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Lipomas are among the most common specimens encountered in surgical pathology. Beyond distinguishing a lipoma from an atypical lipomatous tumor or well-differentiated liposarcoma (ALT/WDLS), there are important clinical considerations to keep in mind, as lipomas and lipomatosis may occur in association with various syndromes. These syndromes can be inherited or sporadic, and some are linked to an increased risk of other malignancies. Moreover, several are associated with systemic findings that carry significant morbidity and mortality. Careful attention to specific clinicopathologic features-together with molecular testing when indicated-can facilitate early recognition of these syndromes, which is essential for appropriate counseling and long-term surveillance. Despite numerous case reports and isolated studies, a comprehensive synthesis of these syndromes remains lacking. In this review, we aim to summarize the Syndromes Associated with Lipomatous Tumors (SALT) and highlight key clinicopathologic features relevant to diagnosis and management. We also hope that this overview will stimulate further research by providing a succinct framework for exploring potential biologic networks underlying the origin and development of these common neoplasms.