(1) Background: Rare skin cancers include epithelial, neuroendocrine, and hematopoietic
neoplasias as well as cutaneous sarcomas. Ultraviolet (UV) radiation and sunburns are important
drivers for the incidence of certain cutaneous sarcomas; however, the pathogenetic role of UV
light is less clear in rare skin cancers compared to keratinocyte cancer and melanoma. In this
study, we compared the degree of actinic elastosis (AE) as a surrogate for lifetime UV exposure
among selected rare skin cancers (atypical fibroxanthoma [AFX], pleomorphic dermal sarcoma
[PDS], dermatofibrosarcoma protuberans [DFSP], Kaposi sarcoma [KS], Merkel cell carcinoma [MCC],
and leiomyosarcoma [LMS]) while taking into account relevant clinical variables (age, sex, and
body site). (2) Methods: We newly established a semi-quantitative score for the degree of AE
ranging from 0 = none to 3 = total loss of elastic fibers (basophilic degeneration) and multiplied
it by the perilesional vertical extent (depth), measured histometrically (tumor-associated elastosis
grade (TEG)).We matched the TEG of n = 210 rare skin cancers from 210 patients with their clinical
variables. (3) Results: TEG values were correlated with age and whether tumors arose on UV-exposed
body sites. TEG values were significantly higher in AFX and PDS cases compared to all other
analyzed rare skin cancer types. As expected, TEG values were low in DFSP and KS, while MCC
cases exhibited intermediate TEG values. (4) Conclusions: High cumulative UV exposure is more
strongly associated with AFX/PDS and MCC than with other rare skin cancers. These important
results expand the available data associated with rare skin cancers while also offering insight into
the value of differentiating among these tumor types based on their relationship with sun exposure,
potentially informing preventative, diagnostic and/or therapeutic approaches.