We describe a case of pseudo-xanthoma elasticum (PE) in a child with distinctive skin lesions without other symptoms. Renal ultrasonography showed a characteristic pattern of dotted increased echogenicity in the corticomedullary junction. Pancreas and spleen had similar echographic findings. We point out the importance of ultrasonography in the diagnosis of this rare disorder.

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14532 Background: 5-Fluorouracil (5FU) and Folinic Acid (FA) with either oxaliplatin or irinotecan have become the standard chemotherapy used for metastatic colorectal cancer. However, more than 50% of our patients cannot afford it. Moreover, we lack of predictive markers to 5FU/AF therapy. The methylenetetrahydrofolate reductase (MTHFR) is a key enzyme regulating the folate pool and its substrate 5,10-methylenetetrahydrofolate is needed for modulation of 5FU. The C677T MTHFR gene polymorphism is highly prevalent in Mexican population and is linked to altered enzyme activity and increases substrate levels. Many studies have suggested a better response rate to fluoropyrimidine based therapy related with C677T polymorphism. The aim of this study was to evaluate the presence of this polymorphism and its relation with progression free and overall survival in metastatic colon cancer treated with 5FU/FA Methods: Sections of paraffin- embedded healthy colonic mucosa of 29 patients with metastatic unresectable colorectal cancer treated with 5FU/FA as first line chemotherapy between 1998 and 2004 were collected to obtain DNA and determine the polymorphism by PCR and allele specific digestion. Results: We found a highly proportion of at least one mutated allele in our patients (8 homozygous wild type CC, 15 heterozygous CT, 6 mutated homozygous TT). C677T MTHFR gene polymorphism showed statistically significant differences in median progression free survival non polymorphic CC 3.23±0.68 month versus 4.8±0.18 month in polymorphic group CT and TT (p= 0.011 log rank test) and median overall survival in non polymorphic groups 6.7±2.63month versus 13 ±2.51 month in polymorphic group (p= 0.03 Breslow test).No other variable affected progression free or overall survival on univariate analysis. Conclusions: Our findings suggest that C677T polymorphism could play a role on survival in metastatic colorectal cancer treated with 5FU/FA. Futher studies evaluating standard chemotherapy and the analyzed polymorphism are recommended. No significant financial relationships to disclose.

e21525 Background: Synovial sarcoma (SS) is a relatively rare type of soft tissue sarcoma. Several prognostic factors have been described that modulate patients’ outcome. The aim of this study was to identify prognostic factors in patients with SS. Methods: A retrospective analysis was performed on clinical and histopathological data of patients treated at National Oncology Institute (Panama) between 1995 - 2007. Demographic, clinical, pathological, and therapeutic variables were reviewed. Overall survival and recurrence-free survival were analyzed using Kaplan-Meier and log-rank tests. Results: Twenty seven patients were analyzed, 56% male and 44% female, median age was 28. Tumour site; extremities based 85%, truncal 15%. Median tumour size was 8cm. Presentation; non-metastatic 84%, metastatic 16%. Histological type; monophasic 78%, biphasic 22%. Mitosis/10HPF; twenty and more 33%, less than twenty 67%. Necrosis; absent 63%, less than half 30%, and more than half 7%. Histological grade (FNCLLC); grade 2 48%, grade 3 52%. Surgery; limb sparing surgery 52%, amputation 48%. Treatment: adjuvant treatment 52% (3 chemotherapy only, 7 radiation therapy, 4 both). 90% were doxorubicin based regimens. The median follow up was 25 months. 63% of tumours recurred and 33% of patients died. Univariate analysis revealed a significant positive relationship between histological grade and overall survival (p<0.05). In patients with localized disease, time to recurrence decreased with histological grade (p=0.05 ns). Conclusions: High histological grade is an adverse prognostic factor for overall survival in patients with SS. No significant financial relationships to disclose.