Cutaneous soft tissue tumors

2026-05-04   Paola Dal Cin, PhD 

1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)

Classification

Definition

The diagnosis of soft tissue tumors often arises in the dermis or subcutis is frequently challenging mainly for the superficial, inadequate samplings in many skin biopsies, and closely mimic cases such as melanoma and carcinoma. Therefore, a combination of traditional histopathology, as well as immunohistochemical and molecular genetic studies are recommended. 1-7

 Cutaneous mesenchymal tumors that rarely occur will not be covered specifically in this section, but more comprehensive genetic details will be available in the WHO 2020 Soft Tissue and Bone Tumors. Here, we will focus on unique histological or molecular features that are unique to, or have a predilection for, the skin. The most common benign mesenchymal tumors are superficial and < 50 mm deeper with lipomas, dermatofibromas (fibrous histiocytomas), vascular or smooth muscle lesions, and nerve sheath tumors have the most frequent. Cutaneous sarcomas represent a relatively rare subset of tumors, far outnumbered by carcinomas, except for dermatofibrosarcoma protuberans, which has its peak incidence in middle-aged adults.

Recently, emerging tumor types can occur in superficial locations and harbor gene fusions e.g. nested glomoid neoplasm with GLI1 alterations; clear cell tumor with melanocytic differentiation and ACTIN::MITF, CRTC1::TRIM11 and MITF::CREM, EWSR1::SMAD3 -rearranged fibroblastic tumor, PLAG1-rearranged fibroblastic tumor, superficial ALK-rearranged myxoid spindle cell neoplasm and NTRK-rearranged spindle cell neoplasms. 4,6,7


Article Bibliography

Reference NumberPubmed IDLast YearTitleAuthors
1243783872014Cutaneous mesenchymal tumours: an update.Mentzel T et al
2340231002021Update on Cutaneous Soft Tissue Tumors.Dermawan JK et al
3349584992022Recent advances in the diagnosis, classification and molecular pathogenesis of cutaneous mesenchymal neoplasms.Papke DJ Jr et al
4371567072023Gene fusions in superficial mesenchymal neoplasms: Emerging entities and useful diagnostic adjuncts.Fischer GM et al
5386978272024Mesenchymal skin tumors - Novel entities in the 5th edition of WHO classification of skin tumors.Michal M et al
6392644722024Update on cutaneous mesenchymal tumors in the 5th edition of WHO classification of skin tumors with an emphasis on new fusion-associated neoplasms.Kalmykova AV et al
7403871102025MITF Pathway-Activated Cutaneous Neoplasms.Michal M et al

Citation

Paola Dal Cin, PhD

Cutaneous soft tissue tumors

Atlas Genet Cytogenet Oncol Haematol. 2026-05-04

Online version: http://atlasgeneticsoncology.org/solid-tumor/209369