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A 24-year-old male presented with a 2-month history of painless subcutaneous nodules on his trunk and limbs, associated with night sweats, fatigue, and malaise. On examination, there were widespread palpable subcutaneous lesions with an average diameter of 2–3 cm. There was no palpable lymphadenopathy or organomegaly. Peripheral blood count showed a mild neutropenia and anemia: hemoglobin 12.3 g/dL, platelet count 146 × 109/L, neutrophils 1.2 × 109/L, lymphocytes 0.7 × 109/L. His lactate dehydrogenase and beta-2-microglobulin were elevated. He had a reduced immunoglobulin A level but no monoclonal band was demonstrated on serum protein electrophoresis.

A skin biopsy showed an atypical small-to-medium-sized lymphoid infiltrate with typical panniculitic pattern involving the lobular subcutaneous adipose tissue and sparing the septae (Image 1). The lymphoid cells were seen rimming the adipocytes. The tumor cells were admixed with histiocytes and demonstrated prominent karyorrhexis. There was no hemophagocytosis. The tumor cells expressed CD3, CD2, CD5, CD7, CD8 (Image 2) granzyme B, and perforin, but not CD20, CD4, CD25, CD30, ALK, or CD56, consistent with subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Polymerase chain reaction analysis for T-cell receptor gene rearrangement indicated monoclonal T-cell expansion. Bone marrow biopsy did not demonstrate involvement.

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Figure 1. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

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Figure 2. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

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A half body FDG PET/CT (18F fluoro-2-deoxyglucose positron emission tomograph/computed tomograph) showed multiple metabolically active skin lesions (SUV Max 9) demonstrated on whole body maximum intensity projection and axial images (Image 3, hepatosplenomegaly and low-grade nodal active disease (SUV Max 3, arrows, Image 3).

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Figure 3. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

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The patient underwent treatment with CHOP chemotherapy with resolution of his subcutaneous nodules and B-symptoms. Follow-up FDG PET/CT demonstrated a good partial response (Image 4); however, the patient relapsed within 6 months of completing treatment.

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Figure 4. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]

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SPTCL was first defined as a clinical entity in 1991 as a cytotoxic T-cell lymphoma which preferentially infiltrates subcutaneous tissue; this was further refined in the 2008 WHO classification to exclude γ/δ T-cell lymphoma. [1] It is a rare lymphoma (<1% non-Hodgkin lymphoma) that may affect younger patients (median age 35 years) and has an association with autoimmune disease. Dissemination to lymph nodes and other organs on initial presentation is very unusual. [2] The lesions in this case were FDG avid and demonstrated unexpected nodal involvement. PET/CT may therefore be useful in detecting the occult extracutaneous involvement when staging SCPTL as well as being a useful tool to quantify disease burden and response to treatment.

References

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  2. References
  • 1
    Go RS,Wester SM. Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma. Cancer 2004; 101: 14041413.
  • 2
    Jaffe ES, Gaulard P, Ralfkiaer E, et al. Subcutaneous panniculitis-like T-cell lymphoma. In: SwerdlowSH, CampoE, HarrisNL, et al., editors. WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues, 4th ed: Lyon: IARC; 2008. pp 294295.