Li-Fraumeni syndrome (LFS) is a rare, autosomal dominant cancer syndrome characterized by early-age onset of multiple primary tumors—primarily soft tissue and bone sarcomas, breast cancer, brain tumors, and adrenocortical carcinomas—affecting individuals and multiple family members1 with germline p53 mutations occurring in 50% to 70% of families.2 Melanoma is a rare, controversial component of LFS.
Clinical and histologic features of cutaneous melanomas (all biopsy specimens stained with hematoxylin-eosin, original magnification ×20). A, Right lateral abdominal lesion following initial biopsy (top); no mitoses seen in biopsy specimen despite high-grade cytologic appearance (bottom). B, Right pubic region lesion following initial biopsy (top); dermis in biopsy specimen is diffusely infiltrated by sheets of melanoma cells (bottom). C, Right inframammary lesion (top); biopsy specimen demonstrates nodular junctional activity by atypical melanocytes (bottom). D, Left posterior calf lesion (top); specimen from left posterior calf demonstrates nodular junctional activity by atypical melanocytes showing cytologic atypia and upward migration (bottom). E, In this left skin flap melanocytic lesion, the skin flap was created as part of mandible reconstruction, and the lesion was within the field of previous radiation therapy (top); biopsy specimen demonstrates dermal invasion to a Clark level IV and greatest tumor thickness of 0.95 mm (bottom); the margins were negative for malignancy.
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