Lymphomatoid papulosis with T-cell receptor-gamma delta expression: A clinicopathologic case-series of 26 patients of an underrecognized immunophenotypic variant of lymphomatoid papulosis Journal Article
| Authors: | Mark, E.; Kempf, W.; Guitart, J.; Pulitzer, M.; Mitteldorf, C.; Hristov, A.; Torres-Cabala, C.; Marchi, E.; Cropley, T.; Rodriguez Pinilla, S. M.; Griffin, T.; Fernandez, R.; Pileri, S.; Pileri, A.; Tabanelli, V.; Borretta, L.; Subtil, A.; Plaza, J. A.; Piris, J. A. M. A.; Feldman, A. L.; Cerroni, L.; Gru, A. A. |
| Article Title: | Lymphomatoid papulosis with T-cell receptor-gamma delta expression: A clinicopathologic case-series of 26 patients of an underrecognized immunophenotypic variant of lymphomatoid papulosis |
| Abstract: | Lymphomatoid papulosis (LyP) has several histopathologic presentations. LyP featuring gamma-delta (γδ) T-cell receptor expression may masquerade as and may be misdiagnosed as aggressive cutaneous T-cell lymphoma, particularly primary cutaneous γδ T-cell lymphoma (PCGDTL) or γδ mycosis fungoides. We performed a clinicopathologic analysis of the largest series of LyP featuring γδ T-cell expression. We identified 26 patients with a diagnosis of LyP with γδ T cells from our institutions, as well as through a comprehensive review of the literature, and characterized these cases. Most cases were treated with topical steroids or not treated at all. The majority of cases showed a CD4-CD8+phenotype and featured at least one cytotoxic marker. Histopathologic features included an intraepidermal or dermal infiltrate with large cells and frequent angiotropism. One case was initially misdiagnosed as PCGDTL, requiring further therapy. Our case series, the largest international cohort of γδ T cell predominant LyP cases, confirms marked clinicopathologic heterogeneity that may contribute to misdiagnosis, reasserting the need to identify classic clinical features, CD30+T-cell components, and markers of cytotoxicity when dealing with this differential diagnosis. A limitation of this study includes somewhat limited follow-up, histologic, and immunophenotypic information for some cases. © 2024 Wolters Kluwer Health. All rights reserved. |
| Keywords: | immunohistochemistry; adult; child; clinical article; controlled study; protein expression; aged; middle aged; young adult; clinical feature; histopathology; methotrexate; molecular genetics; follow up; cd3 antigen; cd8 antigen; ki 67 antigen; phenotype; skin neoplasms; differential diagnosis; pathology; cutaneous t-cell lymphoma; cd2 antigen; cd7 antigen; t lymphocyte receptor; cutaneous t cell lymphoma; skin tumor; receptors, antigen, t-cell; diagnostic error; immunophenotyping; cd4 antigen; cd5 antigen; mycosis fungoides; lymphocyte antigen receptor; demographics; cd56 antigen; gamma delta t lymphocyte; lymphoma, t-cell, cutaneous; cd4 cd8 ratio; lymphomatoid papulosis; brentuximab vedotin; dermatopathology; humans; human; male; female; article; primary cutaneous gamma-delta t-cell lymphoma; cutaneous gamma delta t cell lymphoma; cd30<sup>+</sup>t-cell lymphoproliferative disorders; gamma-delta t-cells |
| Journal Title: | American Journal of Surgical Pathology |
| Volume: | 48 |
| Issue: | 5 |
| ISSN: | 0147-5185 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2024-05-01 |
| Start Page: | 501 |
| End Page: | 510 |
| Language: | English |
| DOI: | 10.1097/pas.0000000000002200 |
| PUBMED: | 38533681 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | The MSK Cancer Center Support Grant (P30 CA008748) is acknowledged in the PDF -- Source: Scopus |
