Definition
Angiocentric and angiodestructive lymphoproliferative disorder predominantly composed of large atypical 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 B cells
Must be predominiant population
May form confluent sheets
Prominent population of reactive T cells Extensive 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
If atypical cells are rare or only occasional, designate as Grade I or Low Grade Lymphomatoid Granulomatosis
Yasodha Natkunam MD PhD
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
Small cells
T cell phenotype
May be predominant population
Genetic analysis
EBV EBER positive by in situ hybridization
Differential Diagnosis
Both contain EBV+ B cells
Both are angiocentric
Both may involve ENT sites and lung, are angiocentric, necrotizing and are EBV+
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
Poor prognosis
Grading / Staging / Report
Grade III or High Grade in the following Lymphomatoid Granulomatosis grading systems corresponds to lymphoma
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
Predomiant population of large atypical cells
Extensive necrosis
Two tier grading system favored by some over original three tier system
Low grade
Few large atypical cells
Few mitotic figures
Little necrosis
Some cases spontaneously resolve
High grade
Predomiant population of large atypical cells
Extensive necrosis
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:
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
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.
