Lymphoma & related disorders

Mature T/NK cell disorders

Cutaneous / soft tissue involvement

Subcutaneous panniculitis-like T cell lymphoma



Last author update: 14 June 2023
Last staff update: 14 June 2023

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PubMed Search: Subcutaneous panniculitis-like T cell lymphoma SPTCL

Mario L. Marques-Piubelli, M.D.
Roberto N. Miranda, M.D.
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Cite this page: Marques-Piubelli ML, Torres-Cabala CA, Miranda RN. Subcutaneous panniculitis-like T cell lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBsubcutaneouspan.html. Accessed March 28th, 2024.
Definition / general
Terminology
  • T cell lymphoma involving subcutaneous tissue
ICD coding
  • ICD-10: C86.3 - subcutaneous panniculitis-like T cell lymphoma
Etiology
Clinical features
Diagnosis
  • Association of clinicopathologic features and correct interpretation of histologic findings (JAMA Dermatol 2022;158:1167, J Clin Oncol 2015;33:1216)
    • Unrelated malignant and nonmalignant confounding histopathology can masquerade as subcutaneous panniculitis-like T cell lymphoma (SPTCL)
Laboratory
Radiology description
Radiology images

Contributed by Roberto N. Miranda, M.D.
Subcutaneous uptake

Subcutaneous uptake

Prognostic factors
Case reports
  • 26 year old man with a diagnosis of panniculitis-like T cell lymphoma with HAVCR2 mutation and previous history of lupus panniculitis (Intern Med 2023;62:1537)
  • 34 year old woman with liver failure and skin lesions as main manifestation of subcutaneous panniculitis-like T cell lymphoma (Ann Transl Med 2022;10:1408)
  • 39 year old man with a diagnosis of panniculitis-like T cell lymphoma presenting as an abscess following arthropod bite (Dermatol Online J 2022;28:3)
  • 42 year old man diagnosed with panniculitis-like T cell lymphoma in complete remission after 2 cycles of romidepsin (Case Rep Oncol 2022;15:1088)
  • 47 year old woman with a diagnosis of panniculitis-like T cell lymphoma with inverted FDG uptake pattern in 18F-FDG PET (Clin Nucl Med 2023;48:186)
Treatment
  • No standard treatment
    • Immune modulatory treatments as single agents or combined (JAMA Dermatol 2022;158:1167, Surg Pathol Clin 2021;14:177)
      • Cyclosporine A, systemic steroids, bexarotene, low dose methotrexate, chlorambucil, azathioprine
      • Overall response rate (ORR): ~50%
    • Chemotherapy
      • CHOP: cyclophosphamide, doxorubicin, vincristine and prednisone
      • CHOP-like regimens
    • Radiation
      • Localized disease
    • Hematopoietic stem cell transplantation (Asia Pac J Clin Oncol 2023;19:27)
      • Refractory / relapsed or disseminated cases
Clinical images

Contributed by Roberto N. Miranda, M.D.
Subcutaneous lesions

Subcutaneous lesions

Subcutaneous plaque

Subcutaneous plaque

Microscopic (histologic) description
  • Skin involvement (JAMA Dermatol 2022;158:1167, Surg Pathol Clin 2021;14:177, Histopathology 2013;62:1057, Blood Adv 2021;5:3919, J Clin Pathol 2015;68:954)
    • Epidermis and dermis are usually spared
      • Interface dermatitis, plasma cell aggregates, follicular plugging or mucin deposits may occur
      • Minimal involvement by lymphoma cells of deep dermis can be observed
    • Lobular infiltrate
      • Variable size (small to intermediate) lymphocytes with hyperchromatic and irregular nuclei and scant pale / clear cytoplasm
        • Subset of cases with large cell or pleomorphic morphology
      • Variable density of atypical lymphocytes involving lobules and sparing septa
        • Rimming adipocytes or lipid vacuoles
      • Background
        • Scattered reactive plasma cells and neutrophils
        • Absence of plasmacytoid dendritic cells aggregates (> 10 cells)
        • Reactive histiocytes, sometimes dense and forming granulomas or lipogranulomas
    • Necrosis may vary from mild to extensive
    • Karyorrhexis, mitotic figures and hemorrhage can be present
    • Hemophagocytosis may be detected in skin or other tissues (e.g., bone marrow)
  • Bone marrow is not involved by overt lymphoma cells but aggregates of small lymphocytes can be detected around adipocytes
Microscopic (histologic) images

Contributed by Roberto N. Miranda, M.D.
Deep dermis and subcutaneous involvement

Deep dermis and subcutaneous involvement

Epidermis and reticular dermis sparing

Epidermis and reticular dermis sparing

Lobular distribution Lobular distribution

Lobular distribution

Acellular hyaline change

Acellular hyaline change

Adipocyte rimming

Adipocyte rimming


CD8 positivity

CD8 positivity

CD4 negativity

CD4 negativity

TIA1 positivity

TIA1 positivity

βF1 (TCR α / β) positivity

βF1 (TCRα / β) positivity

TIM-3 positivity

TIM-3 positivity

Molecular / cytogenetics description
Sample pathology report
  • Skin, right thigh lesion, punch:
    • Subcutaneous panniculitis-like T cell lymphoma (see comment)
    • Comment: According to clinical notes, this is a 37 year old woman with a history of a solitary, tender and painless lesion in distal thigh (3 cm in the greater axis) that the patient noted wax and wane for several months.
    • Histologic sections of the specimen designated right thigh lesion demonstrate a punch skin biopsy that includes epidermis, dermis and subcutaneous adipose tissue. Epidermis is unremarkable. Deep dermis and subcutaneous adipose tissue show dense small to medium sized lymphoid cells, neutrophils and histiocytes. The lymphocytes show irregular nuclear contours, hyperchromatic chromatin and scant pale cytoplasm. The distribution is mainly involving the lobules with fat rimming and sparing the septa. Mitotic figures are frequent and karyorrhectic debris is present.
    • Immunohistochemical studies showed the atypical lymphocytes are positive for CD2, CD3, CD8, CD43, TIA1, perforin and TCRα-β (βF1). The abnormal lymphocytes are negative for CD4, CD7, CD30, CD56 and TCRγ / δ. Ki67 index is ~70% (variable, ranging from 40 - 100%). Epstein-Barr virus encoded small RNAs (EBER) in situ hybridization is negative.
    • Polymerase chain reaction to assess for clonality of T cell receptors gamma and delta chains clonal TCRβ (TRB) and polyclonal TCRγ (TRG) and TCRδ (TRD).
Differential diagnosis
Board review style question #1

Which of the following is true about subcutaneous panniculitis-like T cell lymphoma?

  1. Frequently presents with an aggressive clinical behavior
  2. Localized or multiple subcutaneous nodules are the most common clinical presentation
  3. Lymph nodes are usually involved at initial diagnosis
  4. Somatic mutations of HAVCR2 are common
Board review style answer #1
B. Localized or multiple subcutaneous nodules are the most common clinical presentation. Subcutaneous panniculitis-like T cell lymphoma usually presents as localized or multiple subcutaneous nodules, usually in the legs, arms or trunk and rarely with lymph node involvement. Germline mutations of HAVCR2 are classically described as a predisposing factor in up to 85% of patients.

Comment Here

Reference: Subcutaneous panniculitis-like T cell lymphoma
Board review style question #2

Which of the following immunophenotypic pattern corresponds to subcutaneous panniculitis-like T cell lymphoma?

  1. CD3-, CD4-, CD8+, TIA1-, granzyme B+, TCRγδ+
  2. CD3-, CD4+, CD8+, TIA1-, granzyme B-, TCRγδ+
  3. CD3+, CD4-, CD8+, TIA1+, granzyme B+, TCRαβ+
  4. CD3+, CD4+, CD8-, TIA1+, granzyme B-, TCRαβ+
Board review style answer #2
C. CD3+, CD4-, CD8+, TIA1+, granzyme B+, TCRαβ+. The typical immunophenotype is CD3+, CD4-, CD8+, TIA1+, granzyme B+ and TCRαβ+. The picture shows positivity in the neoplastic cells and highlights rimming of adipocytes.

Comment Here

Reference: Subcutaneous panniculitis-like T cell lymphoma
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