#dermpathJC October 2020 summary

#dermpathJC October 2020:

Thursday, October 29th, 9pm EST

Article Discussed: A review of CD30 expression in cutaneous neoplasms

Authors: Drs. Franziska Kampa and Christina Mitteldorf

Temporary free access courtesy of Journal of Cutaneous Pathology, link: https://onlinelibrary.wiley.com/doi/abs/10.1111/cup.13894

Summary prepared by: Maryam Aghighi, MD (@maryam_aghighi)

Editor: Silvija P. Gottesman, MD (@SGottesmanMD)

Journal Club Summary:

  • The current article reviewed the literature for CD30 expression in lymphoproliferative disorders and solid tumors of the skin.
  • CD30 (Ki-1) is a transmembrane protein in the tumor necrosis factor receptor superfamily. It is expressed by Reed-Steinberg cells. The CD30 ligand (cytokine) is detected on activated lymphocytes, histiocytes and granulocytes.
  • CD30 is expressed mainly on Th2 cells, but there is some published data that Th0 and Th1 cells show CD30 expression. As for B-cells, CD30 is expressed in B-cell immunoblasts, found at the germinal center periphery.
  • CD30 is expressed in anaplastic large cell lymphoma (ALCL), Hodgkin lymphoma (HL), lymphomatoid papulosis (LyP), mycosis fungoides (MF), follicle center cell lymphoma (FCL) and Epstein-Barr virus (EBV)-associated lymphomas.
  • Brentuximab vedotin (BV) is an anti-CD30 monoclonal antibody, conjugated with monomethylauristatin, and used in the treatment of CD30+ HL, systemic ALCL, CD30 expressing peripheral T-cell lymphomas and CD30+ cutaneous T-cell lymphomas.
  • Treatment with BV has been effective in cutaneous T-cell lymphomas (CTCL) and diffuse large B-cell lymphoma (DLBCL) including in cases with low or absent CD30 expression.
  • In cutaneous lymphomas, the CD30 positivity threshold is under debate. Up to 40% of hematopathologists use a positive cut-off value of >20% CD30+ cells, while the response cutoff in BV clinical trials was >10% CD30+.
  • Cutaneous and folliculotropic MF patients with a higher dermal CD30 expression showed a more advanced stage at diagnosis, higher maximum stage, lower survival rate and poor prognosis. However, patients with high epidermal CD30 expression revealed higher survival rate.
  • Transformed MF (T-MF) is diagnosed if >25% large cells (>4x the size of a lymphocyte) are present. Dermal CD30 expression is related to a better prognosis in T-MF and absence of CD30 expression was associated with poorer prognosis. Interestingly, higher epidermal CD30 expression compared to dermal CD30 expression was associated with a poorer survival.
  • In other rare types of CTCLs, published literature to date of CD30 expression is as follows:
    • In pagetoid reticulosis (PR), more than 50% of the tumor cells were CD30+.
    • Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) reported CD30 expression as follows:
      • One study showed low expression of CD30 (<5%) in most of their patients.
      • One study showed more than half the studied patients to have a CD30 expression of 25% or more.
      • In small-medium pleomorphic T-cell lymphoma (SMPTCL) CD30-positive expression was reported between <1% and 30%.
    • In cases of extranodal NK/T-cell-lymphoma (ENK/TCL), CD30 positivity has been seen and in one case reaching >80%.
    • Primary cutaneous gamma-delta T-cell lymphoma (pcGD-TCL), primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECTCL) and subcutaneous panniculitis-like T-cell Lymphoma (SPTL) reported CD30 expression (further reading recommended to explore the amount of CD30 expression in each of these distinct entities).
    • A single case of primary cutaneous acral CD8+ T-cell lymphoma (pcATCL) reported negative CD30 expression.
    • Adult T-cell leukemia/lymphoma (ATLL) reported positive CD30 expression but skin expression of CD30 positivity was not mentioned.
  • Observed CD30 expression in primary cutaneous B-cell lymphomas is as follows:
    • Primary cutaneous follicle center B-cell lymphomas with CD30 expression was seen in few patients and primary cutaneous marginal zone B-cell lymphoma showing CD30 positivity was seen in even fewer patients.
    • Four diffuse large B-cell lymphoma leg type patients reported negative CD30 staining.
  • EDV-positive mucocutaneous ulcer and EDV-associated lymphoproliferative disorders reported high CD30 expression.
  • Blastic plasmacytoid dendritic cell neoplasm – negative CD30 expression reported thus far.
  • Cutaneous mastocytosis had CD30 expression in 96.5% of cases (29 study patients).
  • Other notable neoplasms that also showed expression of CD30 were lymphadenomas. CD30 expression was reported in the the tumor microenvironment (TME) of BCC, SCC and keratoacanthomas.
  • There was no data reported regarding CD30 expression in Merkel cell carcinoma and only a couple of cases of atypical fibroxanthoma with reported CD30 expression.  

Memorable Tweets from the Journal Club Discussion:

Until next month #dermpathJC, stay safe!

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