Metastatic Spiradenocarcinoma Managed With PD-1 Inhibition

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Jeffrey J. Wargo Division of Dermatology, and

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David R. Carr Division of Dermatology, and

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Jose A. Plaza Department of Pathology, The Ohio State University Wexner Medical Center; and

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Claire F. Verschraegen Division of Medical Oncology, The Ohio State University James Cancer Hospital, Columbus, Ohio.

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Spiradenomas are rare skin adnexal tumors, usually benign, appearing in early adulthood. The etiology of this tumor is still debated. The tumor suppressor gene CYLD, responsible for the Brooke-Spiegler syndrome, causes spiradenomas, trichoepitheliomas, and cylindromas. With time, spiradenomas can degenerate into aggressive spiradenocarcinomas. With only 117 malignant cases reported, treatment recommendations are based on case reports and expert opinion. There is no standard of care beyond surgical resection for localized disease and no guidelines for management of metastatic disease. With the advent of immunotherapy and PD-1 inhibition, we present the first reported case of a metastatic spiradenocarcinoma managed with pembrolizumab.

Submitted February 5, 2021; final revision received November 17, 2021; accepted for publication December 6, 2021. Published online February 23, 2022.

Disclosures: The authors have disclosed that they have not received any financial consideration from any person or organization to support the preparation, analysis, results, or discussion of this article.

Correspondence: Jeffrey J. Wargo, MD, Division of Dermatology, The Ohio State University Wexner Medical Center, 540 Officenter Place, Suite 240, Gahanna, OH 43230. Email: jeffrey.wargo@osumc.edu
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