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
QUESTIONS 1 of 6 1 2 3 4 5 6 Previous Next (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 Type & Select Correct Answer 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 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic (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: A B Type & Select Correct Answer 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 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic 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? QID: 442 FIGURES: A B Type & Select Correct Answer 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 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic
All Videos (3) Podcasts (1) Login to View Community Videos Login to View Community Videos oncology Radical Sacrectomy and Reconstruction for a High-Grade Primary Sarcoma of the Sacrum Mohammed Al Sobeai General - Chordoma E 1/30/2017 121 views 0.0 (0) Login to View Community Videos Login to View Community Videos Chordoma - Histology Rounds General - Chordoma E 10/30/2012 248 views 1.7 (3) Login to View Community Videos Login to View Community Videos Sacral Chordoma Resection and Cryosurgery - Dr. James C. Wittig Pathology - Chordoma D 10/30/2012 915 views 4.4 (7) Pathology⎪Chordoma Team Orthobullets 4 Pathology - Chordoma Listen Now 8:22 min 10/15/2019 125 plays 5.0 (1)