Purpose or Case Report: Hypertrophic pulmonary osteoarthropathy (HPOA) is a syndrome characterized by excessive proliferation of skin and bone in the distal extremities. The classic imaging finding is symmetric, smooth periosteal reaction of the bones of the forearm or lower leg. The pathogenesis of this new bone formation is not well understood. Proposed mechanisms include growth factor release mediated by the tumor itself or the shunting of megakaryocytes through the pulmonary vasculature. These growth factors may contribute to vascular proliferation and bone formation. HPOA can be idiopathic but is more commonly secondary in patients with a variety of pulmonary disorders, congenital heart disease, and inflammatory bowel disease. HPOA is more commonly seen in adults than children. We present a case of HPOA in a patient with pleuropulmonary blastoma, a rare pediatric intrathoracic tumor.


A 3 year-old girl was diagnosed with pleuropulmonary blastoma after presenting to the emergency department with a twelve-day history of fever and leg pain. As part of a fever of unknown origin workup, chest radiographs were performed, which showed a large left lower lobe mass. Contrast-enhanced computed tomography characterized the mass as mixed cystic and solid. As part of the preoperative evaluation for osseous metastatic disease, a technetium-99m bone scan was performed, which revealed bilateral, symmetric radiotracer uptake within the ulnar and fibular shafts, and the distal humeri. Subsequent radiographs of these bones demonstrated bilateral, symmetric smooth periosteal reaction. Following surgical resection of the pleuropulmonary blastoma and completion of chemotherapy, a bone scan was repeated which showed resolution of the previously seen scintigraphic findings.

To date, HPOA has never been reported in a patient with pleuropulmonary blastoma. This case report highlights that HPOA, commonly thought of as an entity of adult lung disease, can also present in children.
Methods & Materials:
Results:
Conclusions: