DEFINITION AND PATHOGENESIS
- Arises from embryonic notochord rests
- 1-4% of all primary malignant tumors of bone
- 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)
- 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!)
- 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
- Mucinous, gelatinous with areas of visible hemorrhage
- 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
- + mucicarmine, PAS, and alcian blue staining because of hyaluronic acid
- + S-100 staining
- + cytokeratin
- EM: cytoplasmic vacuoles → glycogen
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- 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)
- 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)
- Isochromosome 1q as the probable locus involved in susceptibility
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