Melorheostosis: A Clinical, Pathologic, and Radiologic Case Series. (November 2019)

• Record Type: Journal Article
• Authors: Fick, Cameron N.; Fratzl-Zelman, Nadja; Roschger, Paul; Klaushofer, Klaus; Jha, Smita; Marini, Joan C.; Bhattacharyya, Timothy
• Is Part Of: American journal of surgical pathology. Volume 43:Number 11(2019)
• Publication Date: 2019-11
• Subjects: sclerosing bone dysplasia -- hyperostosis -- bone biopsies -- histology -- osteoidosis; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000001310
• Languages: English
• ISSNs: 0147-5185

Abstract

Abstract : Melorheostosis is a rare sclerosing bone disease characterized by excessive cortical bone deposition that is frequently on the differential diagnosis for bone biopsies. Although the radiologic pattern of "dripping candle wax" is well known, the pathologic findings have been poorly defined. Here, we comprehensively describe the histology of melorheostosis in 15 patients who underwent bone biopsies. Common histologic findings included: dense cortical bone (73.3%), woven bone (60%), and hypervascular features and increased porosity (66.7%). One third of the patients (5/15) also had prominent cement lines. Multiple patients had >1 histologic pattern (ie, dense cortical bone and hypervascularity). Overall, this study suggests that melorheostosis exists with several histologically distinct patterns. When confronted with a case of suspected melorheostosis, the clinical pathologist should use the histologic features common to melorheostotic lesions presented here in conjunction with the patient's clinical presentation and radiographic findings to arrive at a diagnosis. An illustrative case is presented.