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Cutaneous sarcomas are a diverse group of mesenchymal tumors arising in the dermis and subcutaneous tissues. Some are unique to this location while others represent the same tumor as is found in the deeper soft tissues. In general, superficial sarcomas have a better prognosis than their deeper counterparts.

Diagnosis is often challenging as these tumors are relatively rare. Frequently, the pathology needs to be reviewed by pathologists with significant experience in diagnosing sarcoma, which often means referral to the pathology department of a bone and soft tissue unit. The use of diagnostic immunohistochemistry (IHC) is required for practically all tumor types, and due to advancements in molecular pathology, diagnostic molecular techniques are frequently used.

With the possible exception for atypical fibroxanthoma (AFX), discussion at a multidisciplinary meeting (MDM) is almost always appropriate. There is a significant body of literature on many of these tumors that outlines the use of radiotherapy or systemic therapy, so this is the perfect forum to discuss these options.

Surgery is the definitive therapy and, since successful complete removal correlates with an improved outcome, a complete margin assessment is of particular benefit. Significant spread beyond what is evident clinically is a frequent feature. Once again it is worth emphasizing that margin control surgery (MCS) is the elimination of guesswork; wide local excision (WLE) following recommended margin sizes is guessing the actual size of the tumor. Unpredictability in the tumor’s size means difficulty in both predicting surgical operating times and estimating the size of the resultant defect.

It is almost always appropriate to debulk the central tumor for paraffin assessment of the tumor parameters and confirmation of the diagnosis. This tissue can also be used for molecular analyses or research purposes.

Italian painters in the Renaissance period believed that no boundary in nature is sharply delineated and they developed the sfumato technique, which subtly blurred the transition between darker and lighter colors so that gradients could be reflected in their paintings. Somewhat similarly, aggressive sarcomas often have a histologic tumor border which melts into the surrounding normal tissue without a perceptible abrupt transition. In these cases, a precise histologic delineation of the tumor may be impossible, and a final wider paraffin safety margin may be needed.

The efficacy of MCS in treating some highly invasive sarcomas (e.g., angiosarcoma) is inconclusive in the literature. In this context, MCS may serve as a basic guide for the size and shape of the excision (see Chap. 6). This guidance is particularly helpful for tumors arising within a cosmetically or functionally sensitive anatomy.


These tumors predominantly affect elderly patients with sun-damaged skin and have a predilection for the head and neck region, particularly the scalp. Clinically, they may present as a nodule or plaque, sometimes ulcerated, and often measure more ...

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