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Year : 2001  |  Volume : 11  |  Issue : 2  |  Page : 96-97
Gorham's disease (a case report)


Gandhi Medical College/Hospital, Hyderabad/Secunderabad, Andhra Pradesh, India

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How to cite this article:
Durga K, Hayath M S, Aruna C A, Ram Mohan Rao N V. Gorham's disease (a case report). Indian J Radiol Imaging 2001;11:96-7

How to cite this URL:
Durga K, Hayath M S, Aruna C A, Ram Mohan Rao N V. Gorham's disease (a case report). Indian J Radiol Imaging [serial online] 2001 [cited 2019 Jun 19];11:96-7. Available from: http://www.ijri.org/text.asp?2001/11/2/96/28386
Sir,

Gorham's syndrome is an extremely rare osteolytic disorder causing extensive loss of bone matrix with vascular tumor formation [1]. About 135 cases have been documented in the literature [2]. Any part of the skeletal system can be affected. We present a woman who revealed massive osteolysis due to a malignant vascular tumor in the left lower forearm.

A twenty-two-years old woman complained of swelling of the left wrist for two months. The swelling gradually enlarged. History revealed nothing of significance. Physical examination revealed a fusiform tender swelling of a size 5x4x2 cm in the lower 1/3rd of the left forearm. Systemic examination and skeletal survey of other bones were normal.

Radiological examination revealed an expansile, osteolytic lesion in the lower 1/3rd of the left radius with a short, narrow zone of transition. The entire distal half of the left ulna showed osteolytic destruction with well-defined defects in the mid-diaphysis and few residual bone trabeculae in the zone of transition [Figure - 1].

FNAC of the swelling revealed high cellular yield with cells in sheets, tight clusters as well as rosette pattern. The individual cells were spindle-shaped with vesicular type of nuclei and prominent nucleoli. Large areas of hemorrhage were noticed [Figure - 2]. A diagnosis of aggressive soft tissue tumor was made and biopsy was advised. Multiple bony grey, red and white bits about 1.5 cc taken from the osteolytic areas were sent for histopathological examination. Microscopic examination revealed anastomosing vascular channels lined by plumpy cells with pleomorphic hyperchromatic nuclei. There was evidence of a hobnail pattern of cells. In some areas' tumor cells were seen compactly with attempted lumen formation against hemorrhagic background suggesting the features of an angiosarcoma [Figure - 3].

Gorham et al in 1955 described association of this lesion with a vascular abnormality [1]. The commonest sites are upper arm or shoulder girdle (26%) followed by mandible (15%) [3]. Most of the cases reveal benign proliferating vascular lesions except in one, wherein a malignant vascular tumor is documented [3].

Different forms of osteolysis are seen differentiated on the basis of clinical, radiological and genetic criteria. Our patient was type IV, which includes a unicentric nonhereditary form with angiomatosis [4]. Differential diagnosis includes skeletal angiosarcoma, essential osteolysis and hereditary osteolysis [4]. Diagnosis of Gorham's disease is based on clinical and radiological features of loss of bones with histological evidence of angiomatous tumor. The biological behavior of this disease is unpredictable. It may spontaneously arrest or it may progress relentlessly until all osseous tissue disappears.

 
   References Top

1.Gorham LW, Wright AW, Schultz HH, Maxon FC Jr: Disappearing bones: a rare form of osteolysis: report of two cases, one with autopsy findings. Am J Med 1954; 17:674-678.  Back to cited text no. 1    
2.Nathan D.Choma, Charles V.Biscotti, Thomas W.Bauer, Atul C.Mehta, Angelo A.Licata: Gorham's syndrome: A case report and review of literature. Am J Med 1987; 83: 1151-1154.  Back to cited text no. 2    
3.Prabhu SS, Colaco MP, Pradhan MR, Johari AN, Devany G: Massive osteolysis-Gorham's syndrome. Indian Pediatrics 1994; 31: 1542-44.  Back to cited text no. 3    
4.Johnson PM, McClure JG: Observations on massive osteolysis. Radiology 1958; 71: 28-42.  Back to cited text no. 4    

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Correspondence Address:
K Durga
Gandhi Medical College/Hospital, Hyderabad/Secunderabad, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


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[Figure - 1], [Figure - 2], [Figure - 3]



 

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