Tom 8, Nr 4 (2023)
Obrazy w medycynie
Opublikowany online: 2023-07-04

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Rare skin tumor – primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Damian W. Trzos1, Wojciech M. Wysocki213
Biuletyn Polskiego Towarzystwa Onkologicznego Nowotwory 2023;8(4):310.

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Obrazy w onkologii / Pictures in oncology

Biuletyn Polskiego
Towarzystwa Onkologicznego
NOWOTWORY

2023, tom 8, nr 4, 310

© Polskie Towarzystwo Onkologiczne

ISSN: 2543–5248, e-ISSN: 2543–8077

www.nowotwory.edu.pl

Rare skin tumor – primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Damian W. Trzos1Wojciech M. Wysocki123
1Department of General and Oncological Surgery, 5th Military Clinical Hospital in Krakow, Krakow, Poland
2Chair of Surgery, Faculty of Medicine and Health Sciences, Andrzej Frycz Modrzewski Krakow University, Krakow, Poland
3Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

Jak cytować / How to cite:

Trzos DW, Wysocki WM. Rare skin tumor primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. NOWOTWORY J Oncol 2023; 73: 256.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCS-TCLPD) is a rare disease with no clear diagnostic and treatment guidelines [1]. According to the WHO classification of hematopoietic neoplasms, this is an indolent T-cell lymphoproliferative disorder confined to the skin, with a characteristic population of T cells with a follicular T-helper phenotype [2]. So far, this poorly defined disease has an undetermined malignant potential [3].

We present a case report of a 46-years-old Caucasian male who presented with a flat circular erythematous skin lesion on his forehead (fig. 1). The lesion was excised and histopathology revealed a skin covered with epithelium without atypia, massive lymphocytic infiltration extending into the subcutaneous tissue. There was perivascular infiltration and infiltration of skin appendages; CD3+ T cells predominate the lesion; CD4+ significantly predominate over CD8–/+, CD30–. The image most closely matched PCS-TCLPD.

Figure 1. Patient’s nodular lesion on the forehead

PCS-TCLPD has no long-term risk of secondary lymphomas and an excellent prognosis. It has an indolent clinical behavior with a 5-year survival rate of 100% [1, 2]. Imaging modalities and bone marrow evaluations are of a relatively low diagnostic value and are not mandatory [1]. Local surgical treatment can be used with a high degree of success and should be considered before other options [1]. In summary, PCS-TCLPD is a rare disease, usually presenting as a plaque or nodule in the head and neck region and can be treated successfully by simple surgical excision with clear margins [3].

References

  1. Besch-Stokes JG, Costello CM, Severson KJ, et al. Primary cutaneous CD4 small/medium T-cell lymphoproliferative disorder: Diagnosis and management. J Am Acad Dermatol. 2022; 86(5): 1167–1169, doi: 10.1016/j.jaad.2021.04.067, indexed in Pubmed: 33915243.
  2. Gru AA, Wick MR, Eid M. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg. 2018; 37(1): 39–48, doi: 10.12788/j.sder.2018.006, indexed in Pubmed: 29719019.
  3. Surmanowicz P, Doherty S, Sivanand A, et al. The Clinical Spectrum of Primary Cutaneous CD4+ Small/Medium-Sized Pleomorphic T-Cell Lymphoproliferative Disorder: An Updated Systematic Literature Review and Case Series. Dermatology. 2021; 237(4): 618–628, doi: 10.1159/000511473, indexed in Pubmed: 33326960.



Biuletyn Polskiego Towarzystwa Onkologicznego Nowotwory