Updated: Oct 13, 2009
Tumors of the skull are uncommon lesions that are not reported systematically in the medical literature. Therefore, assessing their true incidence and consequences to the health of the general population is difficult. Recent diagnostic advances have made such lesions easier to recognize, and new skull-base surgery techniques have provided access to previously inoperable skull tumors. Skull tumors are estimated to account for approximately 1% of bone tumors.
Treatment for most tumors is not controversial. However, the differentiation and identification of the tumor type is the greatest clinical challenge. The usual presentation is an enlarging skull mass, with or without pain, or cranial nerve deficits if the tumor involves the base of the skull.
Plain skull radiography with special projections is an important diagnostic tool. The initial classification of a lesion into radiolucent (osteolytic) or radiopaque (osteoblastic) is of considerable significance. The presence of sharply defined or irregular margins, presence or absence of sclerotic borders, and calcifications in the lesion are also important. Head CT scanning, with and without contrast, is useful in determining the extent of intracranial extension and other tumor characteristics.
Most skull tumors share certain MRI characteristics, such as hypointensity on T1-weighted images, hyperintensity on T2-weighted images, and some degree of contrast enhancement. The capability of imaging in multiple planes and enhanced soft tissue discrimination has made MRI an important diagnostic tool. The classification of benign and malignant brain tumors is based on that by Wilkins and Rengachary15 (which is a modified version of the system outlined by Huvos9 ).
The tumor type and behavior determine radiographic appearance (eg, radiolucent, radiopaque). Depending on the primary proliferating cell, benign skull tumors can be any of the following:
One of the most comprehensive series of bone tumors with classification originated from the Mayo Clinic. Of the 7975 patients in the series, 4% had tumors involving the skull (excluding the mandible, maxilla, and nasal cavity). Of these tumors, 19% were benign and 81% were malignant. Because the Mayo Clinic is a tertiary referral center, this series probably reflects some degree of selection bias. Other studies estimate that skull tumors comprise 1% of bone tumors.
Bone-forming tumors: Osteomas are the most common primary brain tumors of the calvaria, affecting 0.4% of the general population. Osteoid osteomas and ossifying fibromas are rare. Osteoblastomas account for approximately 1% of bone tumors.
Cartilage-forming tumors: Chondromas and chondromyxoid fibromas are rare. Chondroblastoma, although rare in some studies, accounted for 10% of the benign skull tumors in the Mayo series.
Connective tissue tumors: Desmoplastic fibroma is very rare in the skull (in the literature, only case reports exist).
Histiocytic tumors: Giant cell granuloma, nonossifying fibroma, and xanthoma are very rare in the skull.
Tumors of blood or blood vessel origin: Eosinophilic granuloma commonly affects the skull. Hemangiomas account for 10% of benign skull tumors (70% in the Mayo series).
Lymphangiomas: These tumors are rare.
Miscellaneous conditions: Aneurysmal bone cysts, epidermoid and dermoid tumors, intraosseous meningiomas, and fibrous dysplasia are relatively rare conditions. The prevalence of Paget disease is believed to be 1-5% in those older than 40 years, with involvement of any bone in the body, but most individuals remain asymptomatic and the condition is undiagnosed.
Most tumors demonstrate no sex predilection.
Physical findings vary according to tumor type.
Benign skull tumors are sporadic in occurrence. However, specific syndromes involving skull tumors have been described.
| Brainstem Gliomas | Migraine Headache |
| Cerebral Aneurysms | Migraine Variants |
| Chronic Paroxysmal Hemicrania | Multiple Sclerosis |
| Cluster Headache | Neural Tube Defects |
| Craniopharyngioma | Oligodendroglioma |
| Ependymoma | Primary Malignant Skull Tumors |
| Glioblastoma Multiforme | Sarcoidosis and Neuropathy |
| Meningioma | Subarachnoid Hemorrhage |
Aneurysmal bone cyst
Angiosarcoma
Bone-forming tumor
Brainstem syndromes
Chondroblastoma
Chondroma
Chondromyxoid fibroma
Chondrosarcoma
Chordoma
Congenital hemolytic anemia
Connective tissue tumor
Desmoplastic fibroma
Dermoid
Eosinophilic granuloma
Encephalocele
Epidermoid
Fibrosarcoma
Fibrous dysplasia
Gardner syndrome
Giant cell granuloma
Giant cell tumor
Hand-Schüller-Christian disease
Hemangioma
Hyperparathyroidism
Lymphangioma
Maffucci syndrome
McCune-Albright syndrome
Meningoencephalocele
Multiple myeloma
Neurocytoma
Nonossifying fibroma
Ollier syndrome
Ossifying fibroma
Osteoblastoma
Osteoid osteoma
Osteoma
Osteoporosis
Osteosarcoma
Osteomyelitis
Pacchionian depression
Paget disease
Syphilis
Tuberculosis
Venous lakes of the skull
Xanthoma
Laboratory studies are not helpful in making the diagnosis.
Biopsy of the lesion is of paramount importance for establishing the diagnosis and considering treatment options.
Osteomas are composed of mature lamellar bone. The typical appearance is a nidus of osteoid tissue in a background of osteoblastic connective tissue, which is enclosed completely by reactive bone. Ossifying fibroma consists of fibrous spindle cells with varying amounts of woven bone. The periphery of the tumor is composed of mature lamellar bone.
Osteoblastoma consists of a fibrous stroma with irregular osteoid deposition. Chondromas (enchondroma, juxtacortical chondroma, osteochondroma) are rare skull tumors consisting of mature hyaline cartilage. Chondroblastomas consist of immature cartilage cells.
Chondromyxoid fibroma is characterized by chondroid and myxoid differentiation with lobular growth. Desmoplastic fibroma is of fibrous connective tissue origin marked by the formation of collagen. Giant cell granuloma manifests with giant cells around hemorrhagic foci, numerous spindle-shaped fibroblastic cells, and new bone formation. The tumor cells are smaller than those of the giant cell tumor of the bone, whereas stromal cells and giant cells resemble each other.
Nonossifying fibroma and xanthoma consist of fibroblast proliferation with multinucleated giant cells and foamy xanthomatous cells. In eosinophilic granuloma, mononuclear histiocytes are mixed with eosinophils. Giant cells and areas of hemorrhage or necrosis may also be observed. The histiocytes stain positive for the protein S-100. On electron microscopy, the Birbeck granules that characterize the Langerhans or X cells are noted. Hemangiomas are visualized macroscopically as brownish red lesions under the skull periosteum. Microscopically, they consist of capillary, cavernous, or venous blood vessels.
Lymphangiomas consist of lymph vessels. Aneurysmal bone cysts consist of large vascular spaces separated by trabeculae of connective tissue and bone. The vascular spaces lack endothelial lining. Epidermoids consist of an epithelial capsule filled by desquamated epithelial cells and keratin.
Dermoids usually contain hair follicles and sebaceous and sweat glands. Fibrous dysplasia is a developmental anomaly in which the normal bone formation is arrested at the woven stage; thus, lamellar bone is not formed. This results in an overgrowth of the fibrous tissue among woven bone, which is the typical histologic feature of this lesion. Paget disease is initially characterized by increased osteoclastic activity, which results in bone resorption, followed by increased osteoblastic activity and bone formation.
The goals of pharmacotherapy are to reduce morbidity and prevent complications.
Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.
DOC for mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
200-800 mg PO q6-8h while symptoms persist; not to exceed 3.2 g/d
<6 months: Not established
6 months to 12 years: 20-70 mg/kg/d PO divided tid/qid; start at lower end of dosing range and titrate; not to exceed 2.4 g/d
>12 years: Administer as in adults
May decrease effects of loop diuretics; may increase PT in patients taking anticoagulants (monitor PT carefully and watch patient for signs of bleeding); may increase serum lithium levels and risk of methotrexate toxicity (eg, stomatitis, bone marrow suppression, nephrotoxicity); probenecid may increase concentrations and probably toxicity of NSAIDs; consider effects on platelet function and gastric mucosa
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Caution in congestive heart failure, hypertension, and decreased renal or hepatic function; caution in anticoagulation abnormalities or during anticoagulant therapy (monitor PT); acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion are at greatest risk of acute renal failure; low WBCs are rare and transient (usually return to normal as therapy continues); persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuing drug
Pain control is essential to quality patient care. These agents ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who have moderate to severe pain.
Indicated for treatment of mild to moderate pain.
15-60 mg PO q4h prn pain
0.5-1 mg/kg/dose PO q4-6h prn pain
Toxicity increases with CNS depressants or TCAs
Documented hypersensitivity
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Significant abuse potential; may cause withdrawal headaches; may result in acute opiate withdrawal symptoms in patients dependent on opiates; caution in severe renal or hepatic dysfunction; babies born to mothers using narcotics regularly may show signs of withdrawal; be aware of total daily dose of acetaminophen; do not exceed 4000 mg/24h of acetaminophen; higher doses may cause liver toxicity
Drug combination indicated for relief of moderate to severe pain; DOC for aspirin-hypersensitive patients.
1-2 tab or cap PO q4-6h prn pain
0.05-0.15 mg/kg/dose oxycodone PO q4-6h prn; not to exceed 5 mg/dose oxycodone
Phenothiazines may decrease analgesic effects; toxicity increases with coadministration of either CNS depressants or TCAs
Documented hypersensitivity
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Duration of action may increase in elderly patients; be aware of total daily dose of acetaminophen; do not exceed 4000 mg/24h of acetaminophen; higher doses may cause liver toxicity
Drug combination indicated for moderate to severe pain.
1-2 tab or cap PO q4-6h prn pain
<12 years: 10-15 mg/kg/dose acetaminophen q4-6h prn; not to exceed 2.6 g/d acetaminophen
>12 years: 750 mg acetaminophen q4h; not to exceed 10 mg hydrocodone bitartrate per dose or 5 doses/24h
Coadministration with phenothiazines may decrease analgesic effects; toxicity increases with CNS depressants or TCAs
Documented hypersensitivity; elevated intracranial pressure
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Tablets contain metabisulfite, which may cause allergic reactions; caution in severe renal or hepatic dysfunction; be aware of total daily dose of acetaminophen; do not exceed 4000 mg/24h of acetaminophen; higher doses may cause liver toxicity
Can reduce inflammation and pain symptoms.
Treats mild to moderate pain and headache. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.
325-650 mg PO q4-6h for osteoid osteoma
Not established
Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; use in children ( <16 y) with flu because of association of aspirin with Reye syndrome
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with history of blood coagulation defects, or who are taking anticoagulants
Inpatient care is not usually required unless skull-base surgery is needed for nerve decompression.
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Huvos AG. Bone Tumors: Diagnosis, Treatment and Prognosis. Philadelphia, Pa: WB Saunders Company; 1979.
Keyserling H, Peterson K, Camacho D, Castillo M. Giant cell angiofibroma of the orbit. AJNR Am J Neuroradiol. Aug 2004;25(7):1266-8. [Medline].
Mirra JM. Bone Tumors: Clinical, Radiological and Pathological Correlations. Philadelphia, Pa: Lea and Febiger; 1989.
Morris JM, Lane JI, Witte RJ, Thompson DM. Giant cell reparative granuloma of the nasal cavity. AJNR Am J Neuroradiol. Aug 2004;25(7):1263-5. [Medline].
Thomas JE, Baker HL Jr. Assessment of roentgenographic lucencies of the skull: a systematic approach. Neurology. Feb 1975;25(2):99-106. [Medline].
Unni KK. 5th ed. Dahlin's Bone Tumors: General Aspects and Data on 11,087 Cases. Philadelphia, Pa: Lippincott Williams & Wilkins; 1996.
Wilkins RH, Rengachary SS. 2nd ed. Neurosurgery. New York, NY: McGraw-Hill; 1996:1503-1528.
Youmans JR. 5th ed. Neurological Surgery. Philadelphia, Pa: WB Saunders Company; 2004:3227-3268.
skull, tumor, aneurysmal bone cyst, bone-forming tumor, chondroma, chondroblastoma, chondromyxoid fibroma, connective tissue tumor, desmoplastic fibroma, dermoid, encephalocele, eosinophilic granuloma, epidermoid, fibrous dysplasia, giant cell granuloma, Gardner's syndrome, Hand-Schüller-Christian disease, hemangioma, lymphangioma, Maffucci's syndrome, McCune-Albright's syndrome, meningoencephalocele, nonossifying fibroma, Ollier's syndrome, ossifying fibroma, osteoblastoma, osteoid osteoma, osteoma, pacchionian depression, venous lakes of the skull, xanthoma
Draga Jichici, MD, FRCP, Associate Clinical Professor, Department of Medicine, Division of Neurology and Critical Care Medicine, McMaster University, Canada
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, and Texas Medical Association
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment
Jorge Kattah, MD, Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria
Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences
Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting
Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Efstathios Papavassiliou, MD to the development and writing of this article.
Related eMedicine topics
Skull Base, Benign Tumors
Skull Base Tumors
Skull Base, Reconstruction
Primary Malignant Skull Tumors
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