Abstract
Atypical fibroxanthomas (AFX) are rare skin tumors. These generally are superficial tumors, usually 376 colonies. The patient could not tolerate additional chemotherapy. She was therefore treated with the oral targeted agent pazopanib. The patient developed a dramatic biopsy-confirmed complete response. After 11 months of pazopanib treatment, a repeat CTC-derived culture sample grew only 8 colonies/10 ml blood. The complete response to pazopanib is still ongoing at over 41 months. To our knowledge, this is the first demonstration of clinical complete response of a PDS tumor following targeted therapy. An additional novel feature was the demonstration that CTC-derived colonies could be grown from the blood of a PDS patient. The number of colonies appeared to correlate with the clinical treatment response and seemed to function as a potential prognostic marker.
Highlights
Atypical fibroxanthomas (AFX) are uncommon spindle cell neoplasms that most often occur on the sun-exposed skin of older patients [1]
These generally are superficial tumors, usually
We present the case of a 64-year old woman who developed a deeply invasive PDS on the vertex of her scalp invading to the galea, with in-transit scalp metastases
Summary
Atypical fibroxanthomas (AFX) are uncommon spindle cell neoplasms that most often occur on the sun-exposed skin of older patients [1] These tumors are generally less than 3 cm in size, and usually exhibit rapid initial growth [1]. Distant organ metastases from a skin histio-fibroblastic primary are more consistent with a UPS diagnosis, 25 cases of “metastatic AFX” are identified in the older literature [6]. UPS is considered to be the more malignant of the two conditions in that it may invade deeper tissues, and seems to have a greater metastatic potential, while AFX and PDS are considered more benign in their clinical behavior This distinction may underestimate the potential aggressiveness of PDS tumors, which can certainly infiltrate the muscle and fascia and produce at least in-transit metastases. This is the first report of a biopsy-confirmed complete response of a metastatic DPS to pazopanib therapy
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