Patient Data
There is absence of the right clavicle, acromion process, and part of the scapular spine and scapular fossa, as well as an approximately 3 cm, well-defined lucent lesion in the body of the scapula in the infraspinous region. There are small linear areas of osseous density in the expected location of the scapular spine and acromion process. There is no periosteal reaction noted or associated soft tissue mass. All other visualized structures are unremarkable.
The CT reveals similar findings to the plain films. The osseous destruction is well delineated, and no associated soft tissue mass is observed.
There is heterogeneous fluid signal along the borders of the remaining bone in the area of destruction outlined in the plain film studies, but the extent of this signal is limited to those regions. Absence of the previously outlined structures is still noted, with no associated soft tissue mass or signal abnormality.
Case Discussion
Absence of the clavicle and destruction of the acromion process, scapular spine and part of the scapular body without an associated destructive soft-tissue mass are findings consistent with Gorham massive osteolysis or "vanishing bone disease." The STIR hyperintense signal adjacent to the remaining superior border of the scapula most likely represents hyperemia and lymphangiomatosis/hemangiomatosis associated with Gorham disease.
Gorham disease is exceptionally rare, and the differential diagnosis for lytic destruction involving multiple adjacent osseous structures and no associated soft tissue mass includes previous infectious destruction or previous surgical resection. The differential diagnosis for the radiographic findings by themselves without cross-sectional imaging could include: osteomyelitis, aneurysmal bone cyst, lytic metastasis, purely lytic osteosarcoma, Ewing sarcoma, lymphoma, Paget disease, as well as other less likely causes.
This case is courtesy of Eric White, MD, who generously shared it with me.