Case 37 - Discussion

Uploaded: 2007-10-28, Updated: 2008-01-08

Hodgkin Lymphoma, Nodular Sclerosing Type

The Key Features

  • Most common subtype in HL (~70%); most common location, mediastinum (80%);

  • Nodular growth pattern with broad fibrous collagen bands, and lacunar cells;

  • CD30+, CD15+, BSAP+, CD45−, EMA−; EBV/LMP1+ in 10-40% cases.

CLINICAL FEATURES

  • Account for ~ 70% of Classic HL, median age 28 years, M=F;

  • Location: mediastinal 80%, spleen/lung 10%, bone marrow 3%;

  • Most patient present with Stage II disease, 40% with B-symptoms.

MICROSCOPIC FINDINGS

  • Nodular growth pattern with broad fibrous collagen bands surrounding at least one nodule;

  • The tumor contains a highly variable number of HRS cells, small lymphocytes and other reactive inflammatory cells;

  • HRS cells tend to have more lobated nuclei with smaller lobes and less prominent nucleoli. Lacunar cells due to retraction of the cytoplasm;

  • Eosinophils are often numerous.

SUBTYPES/VARIANT

  • Syncitial (sarcomatous, monomorphic) variant: lacunar cells may form prominent cellular aggregates with necrotic areas in the nodules.

DIFFERENTIAL DIAGNOSES

  • Use of Immunophenotypic Studies in the Differential Diagnosis of HL

      ALK CD30 EMA CD15 CD3 LCA BSAP TIA1 Clusterin
    ALCL + + + ± + + +
    PC-ALCL + ± + ±
    HD + + +
    PTCL ± + + ±

    DLBCL

    ±

    ±

    +

    +

    ALCL, anaplastic large cell lymphoma; LCA, leukocyte common antigen (CD45); TIA-1, T-cell intracellular antigen; Hodgkin’s, Hodgkin’s lymphoma;

    PC-ALCL, primary cutaneous ALCL; PTCL, peripheral T-cell lymphoma, unspecified; DLBCL, diffuse large B-cell lymphoma.

IMMUNOHISTOCHEMISTRY AND SPECIAL STAINS

  • CD30+ in almost all cases, CD15+ in 75-85% cases;

  • EBV/LMP1+ in 10-40% cases, lowest incidence of all HL;

  • BSAP+ in ~90% cases (B-cell specific activator protein, product of PAX5 gene)

  • CD20+ in 40% cases with variable intensity;

  • CD45−, EMA−, OCT2 and BOB1−.

CYTOGENETIC STUDIES

  • Monoclonal immunoglobulin gene rearrangement in >98% of cases, rarely T-cell receptor gene rearrangement.

TREATMENT AND PROGNOSIS

  • Slightly better than mixed cellularity or lymphocyte depleted subtype;

REFERENCES

  • Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. Lyon (France): IARC Press; 2001.