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.

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 of multiple myeloma for 2 years, showing multiple lytic lesions that involve both the inner and outer tables as well as the diploë.
  View Media Gallery
- 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.

Other Tests

See the list below:

Bone marrow biopsy

Procedures

See the list below:

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

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.

Treatment & Management

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Media Gallery

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.

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Author

Draga Jichici, MD, FRCPC, FAHA Associate Clinical Professor, Division of Medicine, Department of Critical Care Medicine and Neurology, Co-Director, Neurosurgical Intensive Care Unit, Co-Director, Transcranial Doppler Ultrasound, Hamilton Health Sciences, McMaster University School of Medicine, Canada

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

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

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, The Society of Federal Health Professionals (AMSUS), Child Neurology Society, Southern Pediatric Neurology Society

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.