Updated: 6/21/2021

Chordoma

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  • summary
    • Chordomas are malignant tumors of primitive notochordal origin that most commonly occur in the sacrum and coccyx. The tumor typically presents in patients > 50 years of age with insidious low back pain with bowel or bladder dysfunction.
    • Diagnosis is made with biopsy showing foamy, vacuolated, physaliferous cells that are keratin positive on histochemical stains. 
    • Treatment is usually wide margin surgical resection with or without radiation.
  • Epidemiology
    • Incidence
      • most common primary malignant spinal tumor in adults
    • Demographics
      • 3:1 male to female ratio
      • usually in patients > 50 years
    • Anatomic location
      • 50% occur in the sacrum and coccyx
      • 35% in spheno-occiptal region
      • 15% in mobile spine
  • Etiology
    • Pathoanatomy
      • forms from malignant transformation in residual notochordal cells
        • resulting in midline location
  • Symptoms
    • Presentation
      • pain
        • insidious onset of pain
        • may be mistaken for low back or hip pain
      • neurologic
        • often complain of bowel or bladder changes
        • sensory deficits rare due to distal nature of tumor
      • gastrointestinal
        • constipation
        • fecal incontinence
    • Physical exam
      • neurologic
        • motor deficits rare because most lesions at S1 or distal
        • bowel and bladder changes are common
      • rectal exam
        • more than 50% of sacral chordomas are palpable on rectal exam
  • Imaging
    • Radiographs
      • often difficult to see lesion due to overlying bowel gas
    • CT
      • will show midline bone destruction and soft tissue mass
      • calcifications often present within the soft tissue lesion
    • MRI
      • bright on T2
      • useful to evaluate soft tissue extension
  • Histology
    • Biopsy
      • transrectal biopsy is contraindicated
    • Gross
      • lobular and gelatinous
    • Histology
      • findings
        • characterized by foamy, vacuolated, physaliferous cell
        • grows in distinct nodules
      • histochemical staining
        • keratin positive
          • important to distinguish from chondrosarcoma, which is not keratin positive
        • weakly S100 positive
  • Differential
    • Differential of Chordomas
      Sacral lesions in older patients
      Keratin stain positive
      Similar Appearance on Xray
      Treated with wide-resection alone
      Chordoma
      Chondrosarcoma
      Metastatic disease
      Lymphoma
      Multiple Myeloma
      MFH
      Secondary sarcoma
      Enchondroma of hand
      Ollier's
      Maffucci's
      Periosteal chondroma
      Osteochondroma (MHE)
      Parosteal osteosarcoma
      Adamantinoma
      Synovial Sarcoma
      Epitheloid sarcoma
      Squamous cell 
  • Treatment
    • Nonoperative
      • radiation treatment
        • indications
          • inoperable tumors
    • Operative
      • wide margin surgical resection +/- radiation
        • indications
          • standard of care in most patients
        • technique
          • must be willing to sacrifice sacral nerve roots to obtain adequate surgical margins
          • add radiation if margin not achieved
        • outcomes
          • long-term survival 25-50%
          • en bloc corpectomy has best chance of local control with spinal lesions
  • Complications
    • Local recurrence
      • 50% local recurrence common
      • some newer evidence that radiation with proton-photon beams may be beneficial for recurrence
    • Loss of bowel/bladder function postoperatively
      • to preserve near normal bowel/bladder function
        • preserve bilateral S2 nerve roots (at least)
        • preserve unilateral S2, S3, S4 roots
  • Prognosis
    • Metastasis
      • metastatic disease in 30-50%
        • occurs late in the course of the disease so long term follow up required
          • may spread to lung and rarely to bone
    • Survival
      • 60% 5-years survival
      • 25% long term survival
      • local extension may be fatal

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Flashcards (6)
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Questions (6)

(OBQ18.86) A 58-year-old male is referred to your clinic for a sacral mass found on a lumbar spine MRI. He brought a pathology report from a biopsy that was performed at an outside hospital that states “primary malignant bone tumor of notochordal remnant cells.” Staging work-up showed no distant disease. Which of the following describes the best treatment strategy for this patient?

QID: 212982
1

Neo-adjuvant chemotherapy, surgical excision, maintenance chemotherapy

21%

(310/1504)

2

Wide surgical excision

65%

(977/1504)

3

Tumor debulking, chemotherapy

6%

(84/1504)

4

Radiation therapy

7%

(101/1504)

5

Observation with serial imaging

2%

(23/1504)

L 3 A

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(SBQ18SP.48) A 47-year-old man presents with 6 months of progressive back pain, especially while sitting in his recliner at home. He's also noted some recent bowel incontinence. Diagnostic tests are performed and shown in Figures A and B. All of the following are true with respect to this diagnosis EXCEPT?

QID: 211630
FIGURES:
1

The primitive pathological cells are similar to those that derive the nucleus pulposis

9%

(165/1796)

2

Metastatic disease is common at the time of presentation

34%

(604/1796)

3

MRI studies are in most cases sufficient for diagnosis without the need for formal biopsy

32%

(582/1796)

4

The primary location of these lesions generally does not lead to lower extremity motor deficits

10%

(171/1796)

5

Treatment consists of surgical resection with or without radiation

14%

(248/1796)

L 5 A

Select Answer to see Preferred Response

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(OBQ08.56) A 55-year-old man complaining of increasing problems with constipation undergoes a biopsy of a mass after a digital rectal exams reveals a fullness. The radiograph and micrograph of the biopsy specimen are shown in Figures A and B, respectively. Immunohistochemistry is positive for keratin and S-100. Following wide surgical resection, oncologic surveillance is necessary due to the high rate of which of the following?

QID: 442
FIGURES:
1

Regional lymph node metastasis

8%

(268/3271)

2

Liver metastasis

2%

(67/3271)

3

Bone metastasis

14%

(448/3271)

4

Local recurrence

69%

(2265/3271)

5

Malignant transformation

6%

(209/3271)

L 2 D

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