ABSTRACT
We describe the second reported case of multicentric osteosarcoma associated with Rothmund-Thomson syndrome (RTS), a rare hereditary cancer-prone genodermatosis characterized by typical cutaneous lesions and other non-dermatological pathological manifestations, particularly skeletal changes. A high incidence of malignant disorders has been found in RTS patients. This could be explained by the diminished capacity of DNA-repair demonstrated in the fibroblasts of RTS patients following exposure to oncogenic stimuli. The severe cutaneous lesions and the benign bone lesions found in RTS may be considered a predisposing factor to the particularly frequent skin carcinoma and osteogenic sarcoma encountered in these cancer-prone patients. A brief review of the literature is given, with a discussion of the association between these two rare conditions.
Subject(s)
Bone Neoplasms/complications , Osteosarcoma/complications , Rothmund-Thomson Syndrome/complications , Tibia , Bone Neoplasms/diagnostic imaging , Child , Female , Humans , Knee/diagnostic imaging , Osteosarcoma/diagnostic imaging , Osteosarcoma/etiology , Radiography , Syndrome , Tibia/diagnostic imagingSubject(s)
Ambulatory Care , Angiography , Brachial Artery , Radiographic Image Enhancement , Angiography/adverse effects , Angiography/instrumentation , Angiography/methods , Catheterization, Peripheral/instrumentation , Female , Hematoma/etiology , Humans , Male , Sensation Disorders/etiology , Thrombosis/etiology , Vascular Diseases/etiology , VasoconstrictionABSTRACT
One hundred emergency arteriographies (EA) were performed in 87 patients with lower limb trauma due to high-velocity missiles. Thirteen patients had bilateral injuries. In 79 cases, EA findings were positive and led to emergency surgery. In 76 cases an arterial injury was found and treated, a positive predictive value of 96% (76/79). In the other 3 cases, no arterial lesion was found (3 false positives). Among the 21 patients with normal findings from angiography, 10 had surgical exploration because of high clinical suspicion of vascular injury. Arterial injury was found in 2 cases (2 false negatives). In 8 patients, arteriography modified the surgical procedure. In the 11 remaining patients, clinical and echo Doppler follow-up results were normal, a negative predictive value of 90% (19/21). Sensitivity was 97%, specificity 86%, and accuracy 95%. These data show that arteriography in stable patients is a safe and accurate procedure. It permits avoidance of unnecessary surgical exploration in selected patients and helps modify the surgical procedure.
