Introduction A malignant tumor of primitive notochordal origin slow growing and frequently misdiagnosed as low back pain Epidemiology incidence most common primary malignant spinal tumor in adults demographics 3:1 male to female ratio usually in patients > 50 years location 50% occur in the sacrum and coccyx 35% in spheno-occiptal region 15% in mobile spine Pathoanatomy forms from malignant transformation in residual notochordal cells resulting in midline location 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 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 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 Differentials & Groups Sacral lesions in older patients (1) Keratin stain positive Similar Appearance on Xray Treated with wide-resection alone (2) Chordoma • • • • Chondrosarcoma • • • Metastatic disease • • • Lymphoma • • Multiple Myeloma • MFH • Secondary sarcoma Enchondroma of hand Olliers Maffucci's Periosteal chondroma Osteochondroma (MHE) Parosteal osteosarcoma • Adamantinoma • • Synovial sarcoma • Epitheloid sarcoma • Squamous cell (3) • ASSUMPTIONS: (1) Older patient is > 40 yrs; (2) assuming no impending fracture (3) assuming no metastatic disease Location Xray Xray CT B. Scan MRI MRI Histo(1) Case A sacrum Case B sacrum Case C sacrum Case D cervical spine (1) - histology does not always correspond to clinical case
QUESTIONS 1 of 6 1 2 3 4 5 6 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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? Tested Concept QID: 442 FIGURES: A B Type & Select Correct Answer 1 Regional lymph node metastasis 8% (255/3115) 2 Liver metastasis 2% (63/3115) 3 Bone metastasis 14% (427/3115) 4 Local recurrence 70% (2169/3115) 5 Malignant transformation 6% (189/3115) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review tested concept
Evidence Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group Pathology - Chordoma 0 views Evidence Building a global consensus approach to chordoma: a position paper from the medical and patient community Pathology - Chordoma 0 views Evidence En bloc resection of primary sacral tumors: classification of surgical approaches and outcome Pathology - Chordoma 0 views
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