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Chordoma

DEFINITION AND PATHOGENESIS

  • Arises from embryonic notochord rests

IMPORTANCE

  • 1-4% of all primary malignant tumors of bone
  • Distribution
    • 50% of tumors of the sacrococcygeal region (excluding metastases)(most common primary malignancy of the sacrum)
    • 50% in sphenooccipital region (clivus)
    • 35% in the sacrum
    • 15% in other vertebra
  • Low grade (most stage IA or IB)

CLINICAL FEATURES

  • Mean age 56 years (sacral), 47 years (other vertebra)
  • 75% have back pain > 6 months
  • ± buttock pain
  • 20% have rectal dysfunction
  • ± constipation
  • ± urinary frequency
  • Presacral mass in all sacral lesions palpable on rectal exam (DON'T BX TRANSRECTUM!)

RADIOLOGIC FEATURES

  • 40-80% calcification: amorphous and peripheral
  • ↑ activity on bone scan
  • MRI superior to CT for local study
  • Metastases to lungs, soft tissues, LN, bone, liver

GROSS PATHOLOGY

  • Mucinous, gelatinous with areas of visible hemorrhage

HISTOLOGIC FEATURES

  • Physalipherous cell, with a large vaculated ("bubbly") cytoplasmic component (found in 90% of tumors)
  • "Pink cell" containing pink cytoplasm
  • Cells may be arranged in sheets or cords
  • Immunohistochemistry
    • + mucicarmine, PAS, and alcian blue staining because of hyaluronic acid
    • + S-100 staining
    • + cytokeratin
  • EM: cytoplasmic vacuoles → glycogen

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Liposarcoma
  • Chondrosarcoma
  • Signet ring-cell carcinoma
  • Metastatic carcinoma (eg, prostate)(when physaliphorous cells are sparse)
  • Ecchordosis (Ecchordosis physaliphora)

DISEASE COURSE AND TREATMENT

  • Complete resection via posterior, simultaneous or sequential anterior/posterior approaches
  • Proximal extent of sacral tumors the most valuable prognostic indicator (S1 worst)
  • En bloc excision even if marginal is tx of choice
  • Has been reported in the mid-cervical spine
  • Bladder and bowel funtion will be impaired if 2nd sacral roots have to be sacrificed, if both spared ? normal control (only certain if both S3 roots preserved)
  • Must include rectum in resection if transrectal bx done
  • ± XRT (≥50 Gy)(proton beam therapy has been advocated)
  • Local recurrence 64% if contaminated margin
  • Too slow growing for chemotx
  • 50-85% 5 year survival mainly due to local structure involvement; 28-43% 10 yr survival
  • Distant metastases may appear >10yrs after the initial resection (lungs, bone, skin, brain, viscera)

SPECIAL CONSIDERATIONS

DEDIFFERENTIATED CHORDOMA

  • Development of metastatic disease <1yr after wide resection of primary with a 90% rate of distant spread
  • High grade sarcomatous elements present
  • Higher aneuploid DNA content
  • Tx with chemotx indicated (ifosfamide)

PARACHORDOMA

FAMILIAL CHORDOMA

  • Isochromosome 1q as the probable locus involved in susceptibility

REFERENCES

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