Primary Malignant Skull Tumors

Primary Malignant Skull Tumors

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Depending on the primary proliferating cell, both malignant and benign skull tumors can be any of the following:

Bone forming

Cartilage forming

Of connective tissue origin

Histiocytic [1]

Of blood or blood vessel origin

Metastatic to bone

Of neuroepithelial origin

Of squamous cell origin

Of apocrine gland (ie, major and minor salivary, lacrimal) origin [2]

Salivary gland tumors, as well as other malignancies of the head and neck, such as squamous cell carcinoma and esthesioneuroblastoma, may invade the skull base by proximity or by perineural invasion. [3, 2, 4] These tumors cause cranial nerve paralysis by invasion or direct extension; accompanying pain is due to erosion of the involved structures. Involvement of the periosteum or dura is the primary mechanism of direct tumor spread and the causative pathology.

United States

One of the most comprehensive reported series of bone tumors came from the Mayo Clinic. Of the 7975 bone tumors in the series, 4% involved the skull (excluding the mandible, maxilla, and nasal cavity), 19% were benign, and 81% malignant. As the Mayo Clinic is a tertiary referral center, some degree of selection bias probably was in effect.

Other studies estimate that skull tumors constitute 1% of bone tumors.

Bone-forming tumors: Osteosarcoma is the second most frequent malignant skull tumor after multiple myeloma, accounting for 13% of this series.

Cartilage-forming tumors: Chondrosarcoma is the third most common malignant bone tumor, with a frequency of 11-12.5%.

Connective tissue tumors: Fibrosarcoma accounts for fewer than 5% of these tumors.

Histiocytic tumors

Ewing sarcomas account for about 5% of these tumors.

Giant cell tumors (osteoclastomas) also account for about 5% of these tumors. [5]

Tumors of blood or blood vessel origin: Angiosarcomas are rare malignant tumors.

Squamous cell carcinomas of the temporal bone occur with a frequency of 1 case per 25,000 patients with chronic otitis.

See the list below:

Recurrent sinusitis is a common complication of tumors affecting the sinuses.

If the excision is incomplete, many tumors can recur.

Cranial nerve compression can occur in skull-base tumors.

Metastasis

Death

Persistence of disease after treatment is a serious problem, since it leads to persistent morbidity; patients have a significant decrease in the quality of life prior to dying from the disease. The aim of therapy is to control the disease locally.

No racial predilection exists for any malignant skull tumor.

See the list below:

Most malignant skull tumors have no sex predilection (except possibly metastatic disease).

Fibrosarcoma, Ewing sarcoma, and chordomas occur more frequently in men than in women.

See the list below:

Bone-forming tumors and fibrosarcomas usually present in middle-aged adults.

Cartilage-forming tumors may present at any age, with a peak during the second decade.

Ewing sarcoma is a disease of childhood, whereas giant cell tumors are seen mainly between the second and fourth decades. [6]

Angiosarcoma can present at any age.

Multiple myeloma is more common in older adults.

Chordomas usually present in the third or fourth decade.

Metastatic tumors follow the pattern of the primary tumor.

Squamous cell carcinomas occur in the older adult, often in the sixth decade.

Esthesioneuroblastomas occur in young adults in their 30s and 40s.

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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].

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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].

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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].

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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].

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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].

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.

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.

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.

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.

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.

Primary Malignant Skull Tumors

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