Abstract
BackgroundPrimary cutaneous CD30+ lymphoproliferative disorders (pc-CD30-LPD) are a group of clonal T cell lymphoproliferative disorders that despite very similar tumor histology follow different and characteristic clinical courses, suggesting a homeostatic role of the tumor microenvironment. Little is known about tumor microenvironment and there is almost no literature about PD-L1 expression in pc-CD30-LPD.MethodsThis retrospective study presents a fully clinicopathologically characterized series of pc-CD30-LPDs from an academic medical center in Brazil, including 8 lymphomatoid papulomatosis (LyP), 9 primary cutaneous anaplastic large cell lymphoma (pcALCL) and 4 borderline lesions. All the cases were scored for FOXP3+ regulatory T-cells (Treg) and CD8+ cytotoxic tumor infiltrating lymphocytes (TIL) densities, as well as PD-L1 expression in tumor cells and tissue associated macrophages. The CD8+/FOXP3+ ratio was also evaluated.ResultsAmong the 21 cases of pc-CD30-LPD, PD-L1 expression is frequent in both tumor cells and tissue associated macrophages in pc-CD30-LPD across categories, suggesting that the PD-L1 axis may be a common feature of pc-CD30-LPDs. While reactive T cell infiltrates vary widely from case to case, a common feature across pc-CD30-LPDs is higher density of CD8 than FOXP3 + T cells. The distribution of T cells within the lesions however differed between LyP and pcALCL: we found that LyP lesions tend to be permeated by CD8+ and FOXP3+ T cells, whereas pcALCL tend to be surrounded by a rim of CD8+ TIL and FOXP3+ Tregs with relatively lower density infiltrates in the center of the lesion.ConclusionsLyP has a trend to have denser immune cells throughout the lesion, with higher FOXP3+ Treg and CD8+ TIL in the center than the edge comparing with pcALCL. PD-L1+ is frequent in tumor cells and tissue associated macrophages in pc-CD30-LPD. The differential distribution of CD8+ and FOXP3+ TILs in LyP as compared to pcALCL could provide a clue to the relapsing/remitting course of LyP as compared to the less frequent spontaneous regression of pcALCL.
Highlights
Primary cutaneous CD30+ lymphoproliferative disorders include the closely related T lymphoproliferative disorders primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis (LyP) and borderline lesions [1]
One patient with LyP had a subsequent diagnosis of mycosis fungoides (MF) 19 years later
One patient with a borderline lesion had a histological diagnosis of primary cutaneous anaplastic large cell lymphoma (pcALCL) in a biopsy of a solitary nodule on the face, but by clinic-dermatological evaluation she presented generalized self-healing papules and nodules characteristic of LyP, so a diagnosis of borderline lesion was rendered by dermatology-pathology correlation
Summary
Primary cutaneous CD30+ lymphoproliferative disorders (pc-CD30-LPD) include the closely related T lymphoproliferative disorders primary cutaneous anaplastic large cell lymphoma (pcALCL), lymphomatoid papulosis (LyP) and borderline lesions [1]. The typical waxing and waning course of LyP, and the indolent course and occasional spontaneous regression of pcALCL, raise the question of the role of the tumor microenvironment in the clinical course of pc-CD30-LPDs. Immune escape is an active process of immune evasion by neoplastic cells. We provide here a study of the expression of PD-L1 and the density of FOXP3 Treg and CD8+ T-cells to better understand the role of tumor microenvironment in a well characterized cohort of pc-CD30-LPD from an academic medical center in Brazil. Primary cutaneous CD30+ lymphoproliferative disorders (pc-CD30-LPD) are a group of clonal T cell lymphoproliferative disorders that despite very similar tumor histology follow different and characteristic clinical courses, suggesting a homeostatic role of the tumor microenvironment. Little is known about tumor microenvironment and there is almost no literature about PD-L1 expression in pc-CD30-LPD
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