Clinically aggressive cemento-osseous dysplasia (COD) of the mandible with associated aneurysmal-like simple bone cysts (ASBC): three cases of expansive osseous dysplasia?

Introduction

Cemento-osseous dysplasias (COD), a family of benign fibro-osseous lesions of the jawbones, commonly present as asymptomatic, mostly nonexpansile, well-demarcated, mixed radiopacities/radiolucencies (RO/RL) or unilocular RL. Certain types show predilection for middle-aged Black and Asian women. A form of nonfamilial COD exhibiting marked, rapidly progressing jawbone expansion has been reported under the term expansive osseous dysplasia (EOD). Notably, EOD is frequently associated (25%-45%) with concurrent aneurysmal-like or simple bone cysts (ASBC).

Materials and Methods

We present three individuals with mandibular COD associated with ASBC characterized by extensive bony expansion and destruction.

Results

All 3 cases were nonfamilial and affected black-African women 20-52 years of age (median age: 36 years). Radiographically, 2 cases exhibited multiple, demarcated, unilocular, or multilocular RL and/or mixed RO/RL involving the entire body of the mandible with associated expansion, cortical thinning, and perforation. The 3rd and youngest patient presented with a markedly expansile multilocular RL with cortical thinning and small RO foci. One of the individuals, who presented initially with localized COD of the anterior mandible, showed rapid lesional progression within 5 years. Histopathologically, all cases featured irregularly shaped, round to ovoid dystrophic, cementum- or bone-like calcifications associated with cellular and notably vascular fibrocollagenous or fibromyxoid connective tissue. Hemorrhagic cystic spaces lined by slender trabeculae of vital woven bone with or without multinucleated giant cells were present. The 2 individuals with multiple mandibular lesions received a diagnosis of florid COD with ASBC while the youngest patient was diagnosed with the EOD variant of COD with ASBC.

Conclusions

EOD is an uncommon form of fibro-osseous lesion of the jaws characterized by rapid progression with significant bone loss and expansion. Patient management is challenging due to its aggressive clinical behavior.