.

Magenau JM, Schuetze SM (2008) New targets for therapy of sarcoma. Curr Opin Oncol 20:400–406

Radiotherapy • Radioresistant. • *Tolerance dose of spinal cord lower than curative dose of 90Gy. • # Proton beam radiotherapy favorable results reported. *Casali PG, Stacchiotti S, Sangalli C, Olmi P, Gronchi A (2007) Chordoma. Curr Opin Oncol 19:367–370 # Hug EB, Slater JD (2000) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. Neurosurg Clin N Am 11:627–638

Surgical treatment • Margin free, enbloc excision favored. • AA region enbloc excision difficult. – Complications-vertebral artery injury – Dural injury – Worsening of paraplegia/paraparesis.

• *Total Enbloc Spondylectomy is feasible in mid axial spine. * Leitner Y, Shabat S, Boriani L, Boriani S (2007) En bloc resection of a C4 chordoma: surgical technique. Eur Spine J 16:2238–2242

Anterior approaches for Cervical spine s.no.

Approach

advantage

Disadvantage

1.

B/L high anterior cervical

C2 body

Dens not accessible

2.

Anterior midline Transoral ± transpalatine approach

-Midclivus to C3 upper half. -Upto 2cms lateral

Enbloc excision not possible

3.

Transmandibular approach + anterior cervical approach

Clivus to lower cervical spine

-Aggressive -free flap cover required for posterior pharyngeal wall.

Occipital plate

Occipital plate screws

Occipital screw pullout strength, A biomechanical Investigation of Occipital Morphology Thomas R.Haher et. al,Spine vol 24, No-1, pp5-9

OC2 angle

OC2A

• Biological behavior • Gross pathology

Biological behavior • • • •

Slow growing Long asymptomatic evolution Presents with huge masses. Meticulous excision extremely difficult.

Gross pathology • Low vascular tumor. • Gelatinous jelly like. S.no.

Region

Spreads along

1

Sacral chordoma

Piriformis & Sciatic fossa

2

Lumbar chordoma

Psoas muscle

3

Cervical chordoma

Longus colli muscle

•All these involved muscles to be included in resection. •Jelly nature tumor- seeding all along surgical fieldhigher rate of recurrence.

Gross pathology • Epidural extension worsens scenario. • Margins of possible enbloc resection includes unresectable structures.

Conclusion • Location, biological behavior & confusing diagnostic picture, difficulty in managing. • Treatment outcome depends on site, size. • Surgery remains mainstay. • Achieving a real wide margin –challenge to achieve.

References •

• •

• •

•

Boriani S, Bandiera S, Biagini R, Bacchini P, Boriani L, Cappuccio M, Chevalley F, Gasbarrini A, Picci P, Weinstein JN (2006) Chordoma of the mobile spine: fifty years of experience. Spine 31:493–503 Boriani S, Weinstein JN, Biagini R (1997) Primary bone tumors of the spine. Terminology and surgical staging. Spine 22:1036–1044 Casali PG, Stacchiotti S, Sangalli C, Olmi P, Gronchi A (2007) Chordoma. Curr Opin Oncol 19:367–370 Leitner Y, Shabat S, Boriani L, Boriani S (2007) En bloc resection of a C4 chordoma: surgical technique. Eur Spine J 16:2238–2242 Neo M, Asato R, Honda K, Kataoka K, Fujibayashi S, Nakamura T (2007) Transmaxillary and transman- dibular approach to a C1 chordoma. Spine 32:E236– E239

Suchomel P, Buchvald P, Barsa P, Froehlich R, Choutka O, Krejzar Z, Sourkova P, Endrych L, Dzan L (2007) Single-stage total C-2 intralesional spondylectomy for chordoma with three-column reconstruction. Technical note. J Neurosurg Spine 6:611–618

References • • •

Occipital screw pullout strength, A biomechanical Investigation of Occipital Morphology Thomas R.Haher et. al,Spine vol 24, No-1, pp5-9 An anatomic Study of Thickness of Occipital Bone, Implications for Occipitocervical Instrumentation, Nabil et al, Spine Vol 21, no.15,PP 1725-1730. Upper Cervical Spine Chordoma of C2-3. Liang Jiang et al, Eur Spine J, 2009, 18:293-300.