Abstract
After Evans and colleagues identified the lipomatous tumor with a well-differentiated liposarcoma in a subcutaneous location or within a muscle layer, namely, atypical lipomatous tumor (ALT), this malignancy has been investigated to clarify the characteristics of clinical behavior and genomic changes. As one of the important issues for clinicians, it is a hot topic of how to distinguish ALT from benign lipoma in the clinical aspect. Recent studies revealed novel findings to clarify the risk factor for the diagnosis of ALT and molecular targets for the treatment of ALT. Clinical characteristics of superficial-type ALT well reflect the subcutaneous location of the tumor and are slightly different compared to deep-type ALT, such as tumor size. In addition, there has been a recent discovery of novel findings in ALT-related genes, namely, HMG2A (high mobility group protein 2a), YEATS4 (YEATS domain containing 4), and CPM (Carboxypeptidase M). Recent updates on treatment for advanced ALT are well developed including immunotherapy and conducting clinical trials. Finally, this review introduces one of the hot topics of ALT research focused on epigenetic changes: their attention in recent updates on clinical characteristics and the novel discovery of related genes, treatment, and epigenetic modifications in atypical lipomatous tumors.
Highlights
Skin is located mostly on the outside of the human body and consists of three major layers: the epidermis, dermis, and subcutaneous layers
Both CDK4 and JUN upregulations were observed in dedifferentiated liposarcoma, whereas amplification of HMGA2 was associated with the non-dedifferentiated form of atypical lipomatous tumor (ALT)
Trabectedin is applicable to advanced liposarcoma, showing a progression-free survival rate compared with dacarbazine treatment [67]
Summary
Skin is located mostly on the outside of the human body and consists of three major layers: the epidermis, dermis, and subcutaneous layers. Recent studies identified that the fat layer plays an additive function such as antimicrobial actions [3] These findings provide us with a more detailed, precise, important role of the subcutaneous layer in the human body. Evans and colleagues identified the lipomatous tumor as a well-differentiated liposarcoma in a subcutaneous layer or within a muscle layer [5] with higher frequencies of the local recurrence rate and distant organ metastasis. They named this tumor with unique clinical characteristics as ALT. We focused on the recent hot topics of clinical characteristics of ALT, in addition to the recent discovery of ALT-related genes, treatment, and epigenetic modification in ALT
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