Primary Malignant Skull Tumors Clinical Presentation

Updated: Aug 09, 2017
  • Author: Draga Jichici, MD, FRCP, FAHA; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Presentation

History

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  • 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. [7]

  • 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

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Physical

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  • 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

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Causes

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  • 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.

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