Clinical and radiological features of central giant-cell lesions of the jaw

Department of Oral and Maxillofacial Surgery, Academic Medical Center and Academic Center for Dentistry (ACTA), University of Amsterdam, Meibergdreef 9, 1100 DD Amsterdam, The Netherlands.
Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology (Impact Factor: 1.46). 05/2005; 99(4):464-70. DOI: 10.1016/j.tripleo.2004.11.015
Source: PubMed


The purpose of this study was to evaluate the clinical and radiological features of central giant-cell lesions that were diagnosed in The Netherlands between January 1, 1990, and January 1, 1995.
A population-based retrospective study was carried out, examining all patients with a central giant-cell lesion from this period.
In 83 patients there was a central giant-cell granuloma (89 lesions). Aggressive signs and symptoms (pain, paresthesia, or root resorption) were found in 16 (19.3%) patients. Multiple lesions occurred in 3 (3.6%) patients. The overall recurrence rate was 26.3%, and there was a higher recurrence rate in patients who exhibited aggressive signs and symptoms than in patients without these features (RR 1.6). In 5 patients a clinical diagnosis of cherubism or concomitant neurofibromatosis type 1 was made (14 lesions).
In a general population, large and aggressive lesions are less common than suggested by the literature. Multiple lesions, however, occur more frequently than previously assumed. In patients with aggressive signs and symptoms, surgical curettage is not an effective therapy.

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    • "Histologically, the lesions consist of cellular fibrous tissue containing multiple foci of hemorrhage, clusters of multinucleated giant cells, and occasionally trabeculae of woven bone [45]. Despite the histologic similarities, cherubism can be distinguished from this disease radiologically, because the majority of lesions in central giant-cell granuloma are unilocular, whereas cherubism is usually a multilocular process [46]. It should be noted that cherubism and central giant-cell granuloma are considered histologically indistinguishable, and “should no longer be linked with fibrous dysplasia.” "
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    ABSTRACT: Cherubism is a benign fibro-osseous disease of childhood limited specifically to the maxilla and mandible. The progressive replacement of the jaw bones with expansile multilocular cystic lesions causes eventual prominence of the lower face, and hence the classic "cherubic" phenotype reflecting variable extents of jaw hypertrophy. Histologically, this condition has been characterized as replacement of the normal bone matrix with multicystic pockets of fibrous stroma and osteoclastic giant cells. Because of radiographic features common to both, primarily the presence of multiloculated lucencies with heterogeneous "ground-glass" sclerosis on CT imaging, cherubism was long mistaken for a craniofacial subtype of fibrous dysplasia. In 1999, however, the distinct genetic basis for cherubism was mapped to chromosome 4p16.3 and the SH-3 binding protein SH3BP2. But while there are already three suspected cases of fibrous dysplasia amongst archaeological populations, no definitive cases of cherubism have yet been reported in historical populations. In the current study we describe micro- and macro-structural changes in the face of a 17th century Joseon Dynasty Korean mummy which may coincide with the clinic-pathologic and radiologic features of cherubism.
    PLoS ONE 08/2014; 9(8):e102441. DOI:10.1371/journal.pone.0102441 · 3.23 Impact Factor
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    • "The true prevalence of BT is actually higher than reported in the literature. The likely explanation is that in most patients, the jaw lesions are never diagnosed and will spontaneously disappear when the PTH and calcium levels are corrected [6] [7]. "
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    ABSTRACT: Brown tumour [BT] is an uncommon, non neoplastic complication of Hyperparathyroidism [HPT]. Skeletal changes are the main consequences of this endocrine condition. HPT manifest in three categories. Primary HPT is due to hyperfunction of one or more parathyroid gland, causing an increase of parathyroid hormone secretion resulting in hypercalcemia. Secondary HPT occurs due to chronic renal failure, decreased vitamin D production or with hypocalcemia. Tertiary HPT occurs when the parathyroid activity turns autonomous and excessive, leading to hypercalcemia. BT occurs frequently in the mandible than the maxilla. They are more common in women aged over 50y and majority of BT are asymptomatic. Radiographically, it appears as well defined radioluceny. The gross specimen usually shows a brown or reddish-brown colour. We report a relatively rare combination of maxillary posterior BT as a clinical manifestation of secondary HPT due to vitamin D deficiency with polydactyly, syndactyly and cardiac anomalies.
    08/2014; 8(8):ZD31-3. DOI:10.7860/JCDR/2014/9647.4727
    • "The clinical and histological features of cherubism may sometimes present problems in diagnostic distinction from giant cell granuloma, giant cell tumor, ossifying fibroma, fibrous dysplasia of the jaw and Paget's disease of bone. Giant cell granuloma, in particular, can only be distinguished by radiological findings because most lesions are unilocular, whereas in cherubism the lesions are multilocular.[15] If the mandible alone is involved and there is no family history, it is impossible to differentiate on clinical grounds between cherubism and multiple granulomas in the jaw. "
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    ABSTRACT: Cherubism is a self-limiting non-neoplastic autosomal dominant fibro-osseous disorder of the jaw usually found in children between 2-5 years of age. It occurs predominantly in boys and is characterized clinically by bilateral swelling of cheeks due to bony enlargement of the jaw that gives the patient a typical cherubic look. Cherubism may occur as solitary cases or in many members of family, often in multiple lesions. Radiographically the lesions appear as multilocular bilateral radiolucent areas. The present case report describes a 13-year-old female cherubic child with progressive swelling of cheeks.
    Journal of Oral and Maxillofacial Pathology 04/2014; 18(1):84-8. DOI:10.4103/0973-029X.131920
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