Primary Cutaneous Anaplastic Large Cell Lymphoma With TCR-γδ Expression: A Case Series of Eleven Patients of a Rare Immunophenotypic Variant Journal Article
| Authors: | Erem, A. S.; Kempf, W.; Mitteldorf, C.; Pulitzer, M.; Torres-Cabala, C. A.; Pileri, S.; Pinilla, S. M. R.; Feldman, A. L.; Gru, A. A. |
| Article Title: | Primary Cutaneous Anaplastic Large Cell Lymphoma With TCR-γδ Expression: A Case Series of Eleven Patients of a Rare Immunophenotypic Variant |
| Abstract: | Background: Primary cutaneous anaplastic large cell lymphoma (pcALCL) and lymphomatoid papulosis (LyP) are indolent CD30-positive lymphoproliferative disorders that rarely express TCR-γδ. However, primary cutaneous gamma-delta T-cell lymphoma (pcGDTCL), characterized by TCR-γδ expression on neoplastic cells, is a rare, aggressive cutaneous T-cell lymphoma with a poor prognosis. Accurate differentiation is essential due to distinct clinical behavior and treatment. Methods: We identified 11 TCR-γδ-positive pcALCL cases from internal and consultation files, verified by two cutaneous lymphoma experts, with clinicopathologic data recorded. Results: The median age was 68 years (range 38–95). Most cases presented as a single dermal lesion on the upper extremities. All were ALK-negative, CD30-positive (> 90% tumor cells), TCR-βF1-negative, and diffusely TCR-γδ-positive. CD4−/CD8− (54.5%) and CD4+/CD8− (45.5%) immunophenotypes were observed. CD2 (63.6%) and CD3 (54.5%) were the most common T-cell antigens. Ulceration, inflammation (both 45.5%), and necrosis (36.4%) were frequent. Angiotropism, angiocentricity, and myxoid stroma appeared in one case; epidermotropism in two. Four of ten tested were DUSP22-rearranged, and TP63 was negative in all eight tested. Conclusions: Most ALK-negative TCR-γδ-positive pcALCL were CD4−/CD8−, followed by CD4+/CD8−. DUSP22 rearrangement occurred in 40% of cases, similar to reports in typical pcALCL. Its prognosis and the role of DUSP22 are yet to be clarified. © 2025 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. |
| Keywords: | adult; aged; aged, 80 and over; middle aged; genetics; metabolism; skin neoplasms; pathology; skin tumor; biosynthesis; immunophenotyping; dual-specificity phosphatases; mitogen-activated protein kinase phosphatases; lymphocyte antigen receptor; dual specificity phosphatase; receptors, antigen, t-cell, gamma-delta; lymphoma, large-cell, anaplastic; anaplastic large cell lymphoma; procedures; lymphoma, primary cutaneous anaplastic large cell; very elderly; humans; human; male; female; cd30-positive lymphoproliferative disorders; primary cutaneous anaplastic large cell lymphoma; dusp22; primary cutaneous gamma-delta t-cell lymphoma; tcr gamma delta; dusp22 protein, human; mitogen activated protein kinase phosphatase |
| Journal Title: | Journal of Cutaneous Pathology |
| Volume: | 52 |
| Issue: | 7 |
| ISSN: | 0303-6987 |
| Publisher: | John Wiley & Sons |
| Date Published: | 2025-01-01 |
| Start Page: | 487 |
| End Page: | 496 |
| Language: | English |
| DOI: | 10.1111/cup.14809 |
| PUBMED: | 40202044 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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