Multifocal pleomorphic dermal sarcoma and the role of inflammation and immunosuppression in a lung transplant patient: A case report

Mary E. Anderson, Nemanja Rodic, Antonio Subtil, Dawn Queen, Selim Arcasoy, George W. Niedt, Peter W. Heald, Larisa J. Geskin

Research output: Contribution to journalArticlepeer-review

7 Scopus citations

Abstract

Background: Pleomorphic dermal sarcoma is the cutaneous variant of undifferentiated pleomorphic sarcoma. It is a rare malignancy of unclear histogenesis; it is a diagnosis of exclusion that requires extensive use of immunohistochemistry to rule out other malignancies. Pleomorphic dermal sarcoma typically presents as a solitary tumor in sun-exposed areas and may have unpredictable clinical behavior, with some tumors associated with metastasis and death. Case presentation: We present an unusual case of multifocal pleomorphic dermal sarcoma arising in the areas of alpha-1-antitrypsin deficiency panniculitis in a lung transplant patient. Our patient was a 58-year-old white woman whose initial presentation was consistent with alpha-1-antitrypsin deficiency panniculitis. She then developed extensive multifocal, bleeding, and ulcerated nodules in the areas of the panniculitis. A skin biopsy was consistent with a diagnosis of pleomorphic dermal sarcoma. Her immunosuppressive regimen was decreased, and she was treated with liposomal doxorubicin 40 mg/m2 every 3 weeks with some initial improvement in the size of her tumors. However, soon after beginning therapy, she developed pneumonia and septic shock and ultimately died from multi-organ failure. Conclusions: We hypothesize that chronic, multifocal inflammation in the skin in the setting of immunosuppression led to simultaneous, malignant transformation in numerous skin lesions. We discuss the challenges of diagnosing pleomorphic dermal sarcoma, therapeutic options, and stress the need for multidisciplinary management of these cases.

Original languageEnglish
Article number169
JournalJournal of Medical Case Reports
Volume13
Issue number1
DOIs
StatePublished - 30 May 2019
Externally publishedYes

Keywords

  • Alpha-1-antitrypsin deficiency
  • Immunosuppression
  • Inflammation
  • Pleomorphic dermal sarcoma
  • Transplantation

Fingerprint

Dive into the research topics of 'Multifocal pleomorphic dermal sarcoma and the role of inflammation and immunosuppression in a lung transplant patient: A case report'. Together they form a unique fingerprint.

Cite this