Gorham's massive osteolysis

Case contributed by Dr Matt Skalski

Presentation

Withheld.

Patient Data

Age: 30 years
Gender: Female
X-ray

There is absence of the right clavicle, acromion process, and part of the scapular spine and scapular fossa, as well as an approximately 3cm, 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. 

CT

The CT reveals similar findings to the plain films. The osseous destruction is well delineated, and no associated soft tissue mass is observed. 

MRI

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.

X-ray

One year previously. There is less bone in these images than in the most recent study, possibly indicating response to therapy.

X-ray

Two years previously. Extensive osseous destruction is noted, as described in the preceding images. 

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's 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's disease is exceptionally rare, and the differential diagnosis of lytic distruction invoving 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 advanced 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. 

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Case information

rID: 24243
Case created: 2nd Aug 2013
Last edited: 24th Jun 2017
Inclusion in quiz mode: Included

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