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Vol. 114 No.

1 July 2012

Enormous aneurysmal bone cyst of the mandible: case report and radiologic-pathologic correlation
Galal Omami, BDS, MSc,a Reji Mathew, BDS, MDS,b Dennis Gianoli, DDS,c and Alan Lurie, DDS, PhD,d Farmington and New Britain, Connecticut, and Downers Grove, Illinois
UNIVERSITY OF CONNECTICUT, MIDWESTERN UNIVERSITY COLLEGE OF DENTAL MEDICINE-ILLINOIS, AND THE HOSPITAL OF CENTRAL CONNECTICUT

A 33-year-old patient with a huge aneurysmal bone cyst (ABC) was imaged using cone-beam CT, MRI, and angiography. ABC is an uncommon non-neoplastic, expansile lesion of bone. Although common in the appendicular skeleton and spine, only 2% of the lesions occur in the craniofacial skeleton. The plain radiographic features of gnathic ABC may show an omni-expansile unilocular or multilocular radiolucency. Fluid-uid levels have been reported in cystic compartments of ABCs; however, this feature is not diagnostically specic for ABC. In this article, we present a case of a rapidly growing, extraordinarily large ABC of the posterior mandible, with emphasis on comparative imaging features of this lesion in cone-beam CT, MRI, and carotid angiography. (Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:e75-e79)

Aneurysmal bone cyst (ABC) is neither an aneurysm nor a true cyst, but it is an uncommon non-neoplastic, expansile lesion of bone. The ABC is dened by the World Health Organization as a benign intraosseous lesion, characterized by blood-lled spaces of varying size associated with a broblastic stroma containing multinucleated giant cells, osteoid, and woven bone.1 It commonly occurs in the appendicular skeleton and spine2; however, only 2% of the lesions occur in the craniofacial skeleton.3 Most ABCs of jaws occur in adolescents, predominantly those younger than 20. The lesion is more commonly seen in the mandible than maxilla (3:1), more commonly involving the posterior region of the mandible. The lesion appears to have a slight predilection for females. The pathogenesis of ABC remains uncertain, although most regard it as reactive to prior trauma or pathology.3 The radiographic features of ABC of jaws can vary from unilocular to multilocular expansile radiolucencies. Fluiduid levels may occur when substances of differing densities are contained within a cystic compartment. Fluid-uid levels have been reported to be associated
a

with ABC, demonstrating hemorrhages of different ages; however, this feature is not diagnostically specic.4 Surgical resection or curettage is the treatment of choice; in the jaws, curettage has higher recurrence rates than does resection.5 In this article, we present a case of a rapidly growing, extraordinarily large ABC of the posterior mandible, with emphasis on the imaging features in cone-beam computed tomography (CBCT), magnetic resonance imaging (MRI), and carotid angiographic images and their application to treatment planning of such lesions.

CASE REPORT
A 33-year-old African American woman was referred to the Section of Oral and Maxillofacial Radiology at the University of Connecticut School of Dental Medicine for CBCT evaluation of an extensive, painless, rapidly growing swelling of the left posterior mandible. The patient reported that this swelling had been growing over a period of 5 months. Extraoral examination revealed a huge swelling involving almost the entire lower left side of her face and submandibular region; digital palpation showed rm to hard texture without tenderness or egg-crackling sign. The overlying skin looked slightly erythematous. There was no signicant weight loss, and patients medical history was otherwise noncontributory. Intraoral examination was remarkable only for extensive dental caries. On initial panoramic examination, the lesion was so large that it extended off the image. It showed a faint radiolucent circular mass with a suggestion of a thinly corticated border superimposed over the inferior ramus, posterior body, and submandibular region, with severe destruction of bone inferior to the inferior alveolar canal giving a cookie bite appearance (Figure 1). CBCT imaging showed a well-dened, spheroid, grossly expansile, unilocular, low-density lesion arising from the basal portion of the left ramus and posterior mandibular body, and occupying the left suprahyoid neck. The lesion had caused ballooning of the medial, lateral, and inferior cortices

Diplomate, American Board of Oral and Maxillofacial Radiology; Senior Resident, Oral and Maxillofacial Radiology, University of Connecticut, School of Dental Medicine. b Assistant Professor, Oral and Maxillofacial Radiology, Midwestern University College of Dental Medicine-Illinois (CDMI). c Chair, Oral and Maxillofacial Surgery, the Hospital of Central Connecticut. d Chair, Oral and Maxillofacial Radiology, University of Connecticut, School of Dental Medicine. Received for publication Nov 23, 2011; returned for revision Jan 18, 2012; accepted for publication Jan 30, 2012. Published by Elsevier Inc. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2012.01.023

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Fig. 1. Panoramic radiograph showing radiolucent lesion causing destruction of left mandibular angle.

of the mandible, and extended anteroposteriorly from the level of left mandibular second premolar to the anterior border of the sternomastoid muscle. Mediolaterally, it extended laterally, creating discernible facial swelling, and medially to encroach on the lateral pharyngeal wall, causing slight airway shift toward the right side. Superoinferiorly, the lesion extended from the level of the coronoid notch of mandible inferiorly to the superior border of thyroid cartilage. The overall estimated dimensions were 67 72 75 mm. The lesion appeared to have destroyed, but not displaced, the oor of the inferior alveolar canal. The expanded cortical margins, whose cortication was intermittently effaced over a large portion of the periphery, were markedly undulating with numerous small indentations of the periosteal surface, and short wispy septa emanated from the endosteal surface of the bony covering (Figure 2). The appearance of this lesion suggested a locally destructive lesion of nonodontogenic origin and, subsequently, differential diagnosis was limited to ABC and central giant cell granuloma. MRI was performed 4 weeks following the CBCT scan to better dene the internal contents of this lesion. Pre- and postcontrast axial and coronal T1-weighted sequences, as well as coronal, axial, and sagittal T2-weighted sequences, showed a 9-cm, expansile, spheroidal, multiloculated mass, arising from the left mandibular angle, with bony destruction and displacement of supercial soft tissues. The lesion consisted of mixed signal-intensity cystic compartments separated by low T1-signal septae, with a peripheral hypointense rim corresponding to the thin bony shell. There were uiduid levels within the cystic compartments (Figure 3). Postgadolinium T1 images demonstrated contrast enhancement of septae (Figure 4). The differential was more directed toward aneurysmal bone cyst with remote possibilities of giant cell tumor (osteoclastoma) and telangiectatic osteosarcoma. Left internal carotid angiography was performed 5 weeks following MRI scan to identify principal feeder vessels and enable a more precise surgical treatment plan. The angiogram demonstrated no evidence of tumor enhancement or other abnormality. The inferior alveolar (mandibular) branch of the left internal maxillary artery was the predominant tumor feeder vessel, and therefore it was embolized so as to minimize blood loss during surgery (Figure 5). The patient was operated on the day following embolization; an immediate preoperative needle aspiration revealed fresh blood. The le-

Fig. 2. A, Axial CBCT image shows expansile low-density lesion of posterior left mandible. Note the scalloping pattern of the uninterrupted smooth cortex. B, Volume-rendered CBCT scan of skull in anterolateral projection demonstrates 3D nature of the lesion. sion was surgically approached via a submandibular incision with retromandibular extension, and was excised by enucleation with preservation of the inferior alveolar nerve. A reconstruction plate was xed across the surgical defect to simulate the contour of inferior border of mandible and reduce the risk of fracture. The 2-week postoperative period was uneventful. Microscopic analysis revealed numerous large, bloodlled, cavernous cystlike spaces throughout; foci of multinucleated giant cells and intravascular emboli; and osteoid and

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Fig. 3. Axial (A) and sagittal (B) T2-weighted MR images demonstrate hyperintense multicystic appearance with numerous uid-uid levels (arrowheads) in the ABC.

Fig. 4. Coronal pre- (A) and postgadolinium (B) T1-weighted MR images of the ABC. Note the contrast enhancement of intercystic septae.

woven bone trabeculae among stromal elements (Figure 6). The pathology report conrmed the diagnosis of aneurysmal bone cyst.

DISCUSSION Bernier and Bhaskar rst reported an ABC involving the craniofacial skeleton in 19586; to date, our search revealed a total of 121 cases of craniomaxillofacial ABC in the English literature.4,7-9 The pathogenesis of ABC is controversial, and theories include trauma, reactive vascular malformation, secondary phenomena, and genetic predisposition.10 The development of ABC following bone fracture or trauma could give credence to the traumatic etiology of this lesion; however, there are arguments against this proposal, particularly the

fact that often it is difcult to establish a history of trauma. Kransdort et al. suggested that ABC may result from a vascular disturbance in the form of sudden venous occlusion or the development of an arteriovenous shunt.2 This would usually occur, if ever it does, in more vascular, immature parts of the skeleton. ABC can occur as a secondary phenomenon arising in a preexisting bone lesion, such as solitary bone cyst, osteoclastoma, osteosarcoma, ossifying broma, brous dysplasia, osteoblastoma, hemangioendothelioma, and hemangioma.11,12 The radiographic features of ABC are not characteristic, and, therefore, radiological differentiation from other expansile jaw lesions may be difcult.

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Fig. 5. Left common carotid angiogram in lateral projection. Note the inferior alveolar artery feeding the lesion (arrow).

Fig. 6. Photomicrograph showing blood-lled spaces in brous stoma containing abundant multinucleated giant cells and osteoid. Arrow indicates a blood vessel showing PVA embolization (hematoxylin-eosin stain, magnication 20). PVA, polyvinyl alcohol.

The plain radiographic features of ABC of the jaw may show a unilocular or multilocular radiolucency causing expansion, perforation, and/or destruction of the bony cortices.2,13 These features are not diagnostically specic. CT is superior to plain radiography in delineating the thin cortex of new subperiosteal bone surrounding the lesion.1 Fluid-uid levels may occur whenever substances of differing densities are contained within a cystic compartment. The levels are depicted when imaging is performed in a gravity-dependent plane as layers of different uid densities. Layering is seen well on CT and MRI because of the

high contrast resolution. Of the 2 modalities, MRI should be more sensitive than CT in detection of uiduid levels, as it has the advantage in both tissue contrast and hemorrhage sensitivity. In MRI, the byproducts resulting from hemoglobin degradation have differing signal characteristics.14 The iron atoms of hemoglobin have a different magnetic effect, depending on the physical and oxidative state of the hemoglobin itself.15 Using a 1.5-Tesla MR unit, hyperacute (fresh) oxygenated blood (hemorrhage no more than a few hours old) has approximately the same MR imaging characteristics as water, being hypointense on T1weighted and hyperintense on T2-weighted images. Acute and subacute hemorrhages (1-7 days old), because of the paramagnetic effect of deoxyhemoglobin, have a low signal on T1- and T2-weighted images. Chronic hemorrhage (older than 7 days), with progressive oxidation of deoxyhemoglobin to methemoglobin, becomes hyperintense on T1-weighted images. The signal on T2-weighted MRI remains low if the methemoglobin is still intracellular (i.e., if oxidation occurred before erythrocyte lysis), therefore having limited motion; however, T2 signal is high when the methemoglobin has become extracellular.15 In summary, the sedimentation effect of stagnant blood is responsible for the appearance of uid-uid levels on CT and MR imaging, with the top uid layer consisting of blood serum and the bottom uid layer consisting of precipitated blood cells. The top layer is hypoattenuating on CT scans, isointense to muscle on T1-weighted MR images, and markedly hyperintense on T2-weighted MR images. The bottom layer is hyperattenuating on CT scans, slightly hyperintense to muscle on T1weighted MR images, and slightly hyperintense on T2-weighted MR images16 (Figure 3). Fluid-uid levels have been reported as being strongly suggestive of ABC on MRI or CT and have suggested the diagnosis of this lesion.17,18 Fluid-uid levels also have been observed in other lesions, however, such as telangiectatic osteosarcoma, osteoclastoma, and chondroblastoma.17,19 CT appearance of uid-uid levels in bone has also been reported in osteomyelitis and simple bone cyst.20,21 Therefore, uid-uid levels are not specic to ABC and should not be considered diagnostic of any particular lesion. Angiograms have been reported to characteristically show arterial branches to the lesion.22 ABC is divided into 3 forms based on microscopic appearances: (1) the vascular classic form shows brous connective stroma with immature broblasts and abundant multinucleated giant cells between numerous blood-lled sinuses that may show signs of thrombosis; (2) the solid variant is characterized by either inconspicuous vascularity or small vascular spaces lined by cellular brous tissue with osteoid

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aneurysmal bone cyst of the mandible. J Craniofac Surg 2011;22(2):745-8. Sun ZJ, Zhao YF, Yang RL, Zwahlen RA. Aneurysmal bone cysts of the jaws: analysis of 17 cases. J Oral Maxillofac Surg 2010;68:2122-8. Martinez V, Sissons HA. Aneurysmal bone cyst. A review of 123 cases including primary lesions and those secondary to other bone pathology. Cancer 1988;61:2291-304. Raubenheimer EJ, van Heerden WF, Noffke CE. Infrequent clinicopathological ndings in 108 ameloblastomas. J Oral Pathol Med 1995;24:227-32. Revel MP, Vanel D, Sigal R, Luboinski B, Michel G, Legrand I, Masselot J. Aneurysmal bone cysts of the jaws: CT and MR ndings. J Comput Assist Tomogr 1992;16:84-6. Gomori JM, Grossman RI. Mechanisms responsible for the MR appearance and evolution of intracranial hemorrhage. RadioGraphics 1988;8:427-40. Polito E, Leccisotti A. Diagnosis and treatment of orbital hemorrhagic lesions. Ann Ophthalmol 1994;26:85-93. Ghai S, Dill-Macky M, Wilson S, Haider M. Fluid-uid levels in cavernous hemangiomas of the liver: bafed? AJR Am J Roentgenol 2005;184:S82-5. Tsai JC, Dalinka MK, Fallon MD, Zlatkin MB, Kressel HY. Fluid-uid level: a nonspecic nding in tumors of bone and soft tissue. Radiology 1990;175:779-82. Asaumi J, Konouchi H, Hisatomi M, Matsuzaki H, Shigehara H, Honda Y, Kishi K. MR features of aneurysmal bone cyst of the mandible and characteristics distinguishing it from other lesions. Eur J Radiol 2003;45:108-12. Matsuura S, Tahara T, Ro T, Masumi T, Kasuya H, Yokota T. Aneurysmal bone cyst of the coronoid process of the mandible. Dentomaxillofac Radiol 1999;28:324-6. Rai M, Firooznia H, Golimbu C, McCauley DI. Hematogenous osteomyelitis with fat-uid level shown by CT. Radiology 1984;153:493-4. Hahn PF, Rosenthal D, Ehrlich MG. Case report 286. Skeletal Radiol 1984;12(3):214-7. Motamedi MH, Yazdi E. Aneurysmal bone cyst of the jaws: analysis of 11 cases. J Oral Maxillofac Surg 1994;52:471-5. Kershisnik M, Batsakis JG. Aneurysmal bone cysts of the jaws. Ann Otol Rhinol Laryngol 1994;103:164-5. Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch Orthop Trauma Surg 2007;127:105-14. Mller B, Claviez A, Moritz JD, Leuschner I, Wiltfang J. Extensive aneurysmal bone cyst of the mandible. J Craniofac Surg 2011;22:841-4.

formation and foci of calcifying bromyxoid tissue; and (3) the mixed variant demonstrates features of both the vascular and solid types, possibly representing a transitional phase of the lesion.23 Selective arterial embolization or injection of sclerosing agents may contribute considerably in the management of ABC.24 Broadly speaking, surgical curettage is recommended as the treatment of choice and hemorrhage in fact is not an operative premonition.25 Conversely, marginal or en bloc resection preferentially should be adopted in recurrent lesions10; however, curettage appears to have a somewhat higher recurrence rate (20%-38%) than does resection (11%-25%).5 Overall, despite recurrences, the long-term prognosis appears favorable. In conclusion, although uid-uid levels are strongly suggestive of ABC, they are nonspecic, as they have been associated with other bone lesions, including telangiectatic osteosarcoma, osteoclastoma, and chondroblastoma. Multimodality imaging may greatly aid in the specic treatment planning of such lesions, especially when they are of great size.
REFERENCES
1. Barnes L, Eveson JW, Reichart P, Sidransky D, editors. Aneurysmal bone cyst. World Health Organization classication of tumors: pathology and genetics of head and neck tumors. 3rd ed. Lyon, France: IARC; 2005. 2. Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol 1995;164:573-80. 3. Kalantar Motamedi MH. Aneurysmal bone cysts of the jaws: clinicopathological features, radiographic evaluation and treatment analysis of 17 cases. J Craniomaxillofac Surg 1998; 26:56-62. 4. Kaffe I, Naor H, Calderon S, Buchner A. Radiological and clinical features of aneurysmal bone cyst of the jaws. Dentomaxillofac Radiol 1999;28:167-72. 5. Barnes L. Tumor and tumor-like lesions of the soft tissue. In: Barnes L, editor. Surgical pathology of the head and neck. 2nd ed. New York: Marcel Dekker; 2000. p. 948. 6. Bernier JL, Bhaskar SN, Aneurysmal bone cysts of the mandible. Oral Surg Oral Med Oral Pathol 1958;11:1018-1028. 7. Motamedi MH, Navi F, Eshkevari PS, Jafari SM, Shams MG, Taheri M, Abbas FM, et al. Variable presentations of aneurysmal bone cysts of the jaws: 51 cases treated during a 30-year period. J Oral Maxillofac Surg 2008;66:2098-103. 8. Roychoudhury A, Rustagi A, Bhatt K, Bhutia O, Seith A. Aneurysmal bone cyst of the mandible: report of 3 cases. J Oral Maxillofac Surg 2009;67:1996-2004. 9. Hebbale M, Munde A, Maria A, Gawande P, Halli R. Giant

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Reprint requests: Galal Omami, BDS, MSc Senior Resident Oral and Maxillofacial Radiology University of Connecticut, School of Dental Medicine 263 Farmington Avenue Farmington, CT 06030 jellodent@yahoo.com