Progression of Atypical Fibroxanthoma to Metastatic Pleomorphic Dermal Sarcoma in a Lung Transplant Recipient

Main Article Content

Mesa Bouni B.S.
Ricardo Guerra MD
Matthew Viveiros B.S.
Grace P. Hobayan B.S.
David R. Carr MD MPH
Kathryn Shahwan MD


AFX, PDS, organ transplant, atypical fibroxanthoma, pleomorphic dermal sarcoma


Introduction: Atypical fibroxanthoma (AFX) and pleomorphic dermal sarcoma (PDS) are rare cutaneous malignancies often seen in elderly patients with photodamaged skin. Although solid organ transplant recipients (SOTRs) with AFX/PDS appear to have worse outcomes than the general population, the risk of progression from AFX to PDS in this group is not well studied.  We present the case of a lung transplant patient with AFX recurring as PDS.

Case Presentation: A 68-year-old male lung transplant patient with an extensive history of skin cancer presented with a 9-millimeter erythematous papule on the right vertex scalp. Biopsy revealed AFX, and the tumor cleared with 1 stage of MMS. Seven months later, the patient developed a rapidly growing, hemorrhagic nodule at the site, which was diagnosed as PDS. Despite initial treatment with doxorubicin, pazopanib, and radiation to the lung, liver, and bone lesions, the patient’s disease progressed. The patient was started on pembrolizumab with prednisone to mitigate the risk of organ rejection but succumbed to pneumonia with septic shock and respiratory failure.

Discussion: Transplant patients have an increased risk of poor outcomes related to PDS. ICIs may be considered in advanced cases in which other treatment options have been exhausted. In conclusion, SOTRs with AFX/PDS should be aggressively treated and monitored as their risk of unfavorable outcomes appears to be increased.  



1. Soleymani T, Aasi SZ, Novoa R, et al. Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma: Updates on Classification and Management. Dermatol Clin 2019;37(3):253-259.

2. Ørholt M, Aaberg FL, Abebe K, et al. Risk factors for local atypical fibroxanthoma recurrence and progression to pleomorphic dermal sarcoma: A meta-analysis of individualized participant data. J Surg Oncol 2022;126(3):555-562.

3. McCoppin HH, Christiansen D, Stasko T, et al. Clinical spectrum of atypical fibroxanthoma and undifferentiated pleomorphic sarcoma in solid organ transplant recipients: a collective experience. Dermatol Surg 2012;38(2):230-239.

4. Klein S, Persa OD, Mauch C, et al. First report on two cases of pleomorphic dermal sarcoma successfully treated with immune checkpoint inhibitors. Oncoimmunology 2019;8(12):e1665977.

5. Klein S, Quaas A, Noh KW, et al. Integrative Analysis of Pleomorphic Dermal Sarcomas Reveals Fibroblastic Differentiation and Susceptibility to Immunotherapy. Clin Cancer Res 2020;26(21):5638-5645.

6. Portuguese AJ, Tykodi SS, Blosser CD, et al. Immune Checkpoint Inhibitor Use in Solid Organ Transplant Recipients: A Systematic Review. J Natl Compr Canc Netw 2022;20(4):406-416.e11.

7. Tsung I, Worden FP, Fontana RJ. A Pilot Study of Checkpoint Inhibitors in Solid Organ Transplant Recipients with Metastatic Cutaneous Squamous Cell Carcinoma. Oncologist 2021;26(2):133-138.

Similar Articles

You may also start an advanced similarity search for this article.