= MASSIVE OSTEOLYSIS = GORHAM-STOUT DISEASE= VANISHING BONE DISEASE = PHANTOM BONE DISEASE

[Lemuel Whittington Gorham (1885–1968), internist, New York]

[Arthur Purdy Stout (1885–1967), surgeon and pathologist at College of Physicians & Surgeons of Columbia University, New York]

= infrequent disorder of bone + soft tissues characterized by aggressive nonneoplastic proliferation of lymphangiomatous tissue → massive local osteolysis

Cause: persistence of dilated lymphatics from 14^th–20^th week EGA

Prevalence:>200 cases confirmed

Histo: proliferation of dilated lymph channels communicating with blood vessels in bone

Age: infant + child + adolescent, M=F

◊Consider Gorham-Stout disease for any osteolytic lesion of unknown cause!

Associated with: cystic hygroma (neck, axilla, chest wall, mediastinum), splenic cysts, chylothorax, soft-tissue hemangiomas without calcifications

• frequently history of severe trauma (50%)
• little / no pain in affected body part

Location: any bone (frequently multiple locations); most commonly major long bones (humerus, shoulder, mandible), pelvis, spine, thorax, short tubular bones of hand + feet (unusual)

• progressive relentless osteolytic resorption / destruction of bone with monocentric spread:
  • patchy osteopenia (early)
  • expanding intramedullary lytic lesion (later)
  • complete resorption of entire bone segments (late) of one bone / group of bones
  • NO regard for joint boundaries
• lack of reaction (NO periosteal reaction, minimal sclerosis)
• advancing edge of destruction not sharply delineated
• tapering margins of bone ends at sites of osteolysis with conelike spicule of bone (early changes)
• NO soft-tissue calcifications / phleboliths
• serous / chylous pleural effusions → significant mortality

MR:

• varying SI depending on disease activity
• hypointense soft tissue around osteolytic area on T1WI
• hyperintense soft tissue around osteolytic area on T2WI
• minimal contrast enhancement

Rx: no known validated treatment; interferon; steroids

DDx: Langerhans cell histiocytosis, fibrous dysplasia, brown tumor of hyperparathyroidism, infection, trauma, tumor, gout, scleroderma