Cutaneous Manifestations of Unspecified Peripheral T-Cell Lymphoma May Be Indicative of Disease Activity and Predict Response to Therapy

Schowalter, Michael; Akilov, Oleg E.; Story, Sara K.; Geskin, Larisa J.

doi:10.1200/jco.2012.41.6644

Cited by 6 publications

(4 citation statements)

References 9 publications

(9 reference statements)

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“…To date, this is the largest study of primary cutaneous PTL NOS using the current criteria for the classification of cutaneous lymphomas (WHO classification 2018 and WHO‐EORTC classification 2018) 4,5 . Secondary cutaneous involvement by nodal PTL NOS which may clinically and histologically present with similar findings to primary cutaneous PTL NOS was excluded by staging examinations 13,14 . We could confirm a male predominance (3 : 1) and a median age at first manifestation in the 7 th decade of life 1,3,14,15 …”

Section: Discussionmentioning

confidence: 81%

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico‐pathological and prognostic features

Kempf

Mitteldorf

Battistella

et al. 2020

Acad Dermatol Venereol

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Background Cutaneous peripheral T‐cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm. Objectives The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome. Methods Retrospective analysis of clinical data and histopathological features by an expert panel. Results Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours 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 tumour cells. All solitary tumours 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 tumour cells was associated with a better outcome than medium to large or large tumour 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 Cutaneous 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.

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Section: Discussionmentioning

confidence: 81%

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico‐pathological and prognostic features

Kempf

Mitteldorf

Battistella

et al. 2020

Acad Dermatol Venereol

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“…4 CPTL-NOS demonstrate poor response to conventional therapies. 9 Complete remission after chemotherapy and radiotherapy is typical for short duration and followed by relapses. 2 According to the WHO-EORTC classification for cutaneous lymphoma, CPTL-NOS have an aggressive behavior.…”

Section: Discussionmentioning

confidence: 99%

A Challenging Cutaneous T-Cell Lymphoma

Khadhar

Chelly²,

Zehani³

et al. 2016

The American Journal of Dermatopathology

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Cutaneous peripheral T-cell lymphomas not otherwise specified (CPTL-NOS) are rare neoplasms accounting for just 2% of cutaneous peripheral T-cell lymphomas (CPTL). Only very few case series have been reported. They represent a phenotypically and prognostically heterogenous group of CPTL that do not fit into any of CPTL well-defined subtypes. The authors report a case of a 64-year-old man with simultaneous plaque-like lesions and disseminated nodules growing rapidly on the face, trunk, and extremities over a 6-month period. There was no a history of preceding patches, erythematous plaques, rash, or pruritic lesions. These lesions were extending over 80% of the skin surface. Histopathologic analysis revealed dense diffuse infiltrates composed of mostly medium-sized to large lymphoid cells throughout the entire dermis without epidermotropism. Neoplastic cells were atypical with markedly pleomorphic nuclei. Immunohistochemistry showed that the tumor cells were positive for CD3, CD4, and CD5 with a loss of CD7. They were negative for CD20, CD8, CD56, CXCL13, PD1, TIA-1, granzyme-B, perforin, CD25, and CD30. The proliferative fraction was low, with MIB-1 labeling less than 10% of cells. The authors diagnosed the patient with primary CPTL-NOS. Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTL especially because most of these lymphomas have an aggressive behavior and exhibit an unfavorable prognosis.

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“…1,2 PTCLs-NOS usually have a nodal presentation, while a skin spread occurs in approximately 20% of cases 3 being regarded as a worse prognostic factor. [4][5][6] PTCLs-NOS featuring skin lesions present at least six months before a hypothetical systemic involvement are regarded as a separate entity, named primary cutaneous PTCLs-NOS (PC-PTCLs-NOS). 3,4,7 Owing to the fact that an increasing number of cutaneous T-cell lymphoma (CTCL) other than mycosis fungoides (MF) has been recognized in recent years, 2,8 the prevalence of PC-PTCL-NOS cases has significantly decreased.…”

Section: Primary Cutaneous Peripheral T-cell Lymphoma Not Otherwise Smentioning

confidence: 99%

The clinical phenotype and a novel COL7A1 mutation in a Chinese family with dystrophic epidermolysis bullosa pruriginosa

Zhang

et al. 2018

Acad Dermatol Venereol

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Cutaneous Manifestations of Unspecified Peripheral T-Cell Lymphoma May Be Indicative of Disease Activity and Predict Response to Therapy

Cited by 6 publications

References 9 publications

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico‐pathological and prognostic features

Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified: results of a multicentre European Organization for Research and Treatment of Cancer (EORTC) cutaneous lymphoma taskforce study on the clinico‐pathological and prognostic features

A Challenging Cutaneous T-Cell Lymphoma

The clinical phenotype and a novel COL7A1 mutation in a Chinese family with dystrophic epidermolysis bullosa pruriginosa

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