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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 25:415-418, March 2004
© 2004 American Society of Neuroradiology

Case Report
BRAIN

Disappearing Calvarium in Gorham Disease: MR Imaging Characteristics with Pathologic Correlation

Chung-Ping Lo^a, Cheng-Yu Chen^a, Shy-Chyi Chin^a, Chun-Jung Juan^a, Chun-Jen Hsueh^a and Ann Chen^b

^a Department of Radiology, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan, Republic of China
^b Department of Pathology, Tri-Service General Hospital and National Defense Medical Center, Taipei, Taiwan, Republic of China

Address correspondence to Cheng-Yu Chen, MD, Department of Radiology, Tri-Service General Hospital and National Defense Medical Center, 325, Sec 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan, Republic of China

Summary: Gorham disease is a rare condition characterized by intraosseous neoplastic proliferation of hemangiomatous tissue with progressive, massive osteolysis. We present a pathologically proved case of Gorham disease that involved the left parietal bone in a 23-year-old man. Imaging studies including conventional radiography of the skull, CT, MR imaging, and Technetium-99 m (Tc-99 m) scintigraphy demonstrated a large skull defect without associated soft tissue mass over the left parietal skull. Contrast enhancement and increased isotope uptake along the margin of the defect were shown at gadolinium-enhanced T1-weighted MR imaging and Tc-99 m methylene diphosphate (Tc-99 m MDP) bone scintigraphy. Pathologic study revealed intraosseous angiomatosis at the periphery of the osteolytic skull lesion.