Primary Cutaneous Gamma-Delta T-cell Lymphoma: Not an Aggressive Disease in All Cases

Khallaayoune, Mehdi; Grange, Florent; Condamina, Morgane; Szablewski, Vanessa; Guillot, B.; Dereure, O.

doi:10.2340/00015555-3340

Cited by 8 publications

(5 citation statements)

References 16 publications

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“…7 Clonal rearrangement of T-cell receptor delta and gamma genes characterizes the disease. 5 This case presented with a classic phenotypic profile with the exception of the weak CD4 immunohistochemical positivity.…”

Section: Answermentioning

confidence: 89%

“…1 Median survival from the time of diagnosis ranges from 15 to 31 months; however, indolent cases have been reported. 3–5…”

Section: Answermentioning

confidence: 99%

“…1 Median survival from the time of diagnosis ranges from 15 to 31 months; however, indolent cases have been reported. [3][4][5] PCGD-TCL is staged per the International Society for Cutaneous Lymphoma/European Organization of Research and Treatment of Cancer (ISCL/EORTC) TNM classification system of cutaneous lymphoma other than mycosis fungoides and Sezary syndrome. 6 The patient in this case underwent CT imaging of the chest/abdomen/pelvis, which was negative for adenopathy or metastatic disease, and was staged by oncology as T3bN0M0.…”

mentioning

confidence: 99%

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Erythematous Patches and Plaques: What to Make of a Rash that Looks like Pepperoni Pizza?

Murray,

Fagan,

Timmermann

et al. 2023

The American Journal of Dermatopathology

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Section: Answermentioning

confidence: 89%

“…1 Median survival from the time of diagnosis ranges from 15 to 31 months; however, indolent cases have been reported. 3–5…”

Section: Answermentioning

confidence: 99%

mentioning

confidence: 99%

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Erythematous Patches and Plaques: What to Make of a Rash that Looks like Pepperoni Pizza?

Murray,

Fagan,

Timmermann

et al. 2023

The American Journal of Dermatopathology

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“…Accumulating evidence exists that PCGDTCL is not an aggressive type of lymphoma in all cases . A predominantly epidermotropic pattern of disease might be associated with a more favorable outcome compared with subcutaneous involvement . However, the rapid progression and deep extension of PCGDTCL in the current case probably indicates a poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Bendamustine Monotherapy for Primary Cutaneous Gamma-Delta T-Cell Lymphoma

et al. 2020

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“…Histologically, some cases of PCGD-TCL predominantly involve the epidermis and/or the dermis, while others are centered in the subcutaneous adipose tissue [ 9 ]. PCGD-TCL is commonly resistant to all forms of treatment, although cases with an indolent clinical course have been reported [ 1 , 10 , 11 ].…”

Section: Introductionmentioning

confidence: 99%

A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma

et al. 2021

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Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an extremely rare and aggressive T-cell neoplasm with complex heterogeneity. We present a series of two patients who presented with firm, subcutaneous nodules and were diagnosed with PCGD-TCL. In both cases, biopsies demonstrated a both superficial and deep adnexotropic infiltrate comprised of angiocentric, medium- to large-sized atypical lymphocytes. The infiltrate extended into the panniculus. Immuno–histochemical stains highlighted atypical lymphocytes that expressed CD3, CD8 and CD56 but were negative for EBV ISH. A brisk histiocytic response with focal aggregation into granulomas was highlighted with a PG-M1 stain. The atypical lymphocytes were positive for gene rearrangements on a TCR delta stain and negative for βF-1. CT and PET scan in one of the two patients demonstrated diffuse, subcutaneous, ground-glass foci; hypermetabolic soft tissue nodules; and lymphadenopathy in the lungs, as well as splenomegaly. A diagnosis of histiocyte-rich PCGD-TCL was rendered. A histiocyte-rich, granulomatous variant of γδ T-cell lymphoma is extremely rare. Its potentially misleading resemblance to inflammatory granulomatous conditions could pose a diagnostic pitfall in this already challenging condition. This variant may resemble granulomatous mycosis fungoides and granulomatous slack skin syndrome, but it has a distinct, aggressive clinical outcome.

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Primary Cutaneous Gamma-Delta T-cell Lymphoma: Not an Aggressive Disease in All Cases

Cited by 8 publications

References 16 publications

Erythematous Patches and Plaques: What to Make of a Rash that Looks like Pepperoni Pizza?

Erythematous Patches and Plaques: What to Make of a Rash that Looks like Pepperoni Pizza?

Bendamustine Monotherapy for Primary Cutaneous Gamma-Delta T-Cell Lymphoma

A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma

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