Access Keys:
Skip to content (Access Key - 0)

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

Ashwood N, Hoskin PJ, Saunders JI: Metastatic chordoma: pattern of spread and response to chemotherapy. Clin Oncol (R Coll Radiol). 6:341-2,1994.
Azzarelli A, Quagliuolo V, Cerasoli S, Zucali R, Bignanni P, Mazzaferro V, Dossena G, Gennari L: Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 37:185-91,1998.
Bas T, Bas P, Prieto M, Ramos V, Bas JL, Espinosa C: A lumbar chordoma treated with a wide resection. Eur Spine J 3:115-7,1994.
Bethke KP, Neifeld JP, Lawrence W Jr: Diagnosis and management of sacrococcygeal chordoma. J Surg Oncol 48:232-8,1991.
Bhadra AK, Casey ATH: Familial chordoma. A report of two cases. JBJS 88B:634-6,2006.
Bianchi PM, Marsella P, Masi R, Andriani G, Tucci FM, Partipilo P, Pierro V: Cervical chordoma in childhood: clinical statistical contribution. Int J Pediatr Otorhinolaryngol 18:39-45,1989.
Bjornsson J, World LE, Ebersold MJ, Laws ER: Chordoma of the mobile spine. A clinicopathological analysis of 40 patients. Cancer 71:735-40,1993.
Boriani S, Chevalley F, Weinstin JN, Biagini R, Campanacci L, Delure F, Piccill P: Chordoma of the spine above the sacrum: treatment and outcome in 21 cases. Spine 21:1569-77,1996.
Cheng EY, Özerdemoglu RA, Transfeldt EE, Thompson RC, Jr: Lumbosacral chordoma: prognostic factors and treatment. Spine 24:1639-45,1999.
Catton C, O'Sullivan B, Bell R, Laperriere N, Cummings B, Fornasier V, Wunder J: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 41:67-72,1996.
Chandawarkar RY: Sacrococcygeal chordoma: review of 50 consecutive patients. World J Surg 20:717-9,1996.
Cheng EY, Ozerdemoglu RA, Transfeldt EE, Thompson RC Jr: Lumbosacral chordoma. Prognostic factors and treatment. Spine 24:1639-45,1999.
Currier BL, Todd LT, Maus TP, Fischer DR, Yaszemski MJ: Anatomic relationship of the internal carotid artery to the C1 vertebra: a case report of cervical reconstruction for chordoma and pilot study to assess the risk of screw fixation of the atlas. Spine 28:E461-7,2003.
Dabska M: Parachordoma: a new clinicopathologic entity. Cancer 40:1586-92,1977.
Dalpra I, Malgara R, Miozzo M, : First cytogenetic study of recurrent familial chordoma of the clivus. Int J Cancer 81:24-30,1999.
D'Haen BD, De Jaegere T, Goffin J, Dom R, Demaerel P, Plets C: Chordoma of the lower cervical spine: case report. Clin Neurol Neurosurg 97:245-8,1995.
Doita M, Harada T, Iguchi T, Sumi M, Sha H, Yoshiya S, Kurosaka M: Total sacrectomy and reconstruction for sacral tumors. Spine 28:E296-301,2003.
Enin IP: Chordoma of the nasopharynx in two members of a family. Vestn Otoinolaringol 26:88-90,1964.
Firooznia H, Golimbu C, Rafii M, Reede DL, Kricheff II, Bjorkengren A: Computed tomography of spinal chordomas. J Comput Assist Tomogr 10:45-50,1986.
Firooznia H, Pinto RS, Lin JP, Baruch HH, Zausner J: Chordoma: radiologic evaluation of 20 cases. AJR 127:797-805,1976.
Fisher C, Miettinen M: Parachordoma: a clinicopathologic and immunohistochemical study of four cases of an unusual soft tissue neoplasm. Ann Diagn Pathol1:3-10,1997.
Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin RS, Samuels BL: Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 72:714-8,1993.
Foote RF, Ablin G, Hall WW: Chordoma in siblings. Calif Med 88:383-6,1958.
Fujita T, Kawahara N, Matsumoto T, Tomita K: Chordoma in the cervical spine managed with en bloc excision. Spine 24:1848-51,1999.
Gray SW, Singhbandhu B, Smith RA, Skandalakis JE: Sacrococcygeal chordomas: report of a case and review of the literature. Surg 78:573-82,1975.
Gunterberg B, Norlen L, Stener B, Sundin T: Neurologic evaluation after resection of the sacrum. Invest Urol (Berl): 13:183-8,1975.
Healy JH, Lane JM: Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am 20:417-26,1989.
Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW: Chordomas and cartilaginous tumours at the skull base. Cancer 32:410-20,1973.
Huang SM, Chen CC, Chiu PC, Lai PH, Ho JT, Tseng HH: Unusual presentation of posterior mediastinal chordoma in a 2-year-old boy. J Pediatr Hematol Oncol 25:743-6,2003.

Hulen CA, Temple HT, Fox WP, Sama AA, Green BA, Eismont FJ: Oncologic and functional outcome following sacrectomy for sacral chordoma. J Bone Joint Surg 88:1532-1539, 2006.
Jena R, Lubana F, Brooke SL, Geader AR, Jefferies SJ, Burton KE, Laing RJ, Burnet NG: Conformal roation therapy with central axis beam block is a feasible alternative to intensity-modulated radiotherapy for chordomas of the cervical spine. Clin Oncol (R Coll Radiol) 16:449-56,2004.
Kamrin RP, Potanos JN, Pool JL: An evaluation of the diagnosis and treatment of chordoma. J Neurol Neurosurg Psychiatry 27:157-65,1964.
Kelley MJ, Korczak JF, Sheridan E, : Familial chordoma, a tumour of notochordal remnants, is linked to chromosome 7q33. Am J Hum Genet 69:454-60,2001.
Kerr WA, Allen KL, Haynes DR, Sellars SL: Familial nasopharyngeal chordoma. S Afr Med J 49:1584,1975.
Koh PK, Tan BK, Hong SW, Tan MH, Tay AG, Song C, Tan KC: The gluteus maximus muscle flap for reconstruction of sacral chordoma defects. Ann Plast Surg 53:44-9,2004.
Krol G, Sze G, Arbit E, Marcove R, Sundaresan N: Intradural metastases of chordoma. AJNR 10:193-5,1989.
Kuzniacka A, Mertens F, Strombeck B, Wiegant J, Mandahl N: Combined binary ratio labeling fluorescence in situ hybridization analysis of chordoma. Cancer Genet Cytogent 151:178-81,2004.
Manzone P, Fiore N, Forlino D, Alcala M, Cabrera CF: Chordoma of the lumbar L2 vertebra: case report and review of the literature. Eur Spine J 7:252-6,1998.
McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM: Chordoma: incidence and survival pattern in the United States, 1973-1995. Cancer Causes Control 12:1-11,2001.
Meyer JE, Lepke RA, Lindfors KK, Pagani JJ, Hirschy JC, Hayman LA, Momose KJ, McGinnis B: Chordomas: Their CT appearance in the cervical, thoracic and lumbar spine. Radiology 153:693-6,1984.
Mindell E: Chordoma. J Bone Joint Surg 63A:501-5,1981.
Miozzo M, Dalpra L, Riva P, : A tumour suppressor locus in familial and sporadic chrodoma maps to 1p36. Int J Cancer 87:68-72,2000.
Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ: Chordoma and chondroid neoplasms of the speno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 72:2943-9,1993.
Murali R, Rovit RL, Benjamin MV: Chordoma of the cervical spine. Neurosurgery 9:253-6,1981.
Noel G, Feuvret L, Ferrand R, Boisserie G, Mazeron JJ, Habrand JL: Radiotherapeutic factors in the management of cervical-basal chordomas and chondrosarcomas. Neurosurgery 55:1252-62,2004.
Ozaki T, Hillmann A, Winkelmann W: Surgical treatment of sacrococcygeal chordoma. J Surg Oncol 64:274-9,1997.
Papagelopoulos PJ, Mavrogenis AF, Galanis EC, Savvidou OD, Boscainos PJ, Katonis PG, Sim FH: Chordoma of the spine: clincicopathological features, diagnosis, and treatment. Orthopedics 27:1256-63,2004.
Rich TA, Schiller A, Suit HD, Mankin HJ: Clinical and pathological review of 48 cases of chordoma. Cancer 56:182-7,1985.
Samson IR, Springfield DS, Suit HD, Mankin HJ: Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Joint Surg 75A:1476-84,1993.
Sawyer JR, Husain M, Al-Mefty O: Identification of isochromosome 1q as a recurring chromosome aberration in skull base chordomas: a new marker for aggressive tumours. Neurosurg Focus 10:1-6,2001.
Shimmura Y, Miura K, Yajima S, Tsutsui Y: Sacrococcygeal chordoma in infancy showing an aggressive clinical course: an autopsy case report. Pathol Int 53:473-7,2003.
Soo MY: Chordoma: review of clinicoradiological features and factors affecting survival. Australas Radiol 45:427-34,2001.
Stener, Gunterberg B: High amputation of the sacrum for extirpation of tumors: principles and technique. Spine 3:351-66,1978.
Stepanek J, Cataldo SA, Ebersold MJ, : Familial chordoma with probable autosomal dominant inheritance. Am J Med Genet 75:335-6,1998.
Sundaresan N: Chordomas. Clin Orthop 204:135-42,1986.
Sundaresan N, Boriani S, Rothman A, Holtzman R: Tumors of the osseous spine. J Neurooncol 69:273-90,2004.
Sundaresan N, Galicich JH, Chu FC, Huvos AG: Spinal chordomas. J Neurosurg 50:312-9,1979.
Sundaresan N, Huvos AG, Krol G, Lane JM, Brennan M: Surgical treatment of spinal chordomas. Arch Surg 122:1479-82,1987.
Sung HW, Shu WP, Wang HM, Yuai SY, Tsai YB: Surgical treatment of primary tumors of the sacrum. Clin Orthop 215:91-8,1987.
Todd LT Jr, Yaszemski MJ, Currier BL, Fuchs B, Kim CW, Sim FH: Bowel and bladder function after major sacral resection. Clin Orthop 397:36-9,2002.
Walaas L, Kindblom LG: Fine needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical and immunocytochemical examination. Hum Pathol 22:22-8,1991.
Yamaguchi T, Suzuki S, Ishiwa H, Shimizu K, Ueda Y: Benign norotchordal cell tumors: a comparative histological study of benign notochrodal cell tumors, classic chordomas, and notochrodal vestiges of fetal intervertebral discs. Am J Surg Pathol 28:756-61,2004.
Yonemoto T, Tatezaki S, Takenouchi T, Ishii T, Satoh T, Moriya H: The surgical management of sacrococcygeal chordoma. Cancer 85:878-83,1999.
Yuh WT, Lozano RL, Flinckinger FW, Sato Y, Kao SS, Menezes AH: Lumbar epidural chordoma: MR findings. J Comput Assist Tomogr 13:508-10,1989.

Content

Download: PDF | EPUB

Famous Quote
"Character is simply long habit continued." Plutarch
Figures
Related Content

Resources for Chordoma and related topics on OrthopaedicsOne.