Sarcoma dermal pleomorphes C49.0

Male patients > 60 years

face, hairless capillitium

The clinical aspect with a symptomless, skin-coloured or reddish, firm lump or plaque is similar to that of atypical fibroxanthoma, only the growth is slower.

The diagnosis is made on the basis of the clinical picture and histology. Immunohistochemistry is important for differentiation from other differential diagnoses.

Mostly poorly defined nodule affecting the cutis and subcutis (also deeper tissue structures) consisting of atypical, spindle-shaped or pleomorphic cells with vesicular or bizarre hyperchromatic nuclei. Bizarrely configured multinucleated giant cells and atypical mitoses are frequently found. The cells are CD34 negative (DD: Dermatofibrosarcoma protuberans) and constantly vimentin positive. The spindle cells are often positive for alpha smooth muscle actin, the macrophage-like cells for CD68. Occasionally a focal keratin expression is observed (so-called keratin-positive AFX). Numerous vessel incisions. Often focal haemorrhages, tumor necrosis and/or perineural infiltration.

Surgical excision with an adequate microscopically controlled safety distance of 2 cm. There is a great tendency for local recurrence.

In metastatic or unresectable PDS, it is important to establish an individualized treatment plan. Immune checkpoint inhibitors are an option in off label use.

10% chance of metastasis.

Follow-up in the form of clinical examinations is recommended at quarterly intervals for the first two years and lymphnote examinations every six months. In the third to fifth year, MSE and sonography are recommended every six months.

1. Ardakani NM et al (2015) Pleomorphic dermal sarcoma withosteosarcoma-like and chondrosarcoma-like elements. Pathology 48:86-89 Griewank KG et al (2014) TERT promoter mutations are frequent in atypical fibroxanthomas and pleomorphic dermal sarcomas. Mod Catholic 27:502-508.
2. Mentzel T (2015) Fibrohistiocytic tumors of the skin: a heterogeneous group of superficially located mesenchymal neoplasms. Pathologist 36:79-88.
3. Miller K et al (2012) Pleomorphic dermal sarcoma: adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. On J Surg Pathologist 36:1317-1326.
4. Tardío JC et al (2015) Pleomorphic dermal sarcoma: a more aggressive neoplasm than previously estimated. J Cutan Pathol 43:101-112.