Hungarian Radiology

[Melorheostosis Uncommon appearances]

KUTKOWSKA-KAZMIERCZAK Anna, EWA Obersztyn, KAZIMIERZ Kozlowski

DECEMBER 20, 2004

Hungarian Radiology - 2004;78(06)

[INTRODUCTION - Melorheostosis is a rare but well publicised disease. About 320 cases were reported up to 1994. The incidence is estimated 0.9 cases per million. CASE REPORT - We report a 40 year-old man diagnosed with severe melorheostosis and unusual radiographic appearances. No dripping candle wax cortical thickening was present. All the long bones of the upper extremities showed extensive periosteal and endosteal hyperostosis with extension of the changes into the hands and shoulder girdle. Osteosclerosis was present at both sides of the right sacro-iliac joint. There was also hypoplasia of the scapulae, distal ulnae, carpal bones and the left thumb. Distinctive features of the clinical history were that the disease was of prenatal origin. The lower extremities were normal and in spite of extensive bone and soft tissue changes in the upper extremities this patient never experienced pain - a prominent feature of adult melorheostosis. CONCLUSION - Melorheostosis is a rare disorder, diagnosis of which can be made from clinical presentation and radiographs. In case of atypical melorheostosis, the lower extremities may be found to be normal on radiographs. The presentation may be painless.]

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[INTRODUCTION - Metastatic tumor of the colon is extremely rare. It could mimic inflammatory bowel disease or ischemic colitis. CASE REPORT - A 59-year-old female underwent gastrectomy because of primary signet-ring cell carcinoma of the stomach in 1999. In November 2003 follow-up CT detected circular and segmental thickening of the wall of the transverse colon with significant targetlike contrast enchancement of the inner layers. Colonoscopy was performed but due to the significant stenosis of the transverse colon, the tumor was not properly examined. Biopsy was taken from the beginning of the stenosis, which did not prove neither specific inflammation or malignancy. Barium enema and virtual colonoscopy was performed. There was mild dilatation of the stenotic part and mucosa seemed to be intact by virtual colonoscopy. Barium enema showed significant stenosis and polypoid alteration of the mucosa in the right part of the colon. There was also an asymmetric bowel wall thickening of the flexura lienalis. Because of subileus the patient was operated on, and an extended right hemicolectomy was made. The histology of the specimen verified secondary signet-ring cell tumor of the colon. CONCLUSION - In case of multiple circular and segmental thickening of the wall of the colon, with striking thickening of the enchancing inner layer with decreased contrast enhancement in the outer layer (target pattern) on the CT, the metastasis of the large bowel should be considered.]

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