graphically normal examination. Any cosmetic surgical intervention should be delayed until growth of the patient has ceased. Few cases of cherubism in conjunction with Noonan syndrome have been reported.5*6 Neither cherubism nor Noonan syndrome was discovered in an investigation of this patient’s family history. While Noonan syndrome does not typically include maxillofacial tumors, it can present with a significant number of head and neck anomalies. These factors, combined with the relative rarity of discovering these conditions separately, let alone in conjunction with each other, supported our efforts to obtain biopsies of representative lesions. A clinical diagnosis of cherubism was confirmed histologically. Since this patient does not manifest any degree of mental retardation or debilitating car-

J Oral Mamllofac


diac disease (both prognostic indicators in Noonan syndrome), we expect to be able to follow him long enough to see his cherubism resolve. References I. Shafer WC, Hine MK, Levy BM: A Textbook of Oral Pathology. Philadelphia, PA, Saunders, 1983, pp 699-702 2. Wyngaarden JB, Smith LH: Cecil Textbook of Medicine. Philadelphia, PA, Saunders, 1988, p 1203 3. Wyngaarden JB. Smith LH: Cecil Textbook of Medicine. Philadelphia, PA, Saunders, 1988, p 1414 4. Goodman RM, Gorlin RJ: Atlas of the Face in Genetic Disorders. St Louis, MO, Mosby, 1977, pp 168-169 5. Dunlop C, Neville B, Vickers RA. et al: The Noonan syndrome/cherubism association. Oral Surg 67:698, 1989 6. Gorlin RJ, Cohen MM Jr, Levin LS: Syndromes of the Head and Neck teds). New York, NY. Oxford University Press, 1990




of the Frontal Bone:

Review of the Literature and Report of a Case THOMAS



Hemangiomas of bone are rare lesions comprising only 0.7% of all osseous neoplasms.’ They occur most frequently in the vertebral bones, followed by the calvarium. Calvarial hemangiomas represent 0.2% of osseous neoplasms, with the parietal and frontal bones being the most frequent sites.’ A review of the literature revealed reports of 23 frontal bone hemangiomas to date (Table 1). A case involving a cavernous hemangioma of the right frontal bone is presented. Report of a Case A 51-year-old man complained of a right supraorbital swelling present for the last 8 years. The mass had inReceived from the University of Missouri-Kansas City Schools of Medicine and Dentistry, Truman Medical Center Department of Oral and Maxillofacial Surgery Graduate Training Program. * Chief Resident. t Chairman and Program Director; Director, Graduate Training OMFS Trinity Lutheran Hospital. Address correspondence and reprint requests to Dr Hiatt: Department of Oral and Maxillofacial Surgery, Truman Medical Center, 2301 Holmes St, Kansas City, MO 64108. 0 1991 American geons



of Oral and Maxillofacial


in size over the last 1% years. The patient denied visual impairment, headache, neurological, or systemic symptoms. The patient’s past medical history was negative. Physical examination revealed a 4.5 x 3.5-cm protuberance in the right supraorbital region (Fig 1). The mass was nontender, hard, and immobile. The overlying skin demonstrated normal mobility and consistency. Eye examination was normal. A computed tomography (CT) scan demonstrated a well-circumscribed, radiodense mass in the right supraorbital rim. The dense cortical bone showed radiating spicules in a “sun-ray” pattern. The mass did not appear to involve the orbit or orbital contents. The entire anterior table of the right frontal sinus was replaced by the lesion. The frontal sinus, posterior table, and intracranial contents were normal (Figs 2 and 3). The patient was taken to the operating room and a bicoronal flap was made. A purplish, a bony hard mass was noted (Fig 4). The supraorbital and supratrochlear neurovascular bundles were seen below the periosteum on the anterior margin of the lesion and were gently freed using fine osteotomes. After exposing the lesion, a Lindemann bur was used to section the mass similar to a torus removal procedure. Initial excision of the mass revealed dense outer cortical bone with cancellous bone in the interior. There was no unusual hemorrhage. Rongeurs were used to remove a surrounding normal margin of bone. This procedure necessitated entering the right frontal sinus. The frontal sinus appeared normal with a patent frontal-nasal duct (Fig 5). The remainder of the mass was removed circumferentially from the normal surrounding creased



Table 1. Reported Cases of Calvarfal Hemangioma Year Pilche? Lanari & Marque’ Eros’ Mazzini & Brachetto-Brian’ Abbott” Janker” Rowbotham” Keleman & Holmes” Holmes. Sweet. & Keleman14 Davis, Rumbaugh, & Petty” Atallah & Nassarlh Roy, Roy. & Biswas” Schneider, Gabrielson, & Hicks’s Davis & Morgan” Gupta, Tiwari. & Pasupathy’” Fouad & Khalifa” Rothstein. Maisel, & Miller”

1894 1925 1928 1935 1937 1941 1942 1948 1952 1966 1971 1973 1973 1974 1975 1979 1985

No. of Cases 1 2 1 1 1 1 3

I 3 1 1 1 1 1 1 2


FIGURE 2. A well-circumscribed radiodense mass of the right supraorbital rim shown on CT scan. The anterior table of the frontal sinus is involved by the lesion. The frontal sinus is normal.

Wyke reported a thorough review of the world literature regarding calvarial hemangiomata in 1949.’ It was found in this study that calvarial hemangiomas occur most frequently in the fourth decade and three times more frequently in females than in males. The parietal bone is most frequently involved, followed by the frontal bone. Diagnosis of

these lesions depends primarily on the radiographic features. Most calvarial hemangiomas are of the cavernous variety and are benign. They comprise 10% of the primary benign neoplasms of the skull.’ It is not possible to diagnose calvarial hemangiomas by clinical examination and history. Current theories regarding this lesion postulate a congenital origin.3 Most lesions occur without a previous history of trauma; however, some lesions are secondary to an injury. The typical history is one of a slowly enlarging, bony hard, nonpainful swelling. However, pain can occur late in the course as the lesion expands and presses on dura, although this is rare. External expansion is the more usual growth pattern. Orbital and petrotemporal lesions, however, can cause local symptoms quite early. Hemangiomata in the orbital region can cause proptosis, diplopia, and other visual problems, while the petrotemporal lesions often produce deafness, tinnitus, and facial paresis. Involvement of the petrous area can produce symptoms of an expanding cholesteatoma.

FIGURE 1. View of patient showing the 4.5 the right supraorbital rim.

FIGURE 3. Lower CT cut showing no involvement globe or periorbita.

Total cases


frontal bone. The postexcision defect involved the majority of the right supraorbital rim and anterior wall of the frontal sinus. Iliac crestal bone was obtained and contoured to cover the defect. The piece was slightly overcontoured to adjust for the likely resorption that would occur and was wired in place (Fig 6). The patient’s recovery and follow-up have been uneventful. The final pathology report was consistent with cavernous hemangioma (Figs 7 and 8). Discussion


3.5-cm mass on

of the




FIGURE 4. A discrete bony mass is seen after reflection of the bicoronal flap. The supratrochlear and supraorbital neurovascular bundles are within the mass.

The outer table of the frontal bone is usually involved and the inner table is spared. Bruits are uncommon.22 Sherman and Wilner have described the radiographic appearance of hemangiomas in various locations.2 Typically, a single oval or round area of rarefaction is seen that can vary in size from 1 to 7 cm in diameter. The internal pattern is characterized by delicate reticulations assuming a “honeycomb” configuration. These striations occasionally are accentuated to create a “sunburst” or “sunray” appearance. The outer table usually is destroyed by the lesion, whereas the inner table is usually normal. The margins are sharp and well delineated. Other lesions with similar clinical and radiographic presentations are meningioma, cho-

lesteatoma, fibrous dysplasia, osteoma, and osteogenic sarcoma. The striations seen with meningioma on the radiograph are parallel rather than radial, as seen in hemangioma. Reactive bony sclerosis and expansion of the inner cortical table seen with meningiomas also helps to differentiate them from hemangiomas. Cholesteatomas are radiolucent and surrounded by a zone of sclerotic bone. Other potential radiolucent lesions of the calvarium to be considered are metastatic carcinoma, multiple myeloma, Hand-&huller-Christian disease, and the rare malignant vascular bone tumors, hemangiopericytoma and hemangioendothelioma.4 Fibrous dysplasia has a “ground glass” appearance. It is found usually in the second and third decades and more commonly in the maxilla. Osteomas radiographically present as uniform areas of

FIGURE 5. The frontal sinus uncovered during the operation. A normal frontonasal duct is present.


FIGURE 6. Right supraorbital rim and anterior table reconstructed with an autogenous iliac chest corticocancellous bone


increased density. Osteogenic sarcoma also is associated with a “sunburst” appearance, but is quite rare in the calvarium. The clinical course of osteogenic sarcoma is rapid and usually fatal. On the radiograph, the poorly defined borders and disruption of both inner and outer tables aids in differentiating osteogenic sarcoma from hemangioma.” Macroscopic inspection of a calvarial hemangioma reveals a hard, deep-blue or purplish exostosis distinctly separated from the surrounding bone. The periosteum reflects easily from the lesion. Microscopic analysis identifies two types of lesions: cavernous and capillary.’ Cavernous hemangioma constitutes the majority of calvarial lesions and consists of large, blood-filled, endothelial-lined spaces.

FIGURE 7. Photomicrograph showing cavernous spaces filled with blood between the bone trabeculae. The spaces are lined by benign flattened endothelium. Some areas of increased osteoblastic activity exist (hematoxylineosin stain, original magnification X 125).

These spaces are surrounded by a fibrous stroma of varying cellularity. Diploic veins are considered to be a possible source of calvarial hemangioma. Theoretically, the hemangioma expands by projecting endothelial buds into the surrounding bone. The buds then canalize and fill with blood. Osteoblasts and osteoclasts are seen throughout the lesion, with normal trabeculae interspersed in the tumor, thus documenting that continual bony remodeling is occurring. Capillary hemangiomas have numerous tortuous pathways of small blood-filled spaces. and are not normally found in the skull. The definitive surgical treatment of hemangiomas is excision with a small margin of normal bone. Resecting a margin of normal bone reduces bleeding.

1022 Proximal vessel control may be necessary for large lesions with feeder vessels documented by angiography. Preoperative angiography should be considered for lesions with bruits, atypical radiographic appearance, and vascular symptoms. Radiation treatment is reserved for unresectable tumors. Bony deformity will remain following radiation therapy, but tumor growth will be curtailed. Immediate reconstruction following surgical resection is desirable. Acknowledgment The authors would like to thank Drs Daniel Bennett, Eric Swanson, and Frederick Hutton for their surgical expertise regarding this case.

References 1. Wyke BD: Primary hemangioma of the skull: A rare cranial tumor. Am J Roentgen01 Radium Ther Nucl Med 61:302, 1946 2. Sherman RS, Wilner D: The roentgen diagnosis of hemangioma of bone. Am J Roentgen01 Radium Ther Nucl Med 86: 1146, 1961 3. Jaffe HL: Tumors and Tumorous Conditions of the Bone and Joints. Philadelphia, PA, Lea & Febiger, 1958 4. Toynbee J: An account of two vascular tumors developed in the substance of bone. Lancet 2:676, 1845 5. Lichtenstein L: Bone tumors (ed 4). St Louis, MO, Mosby, 1972, pp 166-169 6. Pilcher LS: Venous Tumor of the diploe. Tr Am Surg Assn 12:283, 1894 7. Lenair F, Marque A: Angioma of the diploe. Rev Sot Argent Radio1 Electrol 1:70, 1925



8. Eros G: Multiples Hamangioma der Schadel-knochen. Centrabl f allg Path u path Anat 43:532, 1928 9. Mazzini OF, Brachetto-Brian D: Angioma de frontal. Craniectomia. Bol Trab Sot Buenos Aires 19:363, 1935. also, Prensa Med Argent 23:1249, 1936 10. Abbott WD: Angioma of the skull. Ann Surg 113:306, 1941 11. Janker R: Excellent results of radium therapy of extensive cavernous hemangioma of face and cranium. Zentrabl Chir 68: 1975, 1941 12. Rowbotham GF: Hemangiomata arising in bones of the skull. Br J Surg 30:1, 1942 13. Kelemen G, Holmes EM: Cavermous haemangioma of frontal bone. J Laryngol Otol 62557, 1948 14. Holmes FM, Sweet WH, Kelemen G: Hemantiomas of frontal bone; report of three cases. Ann Otol Rhino1 Laryngol 61:45, 1952 IS. Davis DO, Rumbaugh CL, Petty J: Calvarial hemangioma: Tumor stain and meningeal artery blood supply, case report. J Neurosurg 25:561, 1966 16. Atallah NK, Nassar SI: Calvarial hemangioma with blood supply from branches of the internal carotid artery, case report. J Neurosurg 34:823, 1971 17. Roy RN. Roy A, Biswas SK: Primary haemangioma of the skull. Neural India 21:46, 1973 18. Schneider RC, Gabrielson TO, Hicks SP: Calvarial hemangioma, the value of selective external carotid angiography in surgical excision of the lesion. Neurology 23:352, 1973 19. Davis E, Morgan R: Hemangioma of bone. Arch Otolaryngol 99:443, 1974 20. Gupta SD, Tiwari IN, Pasupathy NK: Cavernous haemangioma of the frontal bone: Case report. Br J Surg 62:330, 1975 21. Fouad HA, Khalifa MC: Haemangioma of the frontal bone. J Laryngol Otol 93:513, 1979 22. Rothstein J, Maisel RH, Miller R. et al: Mixed cavernous and capillary hemangioma of the frontal bone. Ear Nose Throat J 64:43. 1985

Hemangioma of the frontal bone: review of the literature and report of a case.

1018 HEMANGIOMA graphically normal examination. Any cosmetic surgical intervention should be delayed until growth of the patient has ceased. Few cas...
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