Paget Disease of the Maxilla

A 72-year-old woman first noticed progressive enlargement of the maxillary area of her face 18 years earlier. She denied facial trauma and significant dental caries. Her medical history consisted of breast cancer managed by a mastectomy and type 2 diabetes mellitus of 3 years’ duration. Harold S. Jacobs, MD, of Colorado Springs, Colo, noted nontender swelling of the patient’s maxillae (A and B). She also had a mastectomy scar in the area of the right breast and bilateral severe hearing loss. There was no lymphadenopathy. Neurologic and cardiopulmonary findings were otherwise normal. Laboratory findings revealed a markedly elevated alkaline phosphatase level (1135 U/L) and normal levels of 5-nucleotidase, aspartate aminotransferase, and alanine aminotransferase. A bone scan showed intense uptake in the maxillary area and calvarium (C); there was no uptake in the pelvis or long bones (D). A CT scan of the facial bones revealed complete replacement of the maxillae by mottled bone (E). The bone scan abnormalities, CT scan findings, and elevated alkaline phosphatase level are usually characteristic of Paget disease of bone. Fibrous dysplasia was considered in the differential diagnosis; however, the patient had no characteristic skin changes (caf au lait spots) and no history of precocious puberty. In addition, the patient’s symptoms started later in life. First described by Sir James Paget in 1877, this disorder is the second most common bone disease after osteoporosis. Paget disease is characterized by focal increases of the bone-remodeling process.¹ It usually affects the skull, long bones, and pelvis; involvement of the maxillae, as in this patient, is quite unusual. The cause of the disease is thought to be a slow viral infection. Complications include congestive heart failure; pathologic fractures; neurologic deficits, such as hearing loss; and, rarely, bone cancer. Paget disease is treated with bisphosphonates, usually for 6 months.² It is important to monitor patients’ alkaline phosphatase levels during the course of treatment and periodically thereafter. This patient received treatment with alendronate for 6 months. Her alkaline phosphatase level subsequently normalized. Her maxillary swelling remains unchanged.