Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis

Stoll, Joseph R.; Willner, Jonathan; Oh, Yuna; Pulitzer, Melissa; Moskowitz, Alison J.; Horwitz, Steven M.; Myskowski, Patricia L.; Noor, Sarah

doi:10.1016/j.jaad.2021.04.080

Cited by 35 publications

(51 citation statements)

References 147 publications

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“…The most common site of primary ENKTCL-NT is the nasopharynx and upper aerodigestive tract, while the skin is the most frequent initial extranasal presentation [ 3 ]. In addition, the skin is also the most common site of secondary spread [ 1 ].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Extremities are the most commonly involved [ 4 ]. Cutaneous ENKTCL-NT could be further classified into three categories, i.e., primary cutaneous ENKTCL-NT, which has no evidence of systemic or extracutaneous disease at the time of diagnosis, ENKTCL-NT with secondary cutaneous involvement, and ENKTCL-NT with extranasal manifestation and secondary cutaneous involvement, with the former type being the most common and the latter type having the worst prognosis [ 1 , 4 ].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Diagnosis of ENKTCL-NT is made by histology and immunohistochemistry findings, comprising large atypical lymphoid cells, expressing CD3, CD8, CD56, and positive Epstein-Barr virus-encoded RNA. T-cell receptor gene rearrangement can be negative in nearly half of patients; however, a negative result does exclude peripheral T-cell lymphoma, not otherwise specified, and subcutaneous panniculitis-like T-cell lymphoma that can mimic ENKTCL-NT, histologically [ 1 , 7 , 8 ].…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare and aggressive EBV-associated non-Hodgkin lymphoma. This entity carries poor response to current treatment, associated with a high relapse rate and mortality [ 1 , 2 ]. Various cutaneous manifestations have been reported, and histopathologic examination can mimic both inflammatory condition and other hematologic malignancies.…”

Section: Introductionmentioning

confidence: 99%

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Nonhealing Ulcers with Rimming of the Adipose Tissues

2022

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Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare condition which has a very aggressive clinical outcome. The most common and typical presentation is the destructive tumor involving the nasal cavity or nasopharynx, referred to as lethal midline granuloma, while cutaneous involvement is found to be the second most involved site. In this report, we describe a case of an otherwise healthy 40-year-old female solely presenting with ulcerative plaques and subcutaneous nodules on her lower extremities. Although the rimming of adipocytes by atypical lymphocytes, which resembles subcutaneous panniculitis-like T-cell lymphoma, was found on histopathology, immunohistochemistry revealed the diagnosis of cutaneous ENKTCL-NT which portends a much worse prognosis. A positron emission tomography scan also detected a hypermetabolic mass on her nasal cavity despite negative findings on history and initial examination. After thorough investigation, her final diagnosis was ENKTCL-NT with extranasal involvement (cutaneous) stage IV.

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Section: Discussion/conclusionmentioning

confidence: 99%

Section: Discussion/conclusionmentioning

confidence: 99%

Section: Discussion/conclusionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Nonhealing Ulcers with Rimming of the Adipose Tissues

2022

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“…This is the staining pattern for primary cutaneous anaplastic large cell lymphoma. 6 C. CD4 1 , CD8 À , CD30 1/À e Incorrect. This is the staining pattern for LyP type B.…”

Section: Dmentioning

confidence: 99%