Lymphomatoid granulomatosis (LYG) is an EBV-positive B-cell lymphoproliferative disease characterized by angiodestructive (vessel-destroying), angiocentric (vessel-centered) growth pattern. Classified as EBV-positive B-cell lymphoproliferative disorder in WHO classification. The lung is the primary organ of involvement (>90%); lymph node involvement may accompany but isolated nodal involvement is rare. Brain (30%), kidney (30%), liver, and skin may also be involved. Typically seen in immunosuppressed patients (HIV, transplant) or more rarely in immunocompetent individuals. Histological grade (1-3) is determined by the number of EBV-positive large B cells; Grade 3 is equivalent to diffuse large B-cell lymphoma. On imaging, bilateral pulmonary nodules/masses + lymphadenopathy pattern is characteristic.
Age Range
30-70
Peak Age
50
Gender
Male predominant
Prevalence
Rare
LYG is a lymphoproliferative disease characterized by angiocentric and angiodestructive proliferation of EBV-positive B cells. In pathogenesis, immunosuppression allows uncontrolled proliferation of EBV-infected B cells. These B cells infiltrate vessel walls (angiocentric growth) and destroy vessel walls (angiodestruction), leading to vascular necrosis and coagulative infarcts. Reactive T cells (CD3+, CD4+) constitute the majority of the lesion composition and form the granulomatous inflammatory background. The number of EBV-positive large B cells determines histological grade: rare in Grade 1, moderate in Grade 2, numerous atypical B cells in Grade 3. The lung is the primary site because the rich pulmonary vascular bed provides a suitable environment for angiocentric growth. On imaging, the lower lobe-predominant distribution and cavitation tendency of pulmonary nodules/masses reflects coagulative necrosis triggered by angiodestruction. Similar angiocentric and angiodestructive patterns are seen in lymph node involvement — necrosis or heterogeneous enhancement on contrast CT reflects this vascular damage. Diffusion restriction on DWI reflects cellular density of lymphomatous infiltrate.
In immunosuppression setting, bilateral lower lobe perivascular-distributed cavitating pulmonary nodules/masses with concurrent lymphadenopathy is highly characteristic of LYG.
On contrast-enhanced CT, bilateral pulmonary nodules/masses are seen — lower lobe predominant, perivascular distribution, may show cavitation. Concurrent mediastinal and hilar lymphadenopathy may accompany. Nodes show moderate to prominent enhancement and may contain necrotic areas (angiodestruction). Ground-glass halo may be seen.
Report Sentence
CT demonstrates multiple nodules/masses with perivascular distribution showing cavitation in bilateral lower lobes and concurrent mediastinal lymphadenopathy; findings are consistent with lymphomatoid granulomatosis.
On B-mode US, enlarged, hypoechoic lymph nodes are seen. Central necrosis (hypoechoic-anechoic area) may accompany in advanced grade disease. Hilum is generally lost.
Report Sentence
On US, enlarged, hypoechoic lymph nodes with loss of hilum are noted.
Mixed vascularity pattern on Doppler — both hilar and peripheral flow signals. Avascularity may be seen in some areas due to angiodestruction.
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Doppler demonstrates mixed vascularity pattern in enlarged lymph nodes with focal avascular areas consistent with angiodestruction.
On DWI, enlarged lymph nodes and pulmonary lesions show diffusion restriction. ADC values in 0.7-1.1 × 10⁻³ mm²/s range. Necrotic areas may show higher ADC values — heterogeneous ADC map.
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DWI demonstrates diffusion restriction in lymph nodes and pulmonary lesions with higher ADC values in necrotic areas.
On FDG PET-CT, pulmonary lesions and enlarged lymph nodes demonstrate intense FDG uptake (SUVmax 5-20+, grade-dependent). Most intense uptake in Grade 3. Brain, kidney, and skin involvements may also be detected. In cavitating lesions, peripheral rim uptake (viable tumor) and central low uptake (necrosis) may be seen.
Report Sentence
FDG PET-CT demonstrates intense FDG uptake in bilateral pulmonary lesions and mediastinal/hilar lymph nodes; findings are consistent with lymphomatoid granulomatosis.
On MRI T2, lymph nodes show heterogeneous signal — viable tumoral area hyperintense, necrotic area variable (hypointense in coagulative necrosis, hyperintense in liquefactive necrosis). Brain involvement may show periventricular white matter lesions.
Report Sentence
On MRI T2, lymph nodes demonstrate heterogeneous signal reflecting coexistence of necrotic and viable tumoral areas.
Criteria
Rare EBV+ large B cells (<5/HPF), polymorphic reactive T-cell predominant infiltrate, minimal necrosis.
Distinct Features
Smaller nodules on imaging, cavitation rare. Lower FDG uptake on PET-CT. Good response to interferon-alpha therapy.
Criteria
Moderate number of EBV+ large B cells (5-20/HPF), prominent angiodestruction, early necrosis.
Distinct Features
Cavitation more common. Moderate uptake on PET-CT. Chemotherapy may be needed.
Criteria
Numerous EBV+ large B cells (>50/HPF), sheet-like proliferation, extensive necrosis. Considered equivalent to DLBCL.
Distinct Features
Large masses, widespread cavitation and necrosis. Very intense uptake on PET-CT. R-CHOP chemotherapy required.
Distinguishing Feature
In primary pulmonary lymphoma single dominant mass usually predominates; in LYG bilateral multiple nodules/masses show lower lobe predominant distribution. Cavitation more common in LYG. EBV association and angiocentric pattern are specific to LYG.
Distinguishing Feature
In sarcoidosis perilymphatic nodules and bilateral hilar LAP predominate, cavitation rare; in LYG perivascular nodules and cavitation predominate. Sarcoidosis granulomas are noncaseating, LYG shows angiodestructive necrosis.
Distinguishing Feature
In TB cavitary lesion is generally upper lobe predominant; in LYG lower lobe predominant. Conglomerate necrotic LAP common in TB; LAP more modest and pulmonary involvement more dominant in LYG.
Distinguishing Feature
Extranodal NK/T-cell lymphoma is also EBV-associated and angiodestructive but tends to involve nasal/midface region; in LYG lung is primary site. Both show angiocentric pattern but cell origin differs (NK/T vs. B-cell).
Urgency
urgentManagement
medicalBiopsy
NeededFollow-up
3-monthLYG diagnosis is made by lung or lymph node biopsy — EBV-EBER ISH and CD20 immunohistochemistry are mandatory. Interferon-alpha or rituximab preferred for Grade 1-2. R-CHOP chemotherapy required for Grade 3 (DLBCL equivalent). Reduction of immunosuppression (if possible) is fundamental. High-dose methotrexate or whole brain radiotherapy added in CNS involvement. Prognosis depends on grade: good for Grade 1-2 (5-year 60-70%), poor for Grade 3 (30-40%).
Treatment of lymphomatoid granulomatosis depends on grade. Interferon-alpha and rituximab are used for Grades 1-2. Grade 3 (DLBCL transformation) requires aggressive chemotherapy (R-CHOP or similar). Reduction of immunosuppression (when possible) is an important component of treatment. Untreated mortality is 60-90%. CNS involvement indicates poor prognosis.