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Case Report/Case Series |

Expression of CD31/PECAM-1 (Platelet Endothelial Cell Adhesion Molecule 1) by Blastic Plasmacytoid Dendritic Cell Neoplasms

Katrin A. Salva, MD1; Anna K. Haemel, MD2,3; Laura B. Pincus, MD2,3; Jing Liu, MD4; Uma Sundram, MD, PhD5,6; Joan Guitart, MD7; B. Jack Longley, MD1; Gary S. Wood, MD1
[+] Author Affiliations
1Department of Dermatology, University of Wisconsin, Madison
2Department of Dermatology, University of California, San Francisco
3Department of Pathology, University of California, San Francisco
4Department of Dermatology, University of Minnesota, Minneapolis
5Department of Dermatology, Stanford University
6Department of Pathology, Stanford University
7Department of Dermatology, Northwestern University, Evanston, Illinois
JAMA Dermatol. 2014;150(1):73-76. doi:10.1001/jamadermatol.2013.7141.
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Importance  Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare malignant neoplasm with cutaneous manifestations and a rapidly progressive clinical course. The diagnosis relies on characteristic clinicopathologic and immunopathologic features. However, the overlap of immunophenotypic features with other cancers, as well as newly discovered interpersonal and intrapersonal phenotypic variations, renders the identification of BPDCN challenging. A greater understanding of the proteins expressed by BPDCN might facilitate its recognition and provide insights into its clinical behavior.

Observations  In 7 of 9 patients at 4 tertiary care institutions, immunohistochemical analysis demonstrated strong CD31/PECAM-1 (platelet endothelial cell adhesion molecule 1) expression by neoplastic cells. Combined with similar findings observed in 1 former patient, 8 of 10 cases of BPDCN were CD31/PECAM-1 positive.

Conclusions and Relevance  Expression of CD31/PECAM-1 by BPDCN adds new information about the antigenic profile of this unusual neoplasm. CD31/PECAM-1 influences multiple cell functions including adhesion, apoptosis, coagulation, host response, and protein synthesis that might affect clinical features of BPDCN such as hemorrhage, aggressive tumor growth, and resistance to therapy. Therefore, the potential role of this molecule in the tumor formation and progression of BPDCN warrants additional exploration.

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Figure 1.
Clinical and Microscopic Images of the Illustrative Case

A, Shiny 2 × 3-cm violaceous nodule displaying a golden contusiform rim, located on the left deltoid. B, Corresponding histopathologic image (hematoxylin-eosin [H&E], original magnification, ×100): dense infiltrate of tumor cells in the upper and lower dermis. C, A ×400 magnification of the infiltrate shows monomorphous medium-sized cells with slightly irregular nuclei, finely dispersed chromatin, and scant cytoplasm. D, Tumor cells infiltrating upper dermis are associated extensive extravasation of erythrocytes (H&E, original magnification, ×200).

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Figure 2.
Immunohistochemical Staining of Formalin-Fixed Paraffin-Embedded Sections of the Lesion Shown in Figure 1

Diaminobenzidine, original magnification, ×200: CD56 (A), CD4 (B), CD123 (C), and CD31/PECAM-1 (platelet endothelial cell adhesion molecule 1) (D).

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