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    Subcutaneous Panniculitis-like T Cell Lymphoma

    Warning: This page is under construction and has not undergone final review.

    Definition

    • Predominantly subcutaneous lymphoma of cytotoxic T phenotype

    Diagnostic Criteria

    • Subcutaneous nodules
      • Diffuse infiltrate
      • Does not spare septa
      • Dermis and epidermis rarely involved
      • Vascular invasion may me seen
    • Atypical cells vary from small, round to large and hyperchromatic
      • Cytoplasm moderate, pale
    • Atypical cells may form rings around necrotic fat cells
    • Necrosis and karyorrhexis common
      • Foamy histiocytes may be present in areas of fat destruction
    • Cytotoxic T cell phenotype
      • Most cases have alpha beta T cell receptors
    • gamma delta variant
      • More frequently also involve dermis and epidermis
      • May be CD8 and CD4 negative and CD56+
      • May be associated with pre-existing immunosuppression
      • May represent 25% of total
      • May instead be part of spectrum of mucosal/cutaneous gamma delta T cell lymphoma


    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Initial posting : pending completion

    Supplemental studies

    Immunohistology and Flow

    • Cytotoxic T cell phenotype
      • T lineage markers positive
      • CD8+, CD4-
      • Cytotoxic protein markers positive
        • TIA1, granzyme B, perforin
      • CD56 negative to rare
    • CD30 25% positive
    • EBV negative
    • gamma delta variant may be CD8 and CD4 negative and CD56 positive

    Genetic analysis

    • Predominantly alpha beta T cell receptors
    • 25% may have gamma delta receptors

    Differential Diagnosis

    Subcutaneous Panniculitis-Like T Cell Lymphoma Inflammatory Panniculitis
    Cytologically atypical population present Lacks cytologic atypia
    Predominant CD8+ population Mixed CD4+ and CD8+ infiltrate
    Cytotoxic protein positive population Infrequent cytotoxic protein positive cells
    Early foci of subcutaneous panniculitis-like T cell lymphoma may be indistinguishable form inflammatory panniculitis

    Clinical

    • Wide age range
    • Multiple subcutaneous nodules
      • 0.5 to several cm
    • Lymphadenopathy infrequent
      • Usually only late in disease
    • Hemophagocytic syndrome common
      • Pancytopenia
      • Fever
      • Hepatosplenomegaly
    • Poor prognosis
    • gamma delta variant associated with pre-existing immunosuppression and chronic antigenic exposure

    Grading / Staging / Report

    • Grading not applicable

    Ann Arbor Staging System

    • Stage I
      • I if involvement of a single lymph node region
      • IE if involvement of a single extralymphatic organ or site
    • Stage II
      • II if two or more lymph node regions on same side of diaphragm
      • IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
    • Stage III
      • III if Involvement of lymph node regions on both sides of the diphragm
      • IIIS if spleen involved
      • IIIE if extralymphatic site involved
    • Stage IV
      • Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
    • Systemic Symptoms in 6 months preceding admission
      • Fever, night sweats, 10% weight loss
      • A = absent
      • B = present
    • Extranodal sites are also designated
      • M+ = marrow
      • L+ = lung
      • H+ = liver
      • P+ = pleura
      • O+ = bone
      • D+ = skin and subcutaneous tissue
    • Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

     

    Lists

    Mature T and NK cell neoplasms (WHO classification)

    Primary cutaneous and subcutaneous T and NK lymphomas

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Kumar S, Krenacs L, Medeiros J, Elenitoba-Johnson KS, Greiner TC, Sorbara L, Kingma DW, Raffeld M, Jaffe ES. Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes. Hum Pathol 1998 Apr;29(4):397-403
    • Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, Wilson DM, Przybylski GK, Lister J, Wasik MA, Swerdlow SH. Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. Am J Surg Pathol 1998 Jul;22(7):881-93
    • Arnulf B, Copie-Bergman C, Delfau-Larue MH, Lavergne-Slove A, Bosq J, Wechsler J, Wassef M, Matuchansky C, Epardeau B, Stern M, Bagot M, Reyes F, Gaulard P. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood 1998 Mar 1;91(5):1723-31
    • Gonzalez CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol 1991 Jan;15(1):17-27
    • Felgar RE, Macon WR, Kinney MC, Roberts S, Pasha T, Salhany KE. TIA-1 expression in lymphoid neoplasms. Identification of subsets with cytotoxic T lymphocyte or natural killer cell differentiation. Am J Pathol 1997 Jun;150(6):1893-900
    • Jaffe ES, Krenacs L, Kumar S, Kingma DW, Raffeld M. Extranodal peripheral T-cell and NK-cell neoplasms. Am J Clin Pathol 1999 Jan;111(1 Suppl 1):S46-55
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