Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Primary Malignant Skull Tumors Workup

  • Author: Draga Jichici, MD, FRCP, FAHA; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
 
Updated: Dec 11, 2014
 

Laboratory Studies

See the list below:

  • Anemia, an elevated white blood count (WBC), and reduced platelets are characteristic of malignancies involving the bone marrow.
  • Immunoglobulins (eg, IgG, IgA, IgM, IgD) are elevated in multiple myeloma.
  • Protein M can be found in the urine and serum in myeloma.
Next

Imaging Studies

See the list below:

  • Plain skull x-ray film and head CT scan
    • Most of the malignant skull tumors appear as radiolucent lesions, single or multiple, with irregular borders and no periosteal reaction.
    • Osteosarcomas appear as osteolytic soft-tissue extensions. They may have calcification within the lesion, no periosteal reaction, and poorly defined margins. The typical (but not frequent) appearance is the sun-ray picture.
    • Chondrosarcomas have no reliable radiological features and are characterized by lytic and sclerotic changes within poorly defined margins.
    • Fibrosarcomas are radiolucent lytic lesions with thinning and widening of the cortex, minimal periosteal involvement, and irregular margins.
    • Ewing sarcoma has a typical onion skin appearance with laminated periosteal changes. On CT scan, it appears as an isodense mass surrounded by a hypodense area and hyperostosis. It is also contrast enhancing.
    • Angiosarcomas are destructive lesions with cortical erosion and reactive ossification. On CT scan, they show heterogeneous enhancement with focal necrosis.
    • Plasmocytomas/multiple myelomas usually present as multiple lytic lesions that involve both the inner and outer tables, as well as the diploë from which they arise. On CT scan, depicted below, they are hyperdense, homogeneous enhancing lesions.
      Head CT scan of a 60-year-old man with a history oHead CT scan of a 60-year-old man with a history of multiple myeloma for 2 years, showing multiple lytic lesions that involve both the inner and outer tables as well as the diploë.
    • Chordomas are soft-tissue masses usually seen in the nasopharyngeal area with various degrees of calcification. CT scan shows a soft-tissue mass with extensive bone destruction.
    • Metastatic neoplasms can be either of the following:
      • Osteoblastic, with sclerosis and thickening (eg, prostate, breast, bladder, hypernephroma)
      • Osteoclastic, with bone destruction and lucency (eg, lung, uterus, GI tract, thyroid, melanoma, neuroblastoma)
    • Giant cell tumors usually involve the sphenoid bone and commonly erode the sellar region. On brain CT scan, giant cell tumors are hyperdense, contrast-enhancing masses.
  • MRI
    • Most tumors are hypointense on T1-weighted images and hyperintense with heterogeneous signal on T2-weighted images.
    • Enhancement is common.
  • Bone scan: 99m technetium scan shows all malignant processes as hot areas.
  • Arteriogram: Tumors of vessel origin or associated with multiple myeloma have a high degree of vascularity.
Previous
Next

Other Tests

See the list below:

  • Bone marrow biopsy
Previous
Next

Procedures

See the list below:

  • Biopsy of the lesion is crucial for establishing the diagnosis and deciding on treatment options.
Previous
Next

Histologic Findings

See the list below:

  • Osteosarcomas are composed of a malignant spindle cell stroma, which directly produces osteoid or immature bone (osteoblastic, chondroblastic, or fibroplastic form).[9]
  • Low-grade chondrosarcomas (myxochondrosarcoma) are characterized by chondroid and immature cartilage deposition in areas of myxomatous change and cystic degeneration. The high-grade type (mesenchymal chondrosarcoma) is characterized by the absence of cartilage lobules and the presence of fibrosarcomatous areas. Groups of chondromatous cells lose their usual lobulation and begin to spindle out. Both types are vimentin positive.
  • Fibrosarcoma is characterized by varying amounts of collagen production and the absence of bone, osteoid, or cartilage. The medullary subtype has a better prognosis than the periosteal subtype.
  • Giant cell tumor (osteoclastoma) consists of a well-vascularized tissue mass of plump, spindle, or ovoid stroma cells together with uniformly dispersed, numerous, large, multinucleated giant cells.
  • Ewing sarcoma appears as uniform, densely packed small cells with indistinct cytoplasmic borders and many mitotic figures. They stain strongly with periodic acid-Schiff (PAS).[3]
  • Angiosarcomas (hemangiopericytoma or hemangioendothelioma) are characterized by the formation of irregular anastomosing vascular channels lined by one or more layers of atypical endothelial cells and pericytes, which have an anaplastic immature appearance.
  • Multiple myeloma is characterized by widespread osteolytic bone destruction by dense tumor cells that look like plasma cells clustered in close aggregates.[10]
  • Chordomas consist of physaliphorous cells (large, vacuolated, mucus containing) with a lobular arrangement and abundant extracellular mucoid tissue.
  • Metastatic tumors have the same or similar histologic features as their primary tumors.
Previous
 
 
Contributor Information and Disclosures
Author

Draga Jichici, MD, FRCP, FAHA Associate Clinical Professor, Department of Neurology and Critical Care Medicine, McMaster University School of Medicine, Canada

Draga Jichici, MD, FRCP, FAHA is a member of the following medical societies: American Academy of Neurology, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian Medical Protective Association, Neurocritical Care Society, Canadian Critical Care Society, Canadian Critical Care Society, Canadian Neurocritical Care Society, Canadian Neurological Sciences Federation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Jorge C Kattah, MD Head, Associate Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Spiros Manolidis, MD Associate Professor of Otolaryngology and Neurological Surgery, Columbia University

Spiros Manolidis, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Head and Neck Society, American Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Society of University Otolaryngologists-Head and Neck Surgeons, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Efstathios Papavassiliou, MD to the development and writing of this article.

References
  1. Krishnamurthy A. Malignant fibrous histiocytoma of the scalp: A rare differential with a dramatic clinical presentation. J Indian Assoc Pediatr Surg. 2014 Oct. 19(4):227-9. [Medline]. [Full Text].

  2. 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].

  3. Lane KA, Katowitz JA. Ewing sarcoma presenting as a subconjunctival mass. Ophthal Plast Reconstr Surg. 2009 Jan-Feb. 25(1):61-3. [Medline].

  4. 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].

  5. Meling TR, Fridrich K, Evensen JF, Nedregaard B. Malignant granular cell tumor of the skull base. Skull Base. 2008 Jan. 18(1):59-66. [Medline]. [Full Text].

  6. 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].

  7. Chugh AP, Gandhoke CS, Mohite AG, Khedkar BV. Primary angiosarcoma of the skull: A rare case report. Surg Neurol Int. 2014. 5:92. [Medline]. [Full Text].

  8. 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].

  9. 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].

  10. 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].

  11. Burger PC, Scheithauer BW, Vogel FS. Surgical Pathology of the Nervous System and Its Coverings. 3rd ed. Churchill Livingstone. 1991:1-66.

  12. 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].

  13. Huvos AG. Bone Tumors: Diagnosis, Treatment and Prognosis. WB Saunders Company. 1979:

  14. Kadar AA, Hearst MJ, Collins MH, Mangano FT, Samy RN. Ewing's Sarcoma of the Petrous Temporal Bone: Case Report and Literature Review. Skull Base. 2010 May. 20(3):213-7. [Medline]. [Full Text].

  15. 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.

  16. Mirra JM. Bone Tumors: Clinical, Radiological and Pathological Correlations. Lea and Febiger. 1989:

  17. Rengachary SS, Wilkins RH, eds. Neurosurgery. 2nd ed. McGraw-Hill. 1996:1503-1528.

  18. 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.

  19. 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].

  20. Thomas JE, Baker HL Jr. Assessment of roentgenographic lucencies of the skull: a systematic approach. Neurology. 1975 Feb. 25(2):99-106. [Medline].

  21. Unni KK. Dahlin's Bone Tumors: General Aspects and Data on 11,087 Cases. 5th ed. Lippincott Williams & Wilkins. 1996:

  22. 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].

Previous
Next
 
Head CT scan of a 60-year-old man with a history of multiple myeloma for 2 years, showing multiple lytic lesions that involve both the inner and outer tables as well as the diploë.
This head CT scan shows multiple lytic lesions of the skull involving both the inner and outer tables.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.