Symptoms, Signs and Physical Examination of Patients with Skull Base Tumours

Part of the Head and Neck Cancer Clinics book series (HNCC)


The clinical presentation of skull base tumours (SBTs) varies greatly and relates directly to the location and growth rate of the lesion. Signs and symptoms can often be misleading and initially interpreted as being infectious or benign diseases. A major limiting factor in the treatment of SBTs is their close proximity to sensitive organs such as the brain stem, chiasm, optic nerves and spinal cord. Complete tumour resection is considered the primary treatment modality for most lesions [1]. Therefore, it is imperative to establish a diagnosis shortly after the appearance of symptoms, as late diagnosis can lead to a worsening prognosis.


Pituitary Adenoma Cranial Nerve Paranasal Sinus Nasal Obstruction Adenoid Cystic Carcinoma 
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  1. 1.
    Combs SE, Nikoghosyan A, Jaekel O, et al. Carbon ion radiotherapy for pediatric patients and young adults treated for tumors of the skull base. Cancer 2009;115:1348–55.CrossRefPubMedGoogle Scholar
  2. 2.
    Lau KK, Joshi SM, Ellamushi H, et al. Isolated bilateral oculomotor nerve palsy in pituitary apoplexy: Case report and review. Br J Neurosurg 2007;21:399–402.CrossRefPubMedGoogle Scholar
  3. 3.
    Newell-Price J, Trainer P, Besser M, et al. The diagnosis and differential diagnosis of Cushing syndrome and pseudo-Cushing’s states. Endocr Rev 1998;19:647–72.PubMedGoogle Scholar
  4. 4.
    Magiakou MA, Mastorakos G, Oldfield EH, et al. Cushing syndrome in children and adolescents. Presentation, diagnosis, and therapy. N Engl J Med 1994;331:629–36.CrossRefPubMedGoogle Scholar
  5. 5.
    Weber A, Trainer PJ, Grossman AB, et al. Investigation, management and therapeutic outcome in 12 cases of childhood and adolescent Cushing syndrome. Clin Endocrinol (Oxf) 1995;43:19–28.CrossRefGoogle Scholar
  6. 6.
    Bickler SW, McMahon TJ, Campbell JR, et al. Preoperative diagnostic evaluation of children with Cushing syndrome. J Pediatr Surg 1994;29:671–6.CrossRefPubMedGoogle Scholar
  7. 7.
    Leinung MC, Zimmerman D. Cushing’s disease in children. Endocrinol Metab Clin North Am 1994;23:629–39.PubMedGoogle Scholar
  8. 8.
    Clayton RN. Cardiovascular function in acromegaly. Endocr Rev 2003;24:272–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Colao A, Ferone D, Marzullo P, et al. Systemic complications of acromegaly: Epidemiology, pathogenesis, and management. Endocr Rev 2004;25:102–52.CrossRefPubMedGoogle Scholar
  10. 10.
    Melmed S. Medical progress: Acromegaly. N Engl J Med 2006;355:2558–73.CrossRefPubMedGoogle Scholar
  11. 11.
    Orth DN. Cushing syndrome. N Engl J Med 1995;332:791–803.CrossRefPubMedGoogle Scholar
  12. 12.
    Ross EJ, Marshall-Jones P, Friedman M. Cushing’s syndrome: Diagnostic criteria. Q J Med 1966;35:149–92.PubMedGoogle Scholar
  13. 13.
    Ross EJ, Linch DC. Cushing’s syndrome—killing disease: Discriminatory value of signs and symptoms aiding early diagnosis. Lancet 1982;2:646–9.CrossRefPubMedGoogle Scholar
  14. 14.
    Yanovski JA, Cutler GB, Jr. Glucocorticoid action and the clinical features of Cushing’s syndrome. Endocrinol Metab Clin North Am 1994;23:487–509.PubMedGoogle Scholar
  15. 15.
    Nakamura M, Struck M, Roser F, et al. Olfactory groove meningiomas: Clinical outcome and recurrence rates after tumor removal through the frontolateral and bifrontal approach. Neurosurgery 2008;62:1224–32.CrossRefPubMedGoogle Scholar
  16. 16.
    Tsikoudas A, Martin-Hirsch DP. Olfactory groove meningiomas. Clin Otolaryngol Allied Sci 1999;24:507–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Midilli R, Karci B, Akyildiz S. Juvenile nasopharyngeal angiofibroma: Analysis of 42 cases and important aspects of endoscopic approach. Int J Pediatr Otorhinolaryngol 2009;73:401–8.CrossRefPubMedGoogle Scholar
  18. 18.
    Yiotakis I, Eleftheriadou A, Davilis D, et al. Juvenile nasopharyngeal angiofibroma stages I and II: A comparative study of surgical approaches. Int J Pediatr Otorhinolaryngol 2008;72:793–800.CrossRefPubMedGoogle Scholar
  19. 19.
    Bales C, Kotapka M, Loevner LA, et al. Craniofacial resection of advanced juvenile nasopharyngeal angiofibroma. Arch Otolaryngol Head Neck Surg 2002;128:1071–8.CrossRefPubMedGoogle Scholar
  20. 20.
    Chapurlat RD, Orcel P. Fibrous dysplasia of bone and McCune-Albright syndrome. Best Pract Res Clin Rheumatol 2008;22:55–69.CrossRefPubMedGoogle Scholar
  21. 21.
    Hehar SS, Jones NS. Fronto-ethmoid osteoma: The place of surgery. J Laryngol Otol 1997;111:372–5.PubMedGoogle Scholar
  22. 22.
    Reh DD, Lewis CM, Metson R. Frontal bullosa: Diagnosis and management of a new variant of frontal mucocele. Arch Otolaryngol Head Neck Surg 2010;136:625–8.CrossRefPubMedGoogle Scholar
  23. 23.
    Har-El G. Endoscopic management of 108 sinus mucoceles. Laryngoscope 2001;111:2131–4.CrossRefPubMedGoogle Scholar
  24. 24.
    Koike Y, Tokoro K, Chiba Y, et al. Intracranial extension of paranasal sinus mucocele: Two case reports. Surg Neurol 1996;45:44–8.CrossRefPubMedGoogle Scholar
  25. 25.
    Tan YC, Yu CC, Chang CN, et al. Optic nerve compression in craniofacial fibrous dysplasia: The role and indications for decompression. Plast Reconstr Surg 2007;120:1957–62.CrossRefPubMedGoogle Scholar
  26. 26.
    Lustig LR, Holliday MJ, McCarthy EF, et al. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001;127:1239–47.CrossRefPubMedGoogle Scholar
  27. 27.
    Zaytoun GM, Dagher WI, Rameh CE. Recurrent facial nerve paralysis: An unusual presentation of fibrous dysplasia of the temporal bone. Eur Arch Otorhinolaryngol 2008;265:255–9.CrossRefPubMedGoogle Scholar
  28. 28.
    Eleno N, Duwel A, Munoz A, et al. Endoglin as a marker in cervical paragangliomas. Head Neck 2010;32:737–43.PubMedGoogle Scholar
  29. 29.
    Boedeker CC, Ridder GJ, Schipper J. Paragangliomas of the head and neck: Diagnosis and treatment. Fam Cancer 2005;4:55–9.CrossRefPubMedGoogle Scholar
  30. 30.
    Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: Clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab 2001;86:5210–16.CrossRefPubMedGoogle Scholar
  31. 31.
    Gjuric M, Gleeson M. Consensus statement and guidelines on the management of paragangliomas of the head and neck. Skull Base 2009;19:109–16.PubMedCentralCrossRefPubMedGoogle Scholar
  32. 32.
    Lee CK, Park KH. Images in clinical medicine. Glomus tympanicum. N Engl J Med 2010;362:e66.CrossRefPubMedGoogle Scholar
  33. 33.
    McMonagle B, Al-Sanosi A, Croxson G, et al. Facial schwannoma: Results of a large case series and review. J Laryngol Otol 2008;122:1139–50.CrossRefPubMedGoogle Scholar
  34. 34.
    Lanser MJ, Sussman SA, Frazer K. Epidemiology, pathogenesis, and genetics of acoustic tumors. Otolaryngol Clin North Am 1992;25:499–520.PubMedGoogle Scholar
  35. 35.
    Evans DG, Moran A, King A, et al. Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10-year period: Higher incidence than previously thought. Otol Neurotol 2005;26:93–7.CrossRefPubMedGoogle Scholar
  36. 36.
    O’Donoghue GM, Brackmann DE, House JW, et al. Neuromas of the facial nerve. Am J Otol 1989;10:49–54.PubMedGoogle Scholar
  37. 37.
    Lawson W, Kaufman MR, Biller HF. Treatment outcomes in the management of inverted papilloma: An analysis of 160 cases. Laryngoscope 2003;113:1548–56.CrossRefPubMedGoogle Scholar
  38. 38.
    Mirza S, Bradley PJ, Acharya A, et al. Sinonasal inverted papillomas: Recurrence, and synchronous and metachronous malignancy. J Laryngol Otol 2007;121:857–64.CrossRefPubMedGoogle Scholar
  39. 39.
    Busquets JM, Hwang PH. Endoscopic resection of sinonasal inverted papilloma: A meta-analysis. Otolaryngol Head Neck Surg 2006;134:476–82.CrossRefPubMedGoogle Scholar
  40. 40.
    Lawson W, Patel ZM. The evolution of management for inverted papilloma: An analysis of 200 cases. Otolaryngol Head Neck Surg 2009;140:330–5.CrossRefPubMedGoogle Scholar
  41. 41.
    Karavitaki N, Cudlip S, Adams CB, et al. Craniopharyngiomas. Endocr Rev 2006;27:371–97.CrossRefPubMedGoogle Scholar
  42. 42.
    Garnett MR, Puget S, Grill J, et al. Craniopharyngioma. Orphanet J Rare Dis 2007;2:18.PubMedCentralCrossRefPubMedGoogle Scholar
  43. 43.
    Jagannathan J, Dumont AS, Jane JA Jr, et al. Pediatric sellar tumors: Diagnostic procedures and management. Neurosurg Focus 2005;18:E6.CrossRefPubMedGoogle Scholar
  44. 44.
    Deutsch H, Kothbauer K, Persky M, et al. Infrasellar craniopharyngiomas: Case report and review of the literature. Skull Base 2001;11:121–8.PubMedCentralCrossRefPubMedGoogle Scholar
  45. 45.
    Jane JA, Jr., Laws ER. Craniopharyngioma. Pituitary 2006;9:323–6.CrossRefPubMedGoogle Scholar
  46. 46.
    Lee TJ, Li SP, Fu CH, et al. Extensive paranasal sinus mucoceles: A 15-year review of 82 cases. Am J Otolaryngol 2009;30:234–8.CrossRefPubMedGoogle Scholar
  47. 47.
    Sautter NB, Citardi MJ, Perry J, et al. Paranasal sinus mucoceles with skull-base and/or orbital erosion: Is the endoscopic approach sufficient? Otolaryngol Head Neck Surg 2008;139:570–4.CrossRefPubMedGoogle Scholar
  48. 48.
    Wind JJ, Caputy AJ, Roberti F. Spontaneous encephaloceles of the temporal lobe. Neurosurg Focus 2008;25:E11.CrossRefPubMedGoogle Scholar
  49. 49.
    Wen S, Ethen M, Langlois PH, et al. Prevalence of encephalocele in Texas, 1999–2002. Am J Med Genet A 2007;143A:2150–5.CrossRefPubMedGoogle Scholar
  50. 50.
    Wilkins RH, Radtke RA, Burger PC. Spontaneous temporal encephalocele. Case report. J Neurosurg 1993;78:492–8.CrossRefPubMedGoogle Scholar
  51. 51.
    Landreneau FE, Mickey B, Coimbra C. Surgical treatment of cerebrospinal fluid fistulae involving lateral extension of the sphenoid sinus. Neurosurgery 1998;42:1101–4; discussion 4–5.CrossRefPubMedGoogle Scholar
  52. 52.
    Lai SY, Kennedy DW, Bolger WE. Sphenoid encephaloceles: Disease management and identification of lesions within the lateral recess of the sphenoid sinus. Laryngoscope 2002;112:1800–5.CrossRefPubMedGoogle Scholar
  53. 53.
    Wei WI, Sham JS. Nasopharyngeal carcinoma. Lancet 2005;365:2041–54.CrossRefPubMedGoogle Scholar
  54. 54.
    Sham JS, Wei WI, Zong YS, et al. Detection of subclinical nasopharyngeal carcinoma by fibreoptic endoscopy and multiple biopsy. Lancet 1990;335:371–4.CrossRefPubMedGoogle Scholar
  55. 55.
    Skinner DW, Van Hasselt CA, Tsao SY. Nasopharyngeal carcinoma: Modes of presentation. Ann Otol Rhinol Laryngol 1991;100:544–51.CrossRefPubMedGoogle Scholar
  56. 56.
    Ellul D, Cutajar J, Borg Xuereb H, et al. Nasopharyngeal carcinoma presenting as carotidynia in an 18-year-old patient. J Laryngol Otol 2008;122:207–9.CrossRefPubMedGoogle Scholar
  57. 57.
    Lee AW, Foo W, Law SC, et al. Nasopharyngeal carcinoma: Presenting symptoms and duration before diagnosis. Hong Kong Med J 1997;3:355–61.PubMedGoogle Scholar
  58. 58.
    Ozyar E, Atahan IL, Akyol FH, et al. Cranial nerve involvement in nasopharyngeal carcinoma: Its prognostic role and response to radiotherapy. Radiat Med 1994;12:65–8.PubMedGoogle Scholar
  59. 59.
    Brennan B. Nasopharyngeal carcinoma. Orphanet J Rare Dis 2006;1:23.PubMedCentralCrossRefPubMedGoogle Scholar
  60. 60.
    Wei WI, Kwong DL. Current management strategy of nasopharyngeal carcinoma. Clin Exp Otorhinolaryngol 2010;3:1–12.PubMedCentralCrossRefPubMedGoogle Scholar
  61. 61.
    Singh H, Harrop J, Schiffmacher P, et al. Ventral surgical approaches to cranio-vertebral junction chordomas. Neurosurgery 2010;66:96–103.CrossRefPubMedGoogle Scholar
  62. 62.
    Martin JJ, Niranjan A, Kondziolka D, et al. Radiosurgery for chordomas and chondrosarcomas of the skull base. J Neurosurg 2007;107:758–64.CrossRefPubMedGoogle Scholar
  63. 63.
    Heffelfinger MJ, Dahlin DC, MacCarty CS, et al. Chordomas and cartilaginous tumors at the skull base. Cancer 1973;32:410–20.CrossRefPubMedGoogle Scholar
  64. 64.
    Stippler M, Gardner PA, Snyderman CH, et al. Endoscopic endonasal approach for clival chordomas. Neurosurgery 2009;64:268–77; discussion 77–8.CrossRefPubMedGoogle Scholar
  65. 65.
    Samii A, Gerganov VM, Herold C, et al. Chordomas of the skull base: Surgical management and outcome. J Neurosurg 2007;107:319–24.CrossRefPubMedGoogle Scholar
  66. 66.
    Cantu G, Bimbi G, Miceli R, et al. Lymph node metastases in malignant tumors of the paranasal sinuses: Prognostic value and treatment. Arch Otolaryngol Head Neck Surg 2008;134:170–7.CrossRefPubMedGoogle Scholar
  67. 67.
    Dulguerov P, Jacobsen MS, Allal AS, et al. Nasal and paranasal sinus carcinoma: Are we making progress? A series of 220 patients and a systematic review. Cancer 2001;92:3012–29.CrossRefPubMedGoogle Scholar
  68. 68.
    Weymuller EA, Jr., Reardon EJ, Nash D. A comparison of treatment modalities in carcinoma of the maxillary antrum. Arch Otolaryngol 1980;106:625–9.CrossRefPubMedGoogle Scholar
  69. 69.
    da Cruz Perez DE, Pires FR, et al. Adenoid cystic carcinoma and mucoepidermoid carcinoma of the maxillary sinus: Report of a 44-year experience of 25 cases from a single institution. J Oral Maxillofac Surg 2006;64:1592–7.CrossRefPubMedGoogle Scholar
  70. 70.
    Rhee CS, Won TB, Lee CH, et al. Adenoid cystic carcinoma of the sinonasal tract: Treatment results. Laryngoscope 2006;116:982–6.CrossRefPubMedGoogle Scholar
  71. 71.
    Gil Z, Carlson DL, Gupta A, et al. Patterns and incidence of neural invasion in patients with cancers of the paranasal sinuses. Arch Otolaryngol Head Neck Surg 2009;135:173–9.CrossRefPubMedGoogle Scholar
  72. 72.
    Hanna E, Vural E, Prokopakis E, et al. The sensitivity and specificity of high-resolution imaging in evaluating perineural spread of adenoid cystic carcinoma to the skull base. Arch Otolaryngol Head Neck Surg 2007;133:541–5.CrossRefPubMedGoogle Scholar
  73. 73.
    Lupinetti AD, Roberts DB, Williams MD, et al. Sinonasal adenoid cystic carcinoma: The M.D. Anderson Cancer Center experience. Cancer 2007;110:2726–31.CrossRefPubMedGoogle Scholar
  74. 74.
    Liu TR, Yang AK, Guo X, et al. Adenoid cystic carcinoma of the nasopharynx: 27-year experience. Laryngoscope 2008;118:1981–8.CrossRefPubMedGoogle Scholar

Copyright information

© The Author(s)  2012

Authors and Affiliations

  1. 1.Department of Otolaryngology, Head and Neck and Maxillofacial SurgeryTel Aviv Sourasky Medical Center, Tel Aviv UniversityTel AvivIsrael
  2. 2.Head and Neck Surgery Unit, Department of Otolaryngology, Head and Neck and Maxillofacial SurgeryTel Aviv Sourasky Medical Center, Tel Aviv UniversityTel AvivIsrael

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