A Case of Primary Cutaneous Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type, With a 22-Year Indolent Clinical Course

Zuriel, Daniel; Fink‐Puches, Regina; Cerroni, Lorenzo

doi:10.1097/dad.0b013e318230ee41

Cited by 19 publications

(10 citation statements)

References 9 publications

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“…However, cases with a chronic indolent course have recently been reported, challenging the concept that this lymphoma shows always an aggressive course and poor prognosis. 25 In analogy, one could argue that our cases represent an epidermotropic and folliculotropic subtype of CGD-TCL characterized by an indolent course and not requiring aggressive therapy. Recently, a case of a 57-year-old woman with a 3-year history of an indolent CGD-TCL was reported by Endly et al 10 This CGD-TCL presented with nodules on the leg and predominantly subcutaneous g/d clonal T-cell infiltrates that rapidly improved within months of starting systemic corticosteroids.…”

Section: Discussionmentioning

confidence: 99%

Two Cases of Primary Cutaneous Lymphoma With a γ/δ+ Phenotype and an Indolent Course

Kempf¹,

Kazakov

Scheidegger

et al. 2014

The American Journal of Dermatopathology

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Cutaneous [gamma]/[delta]+ T-cell lymphoma (CGD-TCL) is a rare but aggressive lymphoma associated with a poor prognosis in most patients. The clinicopathological spectrum is variable including predominantly epidermotropic infiltrates manifesting with patches and plaques or tumors with dermal and/or subcutaneous infiltrates. The diagnosis of CGD-TCL requires the demonstration of a [gamma]/[delta]+ phenotype by immunohistochemistry. We report 2 patients with epidermotropic cutaneous T-cell lymphomas displaying a [gamma]/[delta]+ phenotype, but exhibiting an indolent course. In one patient, the clinical presentation was similar to mycosis fungoides in patch and plaque stage, but recurrent blister formation within the lesions was observed accompanied by fever and arthralgias, whereas the second patient presented with 2 localized erosive plaques on the left temple and dense epidermotropic and dermal diffuse and folliculotropic infiltrates of atypical small-to-medium-sized lymphocytes. exhibiting an indolent course. In one patient, the clinical presentation was similar to mycosis fungoides in patch and plaque stage, but recurrent blister formation within the lesions was observed accompanied by fever and arthralgias, whereas the second patient presented with 2 localized erosive plaques on the left temple and dense epidermotropic and dermal diffuse and folliculotropic infiltrates of atypical small-tomedium-sized lymphocytes. These cases corroborate the view that expression of a g/d+ phenotype in cutaneous T-cell lymphomas per se does not portend a worse prognosis and that CGD-TCL may represent a clinically and prognostically heterogeneous group.

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Section: Discussionmentioning

confidence: 99%

Two Cases of Primary Cutaneous Lymphoma With a γ/δ+ Phenotype and an Indolent Course

Kempf¹,

Kazakov

Scheidegger

et al. 2014

The American Journal of Dermatopathology

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“…The first notable feature of our case is the long lasting indolent biological behavior. Slightly better survival rate were actually already reported in primary cutaneous lesions [7] where two cases run a remarkably favourable course of 10 and 22 years [8] [9]. Considering the nasal area, two recent series described ENKL cases with late relapses (ranging from 40 months to 10 -29 years) after chemotherapy induced remission, among which 2 and 8 patients respectively showed nasal presentation [10] [11]; an additional report dealt with an exceptional case of recurrence after 38 years remission [12].…”

Section: Discussionmentioning

confidence: 84%

A Case of Sinonasal Extranodal NK/T-Cell Lymphoma with Indolent Behaviour and Low-Grade Morphology

Tabanelli¹,

Valli²,

Zanelli³

et al. 2014

CRCM

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Extranodal NK/T-cell lymphoma, nasal type (ENKL), is an uncommon subtype of EBV associated lymphoma usually characterized by aggressive clinical course. We report an atypical sinonasal ENKL case with long-lasting indolent behaviour, developed in the setting of a polymorphic EBVassociated lymphoproliferative disorder (LPD). A 52-year-old woman had suffered from chronic sinusitis and nasal obstruction since 2000, moderately worsened during the last years (marked enlargement of the sino-nasal mucosa at MRI in 2011) with elevated anti-VCA IgG and IgM titers. Three subsequent biopsies revealed slightly increasing morphophenotypic atypia, ranging from a polymorphic Band T-cell EBV positive proliferation (diagnosed in 2011, but not fulfilling CAEBV diagnostic criteria) to an overt monomorphic mildly atypical T LPD without necrosis and angiocentricty diagnosed as ENKL in 2013 upon immunophenotype and TCR-γ gene clonal rearrangement. Clinically indolent ENKL with low-grade morphology is extremely rare and diagnostically challenging; while the few reports in the literature describe long-survival in ENKL treated patients comparing histologically neoplastic lesions at onset and recurrences, no reports are published on the slow progression from a polymorphic EBV-related T/NK proliferation to a histologically overt clinically indolent ENKL in an untreated patient who only received occasional steroid administration.

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“…It can occur extranasally, including in the skin, lungs and the gastrointestinal tract. There are reports of ENKTL‐NT occurring on the forearm, as in Case 1, though this appears to be an unusual site …”

Section: Discussionmentioning

confidence: 88%

Uncommon cutaneous lymphoproliferative disorders in two patients with rheumatoid arthritis

et al. 2016

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A Case of Primary Cutaneous Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type, With a 22-Year Indolent Clinical Course

Cited by 19 publications

References 9 publications

Two Cases of Primary Cutaneous Lymphoma With a γ/δ+ Phenotype and an Indolent Course

Two Cases of Primary Cutaneous Lymphoma With a γ/δ+ Phenotype and an Indolent Course

A Case of Sinonasal Extranodal NK/T-Cell Lymphoma with Indolent Behaviour and Low-Grade Morphology

Uncommon cutaneous lymphoproliferative disorders in two patients with rheumatoid arthritis

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