Journal article
Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (NOS): results of a multicenter EORTC cutaneous lymphoma taskforce study on the clinico-pathological and prognostic features.
- Kempf W Kempf und Pfaltz Histologische Diagnostik, Affolternstrasse 56, 8050, Zürich, Switzerland.
- Mitteldorf C University Medical Center Göttingen, Department of Dermatology, Venereology and Allergology, Robert-Koch-Str. 40, 37073, Göttingen, Germany.
- Battistella M Department of Pathology, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris University, INSERM U976, 75010, Paris, France.
- Willemze R Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
- Cerroni L Department of Dermatology, Medical University of Graz, Graz, Austria.
- Santucci M Department of Health Sciences, University of Florence, School of Human Health Sciences and Division of Histopathology and Molecular Diagnostics, Careggi University Hospital, Florence, Italy.
- Geissinger E Institute of Pathology, University of Würzburg, Würzburg, Germany.
- Jansen P Department of Clinical Pathology, Leiden University Medical Center, Leiden, The Netherlands.
- Vermeer MH Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands.
- Marschalko M Semmelweis Medical University, Department of Dermatology and Venerology, Budapest, Hungary.
- Papadavid E Department of Dermatology-Venereology, Attikon University Hospital, National and Athens, Athens, Greece.
- Piris MA Dept of Pathology, Fundacion Jimenez Diaz, CIBERONC, Madrid, Spain.
- Ortiz-Romero PL Department of Dermatology. Hospital 12 de Octubre. Medical School. Institute i+12, University Complutense. Madrid, Madrid, Spain.
- Novelli M Cutaneous Immunopathology Laboratory, Dermatology Clinic, Department of Medical Sciences, University of Turin, 10127, Turin, Italy.
- Paulli M Department of Molecular Pathology, University of Pavia, Department of Anatomic Pathology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
- Quaglino P Dermatologic Clinic, Dept Medical Sciences, University of Turin Medical School, Torino, Italy.
- Ranki A Department of Dermatology and allergology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
- Rodríguez Peralto JL Department of Pathology, Hospital Universitario 12 de Octubre, Universidad, Complutense, Instituto de Investigación I+12, Madrid, Spain.
- Auschra B Department of Consultation-Liaison Psychiatry and Psychosomatic Medicine, University Hospital Zurich, Zurich, Switzerland.
- Robson A Institute of Oncology, Lisbon, Portugal, Portugal and LDPath London, London, UK.
- 2020-09-30
Published in:
- Journal of the European Academy of Dermatology and Venereology : JEADV. - 2020
English
BACKGROUND
Cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm.
OBJECTIVES
The EORTC cutaneous lymphoma taskforce (CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyze their clinical, histological, immunophenotypic features and outcome.
METHODS
Retrospective analysis of clinical data and histopathological features by an expert panel.
RESULTS
PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumors or disseminated papulo-nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+/CD4-/CD8- and CD3+/CD4+/CD8+. Moreover, 18% of the cases exhibited an aberrant expression of the B-cell marker CD20 by the tumor cells. All solitary tumors were located on the limbs and presented a high expression of GATA-3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow-up of 36 months (mean value; range 3-144). The survival rates were 61% after 3 years (CI 43 - 85%) and 54% after 5 years (CI 36 - 81%). Small to medium-sized morphology of tumor cells was associated with a better outcome than medium to large or large tumor cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA-3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first-line treatment for solitary lesions.
CONCLUSIONS
PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS.
Cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm.
OBJECTIVES
The EORTC cutaneous lymphoma taskforce (CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyze their clinical, histological, immunophenotypic features and outcome.
METHODS
Retrospective analysis of clinical data and histopathological features by an expert panel.
RESULTS
PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumors or disseminated papulo-nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+/CD4-/CD8- and CD3+/CD4+/CD8+. Moreover, 18% of the cases exhibited an aberrant expression of the B-cell marker CD20 by the tumor cells. All solitary tumors were located on the limbs and presented a high expression of GATA-3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow-up of 36 months (mean value; range 3-144). The survival rates were 61% after 3 years (CI 43 - 85%) and 54% after 5 years (CI 36 - 81%). Small to medium-sized morphology of tumor cells was associated with a better outcome than medium to large or large tumor cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA-3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first-line treatment for solitary lesions.
CONCLUSIONS
PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS.
- Language
-
- English
- Open access status
- closed
- Identifiers
-
- DOI 10.1111/jdv.16969
- PMID 32997839
- Persistent URL
- https://folia.unifr.ch/global/documents/121412
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