Search tips
Search criteria 


Logo of skullbasesurgInstructions for AuthorsSubscribe to Skull BaseAbout Skull BaseEditorial BoardThieme Medical PublishingSkull Base An Interdisciplinary Approach ...
Skull base surgery. 1991 October; 1(4): 207–216.
PMCID: PMC1656334

Pathobiology of Selected Tumors of the Base of the Skull


Skull base surgery is a new subspeciality, and, up to this point, most articles on this subject have focused on innovative operative-reconstructive approaches to tumors in this region. It is now important that we embark on a new era, the era of tumor biology, and concentrate on new ways of evaluating these neoplasms from a pathologic viewpoint. The hematoxylin-cosin section is no longer an end point, but just a beginning. This is the age of molecular biology. It is important that these tumors be evaluated, either prospectively or retrospectively, employing immunohistochemical staining, flow cytometry, oncogene expression, cytogenetics, or other techniques in order to identify important prognostic features. Data from these additional studies may then be used to develop new treatment strategies. Skull base societies should develop protocols for one or more of these tumors to ensure that they are indeed evaluated uniformly. In this article I emphasize the importance of accurate histologic classification or subclassification of these neoplasms and focus on contemporary parameters that may or may not impact on prognosis.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (3.1M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Buell P. The effect of migration on the risk of nasopharyngeal cancer among Chinese. Cancer Res. 1974 May;34(5):1189–1191. [PubMed]
  • Lanier A, Bender T, Talbot M, Wilmeth S, Tschopp C, Henle W, Henle G, Ritter D, Terasaki P. Nasopharyngeal carcinoma in Alaskan Eskimos Indians, and Aleuts: a review of cases and study of Epstein-Barr virus, HLA, and environmental risk factors. Cancer. 1980 Nov 1;46(9):2100–2106. [PubMed]
  • Miller D. The etiology of nasopharyngeal cancer and its management. Otolaryngol Clin North Am. 1980;13(3):467–475. [PubMed]
  • Dickson RI. Nasopharyngeal carcinoma: an evaluation of 209 patients. Laryngoscope. 1981 Mar;91(3):333–354. [PubMed]
  • Zhu XN, Chen R, Kong FH, Liu W. Human leukocyte antigens -A, -B, -C, and -DR and nasopharyngeal carcinoma in northern China. Ann Otol Rhinol Laryngol. 1990 Apr;99(4 Pt 1):286–287. [PubMed]
  • Betuel H, Camoun M, Colombani J, Day NE, Ellouz R, de-Thé G. The relationship between nasopharyngeal carcinoma and the HL-A system among Tunisians. Int J Cancer. 1975 Aug 15;16(2):249–254. [PubMed]
  • Moore SB, Pearson GR, Neel HB, 3rd, Weiland LH. HLA and nasopharyngeal carcinoma in North American Caucasoids. Tissue Antigens. 1983 Jul;22(1):72–75. [PubMed]
  • Armstrong RW, Armstrong MJ, Yu MC, Henderson BE. Salted fish and inhalants as risk factors for nasopharyngeal carcinoma in Malaysian Chinese. Cancer Res. 1983 Jun;43(6):2967–2970. [PubMed]
  • Lin TM, Yang CS, Tu SM, Chen CJ, Kuo KC, Hirayama T. Interaction of factors associated with cancer of the nasopharynx. Cancer. 1979 Oct;44(4):1419–1423. [PubMed]
  • zur Hausen H, Schulte-Holthausen H, Klein G, Henle W, Henle G, Clifford P, Santesson L. EBV DNA in biopsies of Burkitt tumours and anaplastic carcinomas of the nasopharynx. Nature. 1970 Dec 12;228(5276):1056–1058. [PubMed]
  • Huang DP, Ho HC, Henle W, Henle G, Saw D, Lui M. Presence of EBNA in nasopharyngeal carcinoma and control patient tissues related to EBV serology. Int J Cancer. 1978 Sep 15;22(3):266–274. [PubMed]
  • Neel HB, 3rd, Pearson GR, Weiland LH, Taylor WF, Goepfert HH, Pilch BZ, Goodman M, Lanier AP, Huang AT, Hyams VJ, et al. Application of Epstein-Barr virus serology to the diagnosis and staging of North American patients with nasopharyngeal carcinoma. Otolaryngol Head Neck Surg. 1983 Jun;91(3):255–262. [PubMed]
  • Neel HB, 3rd, Taylor WF. Epstein-Barr virus-related antibody. Changes in titers after therapy for nasopharyngeal carcinoma. Arch Otolaryngol Head Neck Surg. 1990 Nov;116(11):1287–1290. [PubMed]
  • Hsu MM, Tu SM. Nasopharyngeal carcinoma in Taiwan. Clinical manifestations and results of therapy. Cancer. 1983 Jul 15;52(2):362–368. [PubMed]
  • Counter RT, Linares L, Shaw HJ, Dalley VM. Cancer of the nasopharynx in under 21-year-olds: a review. Clin Oncol. 1980 Sep;6(3):213–220. [PubMed]
  • Easton JM, Levine PH, Hyams VJ. Nasopharyngeal carcinoma in the United States. A pathologic study of 177 US and 30 foreign cases. Arch Otolaryngol. 1980 Feb;106(2):88–91. [PubMed]
  • Jenkin RD, Anderson JR, Jereb B, Thompson JC, Pyesmany A, Wara WM, Hammond D. Nasopharyngeal carcinoma--a retrospective review of patients less than thirty years of age: a report of Children's Cancer Study Group. Cancer. 1981 Jan 15;47(2):360–366. [PubMed]
  • Sham JS, Choy D. Prognostic factors of nasopharyngeal carcinoma: a review of 759 patients. Br J Radiol. 1990 Jan;63(745):51–58. [PubMed]
  • Huang TB. Cancer of the nasopharynx in childhood. Cancer. 1990 Sep 1;66(5):968–971. [PubMed]
  • Hawkins EP, Krischer JP, Smith BE, Hawkins HK, Finegold MJ. Nasopharyngeal carcinoma in children--a retrospective review and demonstration of Epstein-Barr viral genomes in tumor cell cytoplasm: a report of the Pediatric Oncology Group. Hum Pathol. 1990 Aug;21(8):805–810. [PubMed]
  • Dickson RI, Flores AD. Nasopharyngeal carcinoma: an evaluation of 134 patients treated between 1971-1980. Laryngoscope. 1985 Mar;95(3):276–283. [PubMed]
  • Qin DX, Hu YH, Yan JH, Xu GZ, Cai WM, Wu XL, Cao DX, Gu XZ. Analysis of 1379 patients with nasopharyngeal carcinoma treated by radiation. Cancer. 1988 Mar 15;61(6):1117–1124. [PubMed]
  • Baker SR. Nasopharyngeal carcinoma: clinical course and results of therapy. Head Neck Surg. 1980 Sep-Oct;3(1):8–14. [PubMed]
  • Ahmad A, Stefani S. Distant metastases of nasopharyngeal carcinoma: a study of 256 male patients. J Surg Oncol. 1986 Nov;33(3):194–197. [PubMed]
  • Neel HB, 3rd, Taylor WF, Pearson GR. Prognostic determinants and a new view of staging for patients with nasopharyngeal carcinoma. Ann Otol Rhinol Laryngol. 1985 Nov-Dec;94(6 Pt 1):529–537. [PubMed]
  • Shanmugaratnam K, Chan SH, de-Thé G, Goh JE, Khor TH, Simons MJ, Tye CY. Histopathology of nasopharyngeal carcinoma: correlations with epidemiology, survival rates and other biological characteristics. Cancer. 1979 Sep;44(3):1029–1044. [PubMed]
  • Hsu MM, Hsu HC, Lui LT. Local immune reaction in nasopharyngeal carcinoma, with special reference to its prognostic evaluation. Head Neck. 1989 Nov-Dec;11(6):505–510. [PubMed]
  • Looi LM. Tumor-associated tissue eosinophilia in nasopharyngeal carcinoma. A pathologic study of 422 primary and 138 metastatic tumors. Cancer. 1987 Feb 1;59(3):466–470. [PubMed]
  • Prathap K, Looi LM, Prasad U. Localized amyloidosis in nasopharyngeal carcinoma. Histopathology. 1984 Jan;8(1):27–34. [PubMed]
  • Nomori H, Watanabe S, Nakajima T, Shimosato Y, Kameya T. Histiocytes in nasopharyngeal carcinoma in relation to prognosis. Cancer. 1986 Jan 1;57(1):100–105. [PubMed]
  • Nadol JB., Jr Viral particles in nasopharyngeal carcinoma. Laryngoscope. 1977 Nov;87(11):1932–1937. [PubMed]
  • Prasad U, Gogusev J. Intracisternal tubular inclusions in nasopharyngeal carcinoma. J Laryngol Otol. 1978 Nov;92(11):979–989. [PubMed]
  • Weiss LM, Movahed LA, Butler AE, Swanson SA, Frierson HF, Jr, Cooper PH, Colby TV, Mills SE. Analysis of lymphoepithelioma and lymphoepithelioma-like carcinomas for Epstein-Barr viral genomes by in situ hybridization. Am J Surg Pathol. 1989 Aug;13(8):625–631. [PubMed]
  • Akao I, Sato Y, Mukai K, Uhara H, Furuya S, Hoshikawa T, Shimosato Y, Takeyama I. Detection of Epstein-Barr virus DNA in formalin-fixed paraffin-embedded tissue of nasopharyngeal carcinoma using polymerase chain reaction and in situ hybridization. Laryngoscope. 1991 Mar;101(3):279–283. [PubMed]
  • Costello F, Mason BR, Collins RJ, Kearsley JH. A clinical and flow cytometric analysis of patients with nasopharyngeal cancer. Cancer. 1990 Oct 15;66(8):1789–1795. [PubMed]
  • Cheng DS, Campbell BH, Clowry LJ, Hopwood LE, Murray KJ, Toohill RJ, Hoffmann RG. DNA content in nasopharyngeal carcinoma. Am J Otolaryngol. 1990 Nov-Dec;11(6):393–397. [PubMed]
  • Fu YS, Perzin KH. Non-epithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: a clinicopathologic study. 3. Cartilaginous tumors (chondroma, chondrosarcoma). Cancer. 1974 Aug;34(2):453–463. [PubMed]
  • Burkey BB, Hoffman HT, Baker SR, Thornton AF, McClatchey KD. Chondrosarcoma of the head and neck. Laryngoscope. 1990 Dec;100(12):1301–1305. [PubMed]
  • Bloch DM, Bragoli AJ, Collins DN, Batsakis JG. Mesenchymal chondrosarcomas of the head and neck. J Laryngol Otol. 1979 Apr;93(4):405–412. [PubMed]
  • Huvos AG, Rosen G, Dabska M, Marcove RC. Mesenchymal chondrosarcoma. A clinicopathologic analysis of 35 patients with emphasis on treatment. Cancer. 1983 Apr 1;51(7):1230–1237. [PubMed]
  • Liu-Shindo M, Rice DH, Sherrod AE. Extraskeletal myxoid chondrosarcoma of the head and neck: a case report. Otolaryngol Head Neck Surg. 1989 Oct;101(4):485–488. [PubMed]
  • Slootweg PJ. Clear-cell chondrosarcoma of the maxilla: report of a case. Oral Surg Oral Med Oral Pathol. 1980 Sep;50(3):233–237. [PubMed]
  • Bjornsson J, Unni KK, Dahlin DC, Beabout JW, Sim FH. Clear cell chondrosarcoma of bone. Observations in 47 cases. Am J Surg Pathol. 1984 Mar;8(3):223–230. [PubMed]
  • Frassica FJ, Unni KK, Beabout JW, Sim FH. Dedifferentiated chondrosarcoma. A report of the clinicopathological features and treatment of seventy-eight cases. J Bone Joint Surg Am. 1986 Oct;68(8):1197–1205. [PubMed]
  • Cianfriglia F, Pompili A, Occhipinti E. Intracranial malignant cartilaginous tumours. Report of two cases and review of literature. Acta Neurochir (Wien) 1978;45(1-2):163–175. [PubMed]
  • Bahr AL, Gayler BW. Cranial chondrosarcomas. Report of four cases and review of the literature. Radiology. 1977 Jul;124(1):151–156. [PubMed]
  • Hassounah M, Al-Mefty O, Akhtar M, Jinkins JR, Fox JL. Primary cranial and intracranial chondrosarcoma. A survey. Acta Neurochir (Wien) 1985;78(3-4):123–132. [PubMed]
  • Kveton JF, Brackmann DE, Glasscock ME, 3rd, House WF, Hitselberger WE. Chondrosarcoma of the skull base. Otolaryngol Head Neck Surg. 1986 Jan;94(1):23–32. [PubMed]
  • Austin-Seymour M, Munzenrider J, Goitein M, Verhey L, Urie M, Gentry R, Birnbaum S, Ruotolo D, McManus P, Skates S, et al. Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg. 1989 Jan;70(1):13–17. [PubMed]
  • Grossman RI, Davis KR. Cranial computed tomographic appearance of chondrosarcoma of the base of the skull. Radiology. 1981 Nov;141(2):403–408. [PubMed]
  • Adegbite AB, McQueen JD, Paine KW, Rozdilsky B. Primary intracranial chondrosarcoma: a report of two cases. Neurosurgery. 1985 Sep;17(3):490–494. [PubMed]
  • Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of bone: a clinicopathologic analysis with emphasis on histologic grading. Cancer. 1977 Aug;40(2):818–831. [PubMed]
  • Coltrera MD, Googe PB, Harrist TJ, Hyams VJ, Schiller AL, Goodman ML. Chondrosarcoma of the temporal bone. Diagnosis and treatment of 13 cases and review of the literature. Cancer. 1986 Dec 15;58(12):2689–2696. [PubMed]
  • Sen CN, Sekhar LN, Schramm VL, Janecka IP. Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery. 1989 Dec;25(6):931–941. [PubMed]
  • Mandahl N, Heim S, Arheden K, Rydholm A, Willén H, Mitelman F. Chromosomal rearrangements in chondromatous tumors. Cancer. 1990 Jan 15;65(2):242–248. [PubMed]
  • Kreicbergs A, Boquist L, Borssén B, Larsson SE. Prognostic factors in chondrosarcoma: a comparative study of cellular DNA content and clinicopathologic features. Cancer. 1982 Aug 1;50(3):577–583. [PubMed]
  • LIGHTENSTEIN L, BERNSTEIN D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer. 1959 Nov-Dec;12:1142–1157. [PubMed]
  • Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer. 1986 Jun 15;57(12):2444–2453. [PubMed]
  • Scheithauer BW, Rubinstein LJ. Meningeal mesenchymal chondrosarcoma: report of 8 cases with review of the literature. Cancer. 1978 Dec;42(6):2744–2752. [PubMed]
  • Heros RC, Martinez AJ, Ahn HS. Intracranial mesenchymal chondrosarcoma. Surg Neurol. 1980 Oct;14(4):311–317. [PubMed]
  • McCarthy EF, Dorfman HD. Chondrosarcoma of bone with dedifferentiation: a study of eighteen cases. Hum Pathol. 1982 Jan;13(1):36–40. [PubMed]
  • Johnson S, Têtu B, Ayala AG, Chawla SP. Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathologic study of 26 cases. Cancer. 1986 Jul 15;58(2):278–286. [PubMed]
  • Têtu B, Ordóez NG, Ayala AG, Mackay B. Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). II. An immunohistochemical and electron microscopic study. Cancer. 1986 Jul 15;58(2):287–298. [PubMed]
  • Zalupski MM, Ensley JF, Ryan J, Selvaggi S, Baker LH, Wolman SR. A common cytogenetic abnormality and DNA content alterations in dedifferentiated chondrosarcoma. Cancer. 1990 Sep 15;66(6):1176–1182. [PubMed]
  • Jaworski RC. Dedifferentiated chondrosarcoma. An ultrastructural study. Cancer. 1984 Jun 15;53(12):2674–2678. [PubMed]
  • Wright D. Nasopharyngeal and cervical chordoma--some aspects of their development and treatment. J Laryngol Otol. 1967 Dec;81(12):1337–1355. [PubMed]
  • O'Neill P, Bell BA, Miller JD, Jacobson I, Guthrie W. Fifty years of experience with chordomas in southeast Scotland. Neurosurgery. 1985 Feb;16(2):166–170. [PubMed]
  • Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors at the skull base. Cancer. 1973 Aug;32(2):410–420. [PubMed]
  • Higinbotham NL, Phillips RF, Farr HW, Hustu HO. Chordoma. Thirty-five-year study at Memorial Hospital. Cancer. 1967 Nov;20(11):1841–1850. [PubMed]
  • Wold LE, Laws ER., Jr Cranial chordomas in children and young adults. J Neurosurg. 1983 Dec;59(6):1043–1047. [PubMed]
  • Rich TA, Schiller A, Suit HD, Mankin HJ. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985 Jul 1;56(1):182–187. [PubMed]
  • Matsumoto J, Towbin RB, Ball WS., Jr Cranial chordomas in infancy and childhood. A report of two cases and review of the literature. Pediatr Radiol. 1989;20(1-2):28–32. [PubMed]
  • UTNE JR, PUGH DG. The roentgenologic aspects of chordoma. Am J Roentgenol Radium Ther Nucl Med. 1955 Oct;74(4):593–608. [PubMed]
  • Raffel C, Wright DC, Gutin PH, Wilson CB. Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients. Neurosurgery. 1985 Nov;17(5):703–710. [PubMed]
  • Meyer JE, Oot RF, Lindfors KK. CT appearance of clival chordomas. J Comput Assist Tomogr. 1986 Jan-Feb;10(1):34–38. [PubMed]
  • Oot RF, Melville GE, New PF, Austin-Seymour M, Munzenrider J, Pile-Spellman J, Spagnoli M, Shoukimas GM, Momose KJ, Carroll R, et al. The role of MR and CT in evaluating clival chordomas and chondrosarcomas. AJR Am J Roentgenol. 1988 Sep;151(3):567–575. [PubMed]
  • Abenoza P, Sibley RK. Chordoma: an immunohistologic study. Hum Pathol. 1986 Jul;17(7):744–747. [PubMed]
  • Meis JM, Giraldo AA. Chordoma. An immunohistochemical study of 20 cases. Arch Pathol Lab Med. 1988 May;112(5):553–556. [PubMed]
  • Pardo-Mindan FJ, Guillen FJ, Villas C, Vazquez JJ. A comparative ultrastructural study of chondrosarcoma, chordoid sarcoma, and chordoma. Cancer. 1981 Jun 1;47(11):2611–2619. [PubMed]
  • Crumley RL, Gutin PH. Surgical access for clivus chordoma. The University of California, San Francisco, experience. Arch Otolaryngol Head Neck Surg. 1989 Mar;115(3):295–300. [PubMed]
  • Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol. 1979 Nov;72(5):765–776. [PubMed]
  • Hruban RH, Traganos F, Reuter VE, Huvos AG. Chordomas with malignant spindle cell components. A DNA flow cytometric and immunohistochemical study with histogenetic implications. Am J Pathol. 1990 Aug;137(2):435–447. [PubMed]
  • Volpe R, Mazabraud A. A clinicopathologic review of 25 cases of chordoma (a pleomorphic and metastasizing neoplasm). Am J Surg Pathol. 1983 Mar;7(2):161–170. [PubMed]
  • Perzin KH, Pushparaj N. Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. XIV: Chordomas. Cancer. 1986 Feb 15;57(4):784–796. [PubMed]
  • Chu TA. Chondroid chordoma of the sacrococcygeal region. Arch Pathol Lab Med. 1987 Sep;111(9):861–864. [PubMed]
  • Brooks JJ, LiVolsi VA, Trojanowski JQ. Does chondroid chordoma exist? Acta Neuropathol. 1987;72(3):229–235. [PubMed]
  • Valderrama E, Kahn LB, Lipper S, Marc J. Chondroid chordoma. Electron-microscopic study of two cases. Am J Surg Pathol. 1983 Oct;7(7):625–632. [PubMed]
  • Hruban RH, May M, Marcove RC, Huvos AG. Lumbo-sacral chordoma with high-grade malignant cartilaginous and spindle cell components. Am J Surg Pathol. 1990 Apr;14(4):384–389. [PubMed]
  • Belza MG, Urich H. Chordoma and malignant fibrous histiocytoma. Evidence for transformation. Cancer. 1986 Sep 1;58(5):1082–1087. [PubMed]
  • Meis JM, Raymond AK, Evans HL, Charles RE, Giraldo AA. "Dedifferentiated" chordoma. A clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol. 1987 Jul;11(7):516–525. [PubMed]
  • Miettinen M, Karaharju E, Järvinen H. Chordoma with a massive spindle-cell sarcomatous transformation. A light- and electron-microscopic and immunohistological study. Am J Surg Pathol. 1987 Jul;11(7):563–570. [PubMed]

Articles from Skull Base Surgery are provided here courtesy of Thieme Medical Publishers