Workup/Staging

Question 1

How do pts with chordomas present clinically?

Answer 1

Depends on the site of origin, but pain of a gradual and insidious onset is reported to be the most common presenting Sx regardless of location. Chordomas encroaching on the spinal canal may cause compression of the SC or nerve roots, resulting in neurologic Sx. Chordomas involving the cervical region may cause dysphagia, dysphonia, or Horner syndrome. Chordomas in the sacral region can create nerve root dysfunction as well as obstipation, constipation, and tenesmus. Pts with base of skull chordomas can present with intermittent diplopia, HA, neck pain, or other lower CN findings.

Question 2

Is transrectal Bx a recommended method to obtain tissue Dx for chordoma?

Answer 2

No. Due to a propensity to seed along Bx tracts, transrectal Bx should be avoided so as to prevent spread of chordoma into the rectum. All Bx tracts should be marked and removed in the subsequent Sg.

Question 3

What comprises oncologic staging for chordoma?

Answer 3

H&P, MRI/CT of primary site and MRI spinal axis, CT C/A/P, lab studies, and Bx. PET/CT or bone scan (if PET negative primary) may also be considered.

Question 4

What are the T1-T2 MRI features of chordomas?

Answer 4

T1-T2 MRI features of chordomas:

T1: intermediate to low signal intensity, with heterogeneous enhancement with gadolinium

T2: high, heterogenous signal intensity

Question 5

What are the AJCC 8th edition (2017) TNM stage categories for bone tumors? (Note: Lymphoma and multiple myeloma have separate staging systems.)

Answer 5

Appendicular Skeleton, Trunk, Skull, and Facial Bones

T1: ≤8 cm

T2: >8 cm

T3: Discontinuous tumors in primary bone site

Spine

T1: 1 or 2 adjacent vertebra

T2: 3 adjacent vertebra

T3: ≥4 vertebra, or nonadjacent vertebra

T4a: Extension into spinal canal

T4b: Gross vascular invasion or tumor thrombus in great vessels

Pelvis

T1a: ≤8 cm confined to 1 pelvic segment

T1b: >8 cm confined to 1 pelvic segment

T2a: ≤8 cm confined to 1 pelvic segment with extraosseous extension or 2 segments without extraosseous extension

T2b: >8 cm confined to 1 pelvic segment with extraosseous extension or 2 segments without extraosseous extension

T3a: ≤8 cm in 2 pelvic segments with extraosseous extension

T3b: >8 cm in 2 pelvic segments with extraosseous extension

T4a: SI joint/sacral neuroforamen

T4b: Encasement of external iliac vessels or tumor thrombus in major pelvic vessels

N1: Regional LN mets

M1a: Lung

M1b: Bone or other

Question 6

What is the AJCC stage grouping for bone tumors?

Answer 6

Appendicular Skeleton, Trunk, Skull, and Facial Bones

Stage IA: T1, N0, Grade 1–X

Stage IB: T2–3, N0, Grade 1–X

Stage IIA: T1, N0, Grade 2–3

Stage IIB: T2, N0, Grade 2–3

Stage III: T3, N0, Grade 2–3

Stage IVA: M1a

Stage IVB: N1 or M1b

No AJCC group staging for spine and pelvis.