Primary Malignant Skull Tumors Clinical Presentation
- Author: Draga Jichici, MD, FRCP, FAHA; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS more...
See the list below:
- Presentation may include any of the following:
- Rapidly growing mass with pain and swelling
- Mass without pain as in multiple myeloma and osteosarcoma
- Nonspecific headache
- Cranial nerve deficits: These are seen in giant cell tumors, angiosarcomas, and chordomas, as well as in tumors of the head and neck with propensity for perineural spread, such as tumors of salivary origin (eg, adenoid cystic carcinomas, adenocarcinomas, mucoepidermoid carcinomas).
- Fever and malaise
- Location of the tumor
- Although the location of the lesion is of little value in making the diagnosis, certain tumors prefer the convexity more than the skull base and vice versa; lesions of developmental origin have a propensity for the midline.
- Chondrosarcomas, giant cell tumors, angiosarcomas, and chordomas usually involve the skull base.
- Osteosarcomas and fibrosarcomas commonly are found in the mandible and maxilla.[4, 8]
- The remainder usually involve the calvaria.
- Patients may have a history of previous malignancy, fibrous dysplasia, or Paget disease.
- Multiple, small, nonmarginated lesions usually indicate metastatic disease.
- The absence of peripheral sclerosis strongly favors a malignant tumor.
- The differential diagnosis includes the following:
- Benign skull tumors
- Encephalocele, meningoencephalocele, venous lakes of the skull, pacchionian depression
- Fractures, surgical defects
- Osteomyelitis, tuberculosis, sarcoidosis, syphilis
- Hyperparathyroidism, osteoporosis, congenital hemolytic anemia
See the list below:
- Signs include the following:
- Soft or hard lesion
- Cranial nerve deficits: These may include diplopia from involvement of cranial nerves III, IV, or VI; facial paralysis; hearing loss; vertigo; and sensation loss along the distribution of the trigeminal nerve. Voice changes and swallowing disorders, with or without tongue fasciculations/paralysis, signify involvement of the cranial base at the jugular foramen with medial extension.
- Multiple findings related to the primary tumor
- Tender or nontender lesion
See the list below:
- Little information is available concerning the etiology of the malignant skull tumors (except in the case of metastatic disease).
- Chondrosarcomas often are associated with abnormalities of chromosomes 10 and 22.
Li Y, Li LJ, Huang J, Han B, Pan J. Central malignant salivary gland tumors of the jaw: retrospective clinical analysis of 22 cases. J Oral Maxillofac Surg. 2008 Nov. 66(11):2247-53. [Medline].
Lane KA, Katowitz JA. Ewing sarcoma presenting as a subconjunctival mass. Ophthal Plast Reconstr Surg. 2009 Jan-Feb. 25(1):61-3. [Medline].
Vikatmaa P, Mäkitie AA, Railo M, Törnwall J, Albäck A, Lepäntalo M. Midline mandibulotomy and interposition grafting for lesions involving the internal carotid artery below the skull base. J Vasc Surg. 2009 Jan. 49(1):86-92. [Medline].
Moschovi M, Alexiou GA, Tourkantoni N, Balafouta ME, Antypas C, Tsiotra M, et al. Cranial Ewing's sarcoma in children. Neurol Sci. 2011 Aug. 32(4):691-4. [Medline].
Amaral MB, Buchholz I, Freire-Maia B, Reher P, de Souza PE, Marigo Hde A, et al. Advanced osteosarcoma of the maxilla: a case report. Med Oral Patol Oral Cir Bucal. 2008 Aug 1. 13(8):E492-5. [Medline].
Telera S, Carapella C, Covello R, Cristalli G, Carosi MA, Pichi B, et al. Malignant peripheral nerve sheath tumors of the lateral skull base. J Craniofac Surg. 2008 May. 19(3):805-12. [Medline].
Vieira-Leite-Segundo A, Lima Falcão MF, Correia-Lins Filho R, Marques Soares MS, López López J, Chimenos Küstner E. Multiple myeloma with primary manifestation in the mandible: a case report. Med Oral Patol Oral Cir Bucal. 2008 Apr 1. 13(4):E232-4. [Medline].
Burger PC, Scheithauer BW, Vogel FS. Surgical Pathology of the Nervous System and Its Coverings. 3rd ed. Churchill Livingstone. 1991:1-66.
Hayes SM, Jani TN, Rahman SM, Jogai S, Harries PG, Salib RJ. Solitary extra-skeletal sinonasal metastasis from a primary skeletal Ewing's sarcoma. J Laryngol Otol. 2011 Aug. 125(8):861-4. [Medline].
Huvos AG. Bone Tumors: Diagnosis, Treatment and Prognosis. WB Saunders Company. 1979:
Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel AM. Intracranial chondrosarcoma: review of literature and report of 15 cases. J Neurol Neurosurg Psychiatry. July 1998. 65(1):88-92.
Mirra JM. Bone Tumors: Clinical, Radiological and Pathological Correlations. Lea and Febiger. 1989:
Rengachary SS, Wilkins RH, eds. Neurosurgery. 2nd ed. McGraw-Hill. 1996:1503-1528.
Sen CN, Sekhar LN, Schramm VL, Janecka IP. Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery. Dec 1989. 25(6):931-940.
Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. 2004 May. 111(5):997-1008. [Medline].
Thomas JE, Baker HL Jr. Assessment of roentgenographic lucencies of the skull: a systematic approach. Neurology. 1975 Feb. 25(2):99-106. [Medline].
Unni KK. Dahlin's Bone Tumors: General Aspects and Data on 11,087 Cases. 5th ed. Lippincott Williams & Wilkins. 1996:
Yamaguchi S, Nagasawa H, Suzuki T, et al. Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years. Clin Oral Investig. 2004 Jun. 8(2):52-5. [Medline].