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Surgical Pathology Criteria
http://surgpathcriteria.stanford.edu/

Lymphomatoid Granulomatosis

Definition

  • Angiocentric and angiodestructive lymphoproliferative disorder containing EBV positive B cells

Alternate/Historical Names

  • Polymorphic reticulosis
  • Angiocentric immunoproliferative lesion
  • Malignant angiitis
  • Malignant granulomatosis
  • Angiocentric lymphoma

Diagnostic Criteria

  • Angiocentric and angiodestructive lymphoid infiltrate
  • EBV positive large atypical B cells
    • Range from rare to predominiant
  • Prominent population of small reactive T cells
  • Necrosis
  • Virtually all cases involve lung
    • Multiple pulmonary nodules
    • Skin involved in nearly half of cases
      • Dermal and subcutaneous nodules show similar features to lung
      • May show nonspecific plaque like dermal infiltrate
    • CNS and kidney also frequently involved
  • High grade lesions with preponderance of large atypical B cells are considered diffuse large B cell lymphoma, lymphomatoid granulomatosis variant

Yasodha Natkunam MD PhD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting:: May 1, 2006

Supplemental studies

Immunohistology

  • Large atypical cells
    • B lineage (CD20, CD79a+)
    • EBV LMP positive but much less reliable than in situ hybridization
    • CD30 variable
    • CD15 negative
  • Small cells
    • T cell phenotype
    • May be predominant population

Genetic analysis

  • EBV EBER positive by in situ hybridization
    • Required for diagnosis
  • Immunoglobulin gene rearrangements
    • Variable in grade I lesions
    • Present in grade II or III lesions

Differential Diagnosis

Post-transplant Lymphoproliferative Disorder Lymphomatoid Granulomatosis or Lymphomatoid Granulomatosis Lymphoma
T cell poor infiltrate T cell rich infiltrate
Not angiocentric Angiocentric
Definitionally post-transplant Rare cases post-transplant
Both contain EBV+ B cells
The distinction may sometimes be arbitrary

Wegener Granulomatosis Lymphomatoid Granulomatosis or Lymphomatoid Granulomatosis Lymphoma
No prominent lymphoid infiltrate Prominent lymphoid infiltrate
Neutrophilic microabscesses No abscesses
EBV negative EBV+ B cell population
Both are angiocentric

Extranodal NK/T Cell Lymphoma, Nasal Type Lymphomatoid Granulomatosis or Lymphomatoid Granulomatosis Lymphoma
Atypical cells are CD56+ cytotoxic T cells Atypical cells are B cells
Both may involve ENT sites and lung, are angiocentric, necrotizing and are EBV+

Hodgkin Lymphoma Lymphomatoid Granulomatosis or Lymphomatoid Granulomatosis Lymphoma
CD15 80% CD15 negative
CD20 or CD79a 20% CD20 or CD79a 100%
Background with neutrophils, eosinophils, plasma cells Background of small T cells
Rare in lung and skin Typically involves lung and skin
Both may be necrotizing, CD30+ and EBV+

Clinical

  • Virtually all cases involve lung at some point in disease
    • Multiple lung nodules common
    • Skin involved in 40-50% of cases
    • CNS, kidney, GI tract, head and neck also frequently involved
  • Lymphoid organs involved only in late disease
  • Most common in forties but wide age range
  • Some cases reported associated with HIV or organ transplantation immunosuppression

Grading / Staging / Report

Three tier system

  • Grade I
    • No large atypical cells
    • Little necrosis
    • EBV in situ+ cells rare
    • Some cases spontaneously resolve
  • Grade II
    • Occasional large atypical cells
    • Moderate necrosis
    • EBV in situ+ cells 5-20/hpf
    • Some cases spontaneously resolve
  • Grade III

Two tier grading system favored by some over original three tier system

Ann Arbor Staging System

  • Stage IV by definition
  • 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:

  • Results of supplementary studies if performed
  • Relationship to other specimens from the same patient
  • Information relevant to staging if available

 

Lists

Types and variants of large B cell lymphoma

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
  • Colby TV, Koss MN, Travis WD. Tumors of the Lower Respiratory Tract, Atlas of Tumor Pathology, AFIP Tird Series, Fascicle 13, 1995
  • Beaty MW, Toro J, Sorbara L, Stern JB, Pittaluga S, Raffeld M, Wilson WH, Jaffe ES. Cutaneous lymphomatoid granulomatosis: correlation of clinical and biologic features. Am J Surg Pathol. 2001 Sep;25(9):1111-20.
  • Jaffe ES, Wilson WH. Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. Cancer Surv. 1997;30:233-48.
  • Guinee D Jr, Jaffe E, Kingma D, Fishback N, Wallberg K, Krishnan J, Frizzera G, Travis W, Koss M. Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T-cell component and vasculitis. Am J Surg Pathol. 1994 Aug;18(8):753-64.
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