Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 25, 2017
Central Giant Cell Granuloma of the Mandible
- Background:
- Giant cell granuloma (GCG) is an uncommon, benign, proliferative, intraosseous lesion representing < 7% of all benign jaw lesions.
- The etiology is unknown, but is thought to be a reactive process, possibly secondary to trauma or inflammation; however, some believe it is a benign neoplasm.
- There is a central form, which will be discussed below, and a peripheral form, which refers to disease involving the gingival soft tissue.
- The central form involves the bone, most often the mandible (anterior to the first molar) or the maxilla.
- Definitive diagnosis is made histologically.
- Clinical Presentation:
- Central GCG often presents in the second and third decades of life, with a predilection for females (~2:1).
- The disease often follows a slow course, presenting with a small, asymptomatic lesion.
- There is a rare, aggressive type that produces a large, fast-growing lesion, despite the benign histologic appearance. The aggressive type often presents with pain and rapid growth and has a high recurrence rate.
- Key Diagnostic Features:
- There are no pathognomonic, clinical, or radiologic features (histologic diagnosis is required for definitive diagnosis); however, there are several typical imaging features that may allow inclusion in the differential.
- Central GCGs are typically described as expansile, multiloculated lesions with scattered internal mineralization, most frequently involving the mandible and maxilla.
- Avid homogeneous enhancement is usually seen, with extension into surrounding soft tissues.
- When involving the mandible, they frequently cross midline.
- Differential Diagnoses:
- Ewing sarcoma: in the differential for an aggressive mandibular lesion in a young patient; aggressive-type periosteal reaction is more commonly seen.
- Ameloblastoma: usually presents in older patients and typically more posteriorly at the mandibular angle; septa are usually more well-defined.
- Odontogenic myxoma: older patient population; septa are characteristically straight and sharp.
- Aneurysmal bone cyst: pathologic differentiation is often necessary, particularly when fluid-fluid levels are seen; cystic component tends to be larger than solid component; posterior mandible is more commonly involved.
- Brown tumor of hyperparathyroidism: imaging features can be identical; older age or history of primary or secondary hyperparathyroidism is typical.
- Telangiectatic osteosarcoma: in the differential for aggressive mandibular lesion with fluid-fluid levels
- Cherubism (fibrous dysplasia): may be difficult to differentiate by imaging; contains characteristic ground glass matrix and tends to involve posterior mandible
- Treatment:
- There have been reports of central GCGs resolving spontaneously; however, treatment is often required.
- The typical small, slow-growing lesions can be treated with nonsurgical methods, including radiotherapy, daily calcitonin injections, intralesional corticosteroid injections, and interferon alpha.
- The more aggressive lesions typically require surgical treatment ranging from curettage to en bloc resection, but even with surgical treatment, recurrence is as high as 15–20% in these aggressive forms.