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: 26 April 2024

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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 May 19th, 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
  • Lupus erythematosus panniculitis (Am J Dermatopathol 2010;32:24, Asia Pac J Clin Oncol 2023;19:27, Histopathology 2013;62:1057, Am J Surg Pathol 2016;40:745):
    • F > M
    • Multiple or single lipoatrophy, erythema or subcutaneous nodules
    • Extremities (upper arms) > face > trunk
    • Epidermal and dermal changes
      • Lymphocytic lobular / septal panniculitis with mild or no cytological atypia
      • Intradermal mucin, hyaline lipomembranous changes, mild vasculitis, reactive germinal centers and plasma cells
      • Aggregates of CD123+ plasmacytoid dendritic cells
    • Absence of atypical T cells
    • Low Ki67 proliferation index (~< 10%)
    • Usually polyclonal TCRγ gene rearrangement
      • Rare cases with monoclonal peak
  • Cytophagic histiocytic panniculitis (Ital J Pediatr 2014;40:17):
    • Infiltration of subcutaneous by T cells and phagocytic histiocytes
    • Isolated in skin or associated with other malignancies
      • Skin lesions may remain
    • No evidence of monoclonal rearrangements of TRB or TRG gene
  • Cold panniculitis (J Clin Pathol 2015;68:954):
    • Physical panniculitis
    • Appropriate clinical history
    • More common in pediatric patients
      • Face (chin and cheeks)
        • Different chemical composition of the adipose tissue
    • Adults: usually after chronic use of ice pack or environmental exposure
    • Lymphoid infiltrate of lobular distribution
      • Increased density along the dermal - subcutaneous transition, dermal perivascular and periadnexial regions
      • Dermal mucin and interface dermatitis may be present
    • No evidence of monoclonal TCB or TRG gene rearrangements
  • Lyme disease (J Clin Pathol 2015;68:954):
    • Rare cases presenting as lobular panniculitis
      • Dense lymphocytic infiltrate in subcutaneous tissue with scattered plasma cells
        • Small to medium size and moderate atypia
      • No extension to overlying dermis or epidermis
    • Predominance of CD8+ cells
    • Identification of Borrelia burgdorferi DNA by PCR or positive serology
  • Atypical lymphocytic lobular panniculitis (Am J Surg Pathol 2015;39:206):
    • Indolent condition with spontaneous regression
      • Controversial entity
    • Lobular infiltration of clonal, small to intermediate sized T cells and mild nuclear atypia
      • Usually less dense infiltrate
      • Adipocyte rimming is less frequent
      • Hemorrhage and karyorrhexis are usually absent
    • Good prognosis
  • Primary cutaneous gamma delta T cell lymphoma (Surg Pathol Clin 2021;14:177):
    • Plaques, nodules and tumors with rapid progression and ulceration
    • Involvement of epidermis, dermis and subcutaneous tissue
      • Medium to large size lymphocytes with irregular nuclear contours, clumped chromatin and eosinophilic cytoplasm
    • CD3+, CD7+, CD5-, CD4-, CD8-
    • TIA1+, granzyme B+, perforin+
    • TCRδ+, TCRγ+, TCRβ (βF1)-
    • Poor prognosis
  • Primary cutaneous CD30+ T cell lymphoproliferative disorders (Surg Pathol Clin 2021;14:177):
    • Multiple papules or solitary lesion
    • Monomorphic lymphoid infiltrate predominantly in dermis
      • May occur in hypodermis
    • CD3+, CD4 variable, CD8 variable
    • CD30 strong and diffuse
    • Indolent behavior
  • Primary cutaneous peripheral T cell lymphoma, not otherwise specified (J Eur Acad Dermatol Venereol 2021;35:658):
    • Solitary or disseminated papulonodular or tumor lesions with rapid growth and necrosis
    • Nodular or diffuse infiltrate in dermis and subcutaneous tissue
      • Variable morphology, usually large, pleomorphic cells
    • Epidermotropism absent or focal
    • CD3+ / CD4- / CD8- or CD3+ / CD4+ / CD8-
      • CD8+ may also occur
    • Poor prognosis
      • 3 year overall survival: ~60%
      • 5 year overall survival: ~50%
  • Acute myeloid leukemia involving skin (An Bras Dermatol 2018;93:216):
    • Variable clinical presentation
    • Diffuse infiltration of dermis and hypodermis
      • Neoplastic cells can rim adipocytes
  • Extranodal NK / T cell lymphoma involving skin (Surg Pathol Clin 2021;14:177):
    • More common in adults
    • Ulcerated and necrotic tumors
    • Small to large atypical lymphocytes in an inflammatory and necrotic background
    • Angiocentricity and angioinvasion
    • Positive: CD2, CD56, TIA1, granzyme B, perforin, EBER
    • Negative: CD3ε, CD4, CD5, CD8
    • Poor prognosis
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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