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Li Fraumeni Syndrome (LFS) is a rare autosomal disorder characterized by a familial clustering of tumors. Analysis of several series of LFS families have shown that 70% of such families are attributable to germ-line mutations in TP53. We report the case of a patient who had a first degree family antecedent of cancer in young ages. At the age of 31 years, the patient was operated of bladder papillary superficial carcinoma; five years later, he was treated for a high grade pleomorphe sarcoma of the left thigh and treated by surgery, adjuvant chemotherapy and radiotherapy. At the age of 38 years, after abdominal pain, radiologic examination reveled pancreatic tumor with bone and lymphatic metastases. The patient died one month later from pulmonary embolism. Sequencing revealed a germiline mutation of this patient that was confirmed in a member of his family in codon 1009CT, protein Arg337Cys, exon 10 of TP53 gene this mutation was revealed in his nephew (died at the age of 20 from bone sarcoma).

Thyroid gland angiomatoid tumors are an extremely aggressive neoplasms with varied histological patterns and features of endothelial differentiation. The histogenesis of thyroid angiomatoid tumors has been controversial for many years: these tumors may be either a variant of anaplastic carcinoma, or an angiosarcoma. We report a case of thyroid angiomatoid tumor in a 68-year-old woman. We also discuss, through a review of the literature, the pathologic criteria that could be used to distinguish between angiosarcoma and anaplastic carcinoma of the thyroid.