Q:

Biologic features of adult soft tissue sarcomas include the following:

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Biologic features of adult soft tissue sarcomas include the following:


  1. Mutations of p53 in metastatic liposarcoma.
  2. A low (less than 1%) risk of metastasis for small, low-grade lesions.
  3. Recurrent disease in at least 33% of patients.
  4. Lymph node metastasis in less than 3% of patients.
  5. Mutations of p53 in Li-Fraumeni syndrome.

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B. A low (less than 1%) risk of metastasis for small, low-grade lesions.

C. Recurrent disease in at least 33% of patients.

D. Lymph node metastasis in less than 3% of patients.

E. Mutations of p53 in Li-Fraumeni syndrome.

DISCUSSION: Inactivation of the p53 tumor suppressor gene is involved in tumorigenesis of several sarcomas, but not specifically liposarcoma. The relevance of this gene is underscored by the frequent occurrence of soft tissue sarcomas in the Li-Fraumeni syndrome, where all families studied have p53 germ line mutations. Low-grade lesions have a low (less than 1%) risk of subsequent metastasis, whereas high-grade lesions have a high (greater than 50%) risk of subsequent metastasis. Despite optimal multimodality treatment, at least 33% of patients develop recurrent disease (median disease-free interval 18 months). Lymph node metastasis occur in fewer than 3% of adult soft tissue sarcomas.

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