A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma
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...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-06-01
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| Series: | Dermatopathology |
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| Online Access: | https://www.mdpi.com/2296-3529/8/2/27 |
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| author | Tatsiana Pukhalskaya Bruce R. Smoller David M. Menke Olayemi Sokumbi |
| author_facet | Tatsiana Pukhalskaya Bruce R. Smoller David M. Menke Olayemi Sokumbi |
| author_sort | Tatsiana Pukhalskaya |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T10:20:44Z |
| format | Article |
| id | doaj.art-ad75ee5a414342c69a451ce3c4a5bee2 |
| institution | Directory Open Access Journal |
| issn | 2296-3529 |
| language | English |
| last_indexed | 2024-03-10T10:20:44Z |
| publishDate | 2021-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Dermatopathology |
| spelling | doaj.art-ad75ee5a414342c69a451ce3c4a5bee22023-11-22T00:26:44ZengMDPI AGDermatopathology2296-35292021-06-018222122810.3390/dermatopathology8020027A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell LymphomaTatsiana Pukhalskaya0Bruce R. Smoller1David M. Menke2Olayemi Sokumbi3Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY 14642, USADepartment of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY 14642, USADepartment of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, FL 32224, USADepartment of Dermatology, Mayo Clinic, Jacksonville, FL 32224, USAPrimary 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.https://www.mdpi.com/2296-3529/8/2/27primary cutaneous γδ T-cell lymphomaPCGD-TCLhistiocyte-rich PCGD-TCL |
| spellingShingle | Tatsiana Pukhalskaya Bruce R. Smoller David M. Menke Olayemi Sokumbi A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma Dermatopathology primary cutaneous γδ T-cell lymphoma PCGD-TCL histiocyte-rich PCGD-TCL |
| title | A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma |
| title_full | A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma |
| title_fullStr | A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma |
| title_full_unstemmed | A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma |
| title_short | A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma |
| title_sort | previously unrecognized granulomatous variant of gamma delta t cell lymphoma |
| topic | primary cutaneous γδ T-cell lymphoma PCGD-TCL histiocyte-rich PCGD-TCL |
| url | https://www.mdpi.com/2296-3529/8/2/27 |
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