types of lymphomas, imaging modalities, imaging features of lymphomas, treatment follow up and pulmonary lymphoproliferative disorders and their imaging findings.

1. Imaging Lymphoma & lymphoproliferative
disease of lung
By. Irko worku(RR)
5/1/2023 1

2. outlines
• part one-
 Introduction to lymphoma
 Hodgkin lymphoma
 Non-hodgkin’s lymphoma
• Part 2- pulmonary lymphoproliferative diseases
5/1/2023 2

3. Part 1-Lymphoma
Introduction
• Lymphoma is a malignancy arising from lymphocytes or lymphoblasts.
• Lymphoma can be restricted to the lymphatic system or can arise as extranodal
disease.
• 85% of lymphomas are B-cell and 15% T-cell origin.
• The WHO classification of hematopoietic and lymphoid neoplasms of more than
50 entities into five groups.
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4. five groups of WHO classification
1. mature B-cell neoplasms,
2. mature Tcell and NK-cell (natural killer)
neoplasms,
3. Hodgkin’s lymphoma (HL),
4. histiocytic and dendritic cell neoplasms, and
5. posttransplantation lymphoproliferative
disorders (PTLDs)
5/1/2023 4

5. risk factors for lymphomas and lympho proliferative
disease
1. Immunosuppression from any cause is a well known
risk factor.
2. Multiple infectious agents(HIV,HBV,EBV,HTCLV)
3. Genetic mutations
• for simplicity, lymphomas are considered in two primary
groups,
1.HL and
2. non-Hodgkin’s lymphoma (NHL)
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6. IMAGING MODALITIES
• ULTRASOUND
• RADIOGRAPHY
• CT SCAN
• FDG PET , PET/CT
SCAN
• MRI
• PRIMARY
DIAGNOSIS,CHARACTERIZATION
OF THE TUMOR
• TO KNOW TUMOR BURDEN AND
PLAN MODE OF TREATMENT
• FOR FOLLOW UP
• FOR DETECT RECURRENCES.
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7. STAGING OF LYMPHOMA.
• The Ann Arbor staging system and “Cotswolds modifications”
• “Lugano Classification” – use CT in staging and the use of PET as
routine in the initial evaluation and as a baseline for
assessment of treatment response in most lymphomas.
• PET is used in HL and FDG-avid NHL
• PET improves the accuracy of staging compared with CT for both
nodal and extranodal sites.
• It leads to change in stage in 10% to 30% of patients, more often
an upstaging.
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8. LUGANO STAGING CLASSIFICATION
• Patients are now more simply categorized as having
“limited disease” or “advanced disease”.
• “limited disease” (previously Ann Arbor stage I or II) or
• “advanced disease” (previously Ann Arbor stage III or IV)
• “bulky” disease - a mass greater than 10 cm or 1/3 of
the transthoracic diameter
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9. LYMPHOMA TREATMENT RESPONSE EVALUATION
• CT staging remains appropriate for lymphoma types with low or variable FDG
uptake.
Disease burden is defined as the sum of
• the product of lymph node or mass diameters (two perpendicular diameters
for each node), for up to six measurable nodes or masses or extra nodal sites.
• Four categories of lymph node response using CT have been defined:
1. Complete radiologic response, all nodes ≤1.5 cm in longest
diameter or disappearance of all CT findings of lymphoma;
2. partial response, 50% or greater decrease in disease burden;
3. stable disease, less than 50% decrease in disease burden;
4. progressive disease, new or increased adenopathy or new
extranodal lymphoma.
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10. Response assessment using FDG-PET/CT
• For FDG-avid lymphomas.
• based on metabolic activity as determined by FDG uptake.
• visual assessment of FDG uptake as compared to baseline
(five-point scale).
 (1) no FDG uptake above background,
 (2) FDG uptake ≤ mediastinum,
 (3) FDG uptake > mediastinum ≤ liver,
 (4) FDG uptake moderately > liver,
 (5) FDG uptake markedly > liver and/or new lesions.
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11. Response criteria for PET
• complete metabolic response, score of 1, 2, or 3 in with
or without a residual mass;
• partial metabolic response, a score of 4 or 5 with
reduced uptake compared with baseline and residual
mass(es) of any size;
• no metabolic response, a score of 4 or 5 with no obvious
change in FDG uptake; and
• progressive disease, a score 4 or 5 in any lesion with an
increase in intensity of uptake from baseline and/or new
FDG-avid foci consistent with lymphoma.
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12. Classic Hodgkin Lymphoma
• peak incidence is in the third
and eighth decades
• 10% of all lymphomas
• Intrathoracic HL usually is
associated with disease
elsewhere; cervical lymph
nodes are commonly involved.
• HL is characterized histologically
by the presence of Reed-
Sternberg cells.
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13. Con’t
• HL has a predilection for thoracic
involvement.
• Up to 85% of patients with HD
have thoracic involvement at the
time of diagnosis; nearly all of
them have mediastinal lymph
node enlargement.
• HL most often involves superior
mediastinal.
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14. MULTIPLE LN INVOLVEMENT
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15. Lymph Node Involvement in HL
• Multiple node groups -85%,
• single node group can be
seen in only 15% of cases
with node involvement.
• Anterior (prevascular)
lymph nodes are most often
involved as a single group
and this appearance usually
indicates the presence of
nodular sclerosis HD
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16. PATTERN OF ABNORMAL LYMPH NODES IN HL
abnormal lymph nodes may appear:
well defined and discrete
may appear matted
diffuse mediastinal infiltration(individual
lymph nodes being invisible).
homogeneous soft tissue attenuation.
low attenuation or necrosis(10%).
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17. Lymphoma with necrosis
• A: large bilateral mediastinal
mass and right pleural effusion,
• B: Contrast-enhanced CT scan
shows an anterior mediastinal
mass containing an area of low
attenuation (arrows).
• This finding is seen in 10% to
20% of patients with HL.
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18. Con’t
• In 30% to 40% of cases of HL involve
thymus but may be difficult to
distinguish from an anterior
mediastinal lymph node mass unless
the normal thymic shape is
preserved.
• In the presence of thymic
involvement, a visible mediastinal
mass can project to both sides of the
mediastinum.
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19. Con’t
• HL is believed to be unifocal in origin, spreading to
involve contiguous lymph nodes.
• It is unusual for HL to skip lymph node groups, and
• if nodes contiguous with the mediastinum, such as
the lower neck or upper abdomen, are not involved
by HL, it usually is not necessary to scan more distant
regions, such as the pelvis.
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20. HL Lung Involvement
• 10% of patients at the time of presentation.
• It is almost always associated with mediastinal (and
usually ipsilateral hilar) adenopathy.
• lung disease is uncommon in the absence of
radiographically demonstrable lymph node
enlargement.
• however, lung recurrence can be seen without node
enlargement in patients with prior mediastinal radiation.
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21. LUNG INVOLVEMENT IN HL
1. NODULES
2. CONSOLIDATION
3. AIRBRONCHOGRAM
4. CAVITATION
5. PLEURAL EFFUSION
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22. LUNG INVOLVEMENT…
• A variety of manifestations of lung
involvement
• isolated single or multiple lung
nodules, masses, or areas of
consolidation.
• Nodules/mass cavitate, with thick or
thin walls.
• Peripheral, subpleural masses are
relatively common.
• thickening of the
peribronchovascular interstitium.
• Endobronchial HL.
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23. CON’T
• Lung involvement in HL in
the same patient
• B: Large lobulated nodules
are visible in the left lung.
• Some are associated with
small bronchi or contain
air bronchograms (arrow).
Extensive mediastinal and
hilar lymph node
enlargement is present.
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24. LUNG INVOLVEMENT IN RECURRENT HL
• Lung involvement in
recurrent HL. Poorly defined
nodules and areas of
consolidation are visible.
• The large area of
consolidation on the right
contains a number of air
bronchograms.
• There is no obvious lymph
node enlargement.
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25. Con’t
• Cure is the goal and is achievable in the large majority of
cases.
• On CT, reduction of tumor bulk is always seen in patients
with adequately treated tumor.
• complete resolution of lymph node masses -complete
response.
• residual mediastinal mass or lymph node enlargement
often is seen in cured patients following
treatment.(treated nodular sclerosis HL)
• residual masses represent fibrous tissue and seen in
• 88% HL and 40% of patients with NHL.
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26. Residual mediastinal mass or lymph node
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27. RECURRENCE
• Recurrent HD does not commonly involve previously
irradiated (i.e., in field) intrathoracic lymph nodes.
• Common sites of recurrent HD include the upper
mediastinum and lung, both seen in about half of cases
with recurrence.
• Lymph node recurrence also may involve paracardiac
lymph nodes, which usually are excluded from the
treatment field because of their close relation to the
heart (to avoid radiation pericarditis)
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28. NON-HODGKIN’S LYMPHOMA
• refers to a diverse
group of neoplasms,
varying in histology,
clinical presentation,
radiologic findings,
course, and prognosis
• Two groups:
 B CELL-85%
 T CELL
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29. Various types of NHL
B CELL
• Diffuse Large B-cell Lymphoma
• Follicular Lymphoma
• Mantle Cell Lymphoma
• Marginal Zone B-cell Lymphoma and MALT Lymphoma
T-CELL AND NK-CELL NHL
• T-cell lymphomas make up less than 15% of NHLs. There are
many types of T-cell lymphoma, but most are uncommon
5/1/2023 29

30. CHARACTERISTICS OF NHL
• occurs in older persons (40 to 70 years of age; mean, 55 years)
than does HD.
• more common than HD in children
• The incidence of NHL is significantly higher in immunodeficient
patients.
• associated with congenital immunodeficiency syndromes, HIV
infection, and immunosuppressive therapy.
5/1/2023 30

31. Lymph Node Involvement
• mediastinal lymph node
involvement is the most
common thoracic
abnormality in patients with
NHL
• Lymph node enlargement is
present in more than 75% of
patients with intrathoracic
disease
• 75% of patients with an
intrathoracic abnormality
(and 35% of all cases) have
prevascular or pretracheal
node involvement
5/1/2023 31

32. PATTERN OF LN INVOLVEMENT IN NHL
• single node group
involvement -NHL(40%)
than HL(15%).
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33. PATTERN OF LN INVOLVEMENT IN NHL
• the involvement of posterior mediastinal
nodal groups contiguous with upper
abdominal node enlargement is
relatively more common with NHL than
with HL.
• The abdomen, pelvis, and neck must be
scanned in patients with NHL, because
noncontiguous spread is common.
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34. PATTERN OF LN INVOLVEMENT IN NHL…
• Enlarged lymph nodes or mediastinal
masses may appear low in
attenuation due to necrosis or may
be cystic.
• Calcification of nodes or masses is
rare.
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35. Extranodal Disease
• Extranodal involvement is much more common with NHL than
it is with HL.
• Extra nodal disease seen in patients with a thoracic
abnormality includes
 Lung involvement - 30%
 pleural effusion or mass - 45%
 pericardial effusion or mass - 15%
 chest wall involvement - 10%
5/1/2023 35

36. Lung involvement
 discrete nodules or
masses
 air-space consolidation,
 infiltration contiguous
with enlarged lymph
nodes or masses
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37. Lung involvement…
 A: HRCT at a soft tissue
window shows enlarged
mediastinal lymph nodes.
 B: HRCT shows diffuse lung
infiltration with interlobular
septal thickening and some
nodularity closely mimicking
the appearance of
lymphangitic spread of
carcinoma.
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38. Lung involvement…
• lung infiltration may be rapid,
mimicking pneumonia.
• Bronchial narrowing or obstruction
resulting in atelectasis.
• A: Bilateral hilar masses with bronchial
obstruction and atelectasis. The
masses appear lower in attenuation
than the collapsed lung.
• B: There is irregular narrowing of the
left bronchi because of tumor
involvement of the bronchial wall.
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39. NHL pleural and chest wall involvement
5/1/2023 39

40. Primary Mediastinal NHL
• NHL may occur primarily in the
mediastinum.
• The most common cell types
1. T-cell lymphoblastic lymphoma/leukemia
2. primary mediastinal (thymic) large B-cell
lymphoma
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41. T-Cell Lymphoblastic Lymphoma/Leukemia
• 60% of primary
mediastinal NH
• Most patients are
children or young
adults.
• Lymph nodes in other
sites suggest leukemia
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42. Primary Mediastinal (Thymic) Large B-cell Lymphoma
 DLBCL that accounts for about 2%
of all lymphomas.
 Two thirds of cases occur in
women, and most are in their 30s.
 It is fast growing, but it usually
responds well to treatment.
 arise from thymic medullary B cells.
 difficult to distinguish from HL,
both clinically and radiographically
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43. Part 2- pulmonary lymphoproliferative disease
• characterised by abnormal proliferation of indigenous cell lines
or infiltration of lung parenchyma by lymphoid cells.
• classified as reactive or neoplastic on the basis of cellular
morphology and clonality.
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44. 1. Reactive/non-neoplastic lymphoid lesions
• Reactive/non-neoplastic LPD - results primarily
from antigenic stimulation and comprises three
main entities each with differing patterns and
distribution of lymphoid infiltrate::
 nodular lymphoid hyperplasia (NLH): focal
 follicular bronchiolitis (FB): peribronchial
 lymphoid interstitial pneumonia (LIP): diffuse
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45. Nodular Lymphoid Hyperplasia
• Localized proliferation of benign
mononuclear cells.
• “pseudolymphoma.”
KEY CT FINDINGS:
• Well circumscribed Multiple or solitary
nodule or a focal area of consolidation.
• There is no associated
lymphadenopathy.
• air bronchogram
• Note the mild surrounding linear
opacities indicating focal lymphangitic
extension.
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46. Follicular Bronchiolitis
• hyperplasia of the BALT or MALT.
• results from antigenic stimulation of the BALT and polyclonal
lymphoid hyperplasia.
• abundant peribronchiolar lymphoid follicles.
• Lymphoid follicular hyperplasia in the walls of bronchioles
with narrowing of the lumen is the defining feature of FB.
• lacks the extensive alveolar infiltration LIP.
• collagen vascular disease, immunodeficiency, hypersensitivity
disorders.
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47. FB
HRCT
• bilateral centrilobular
nodules,
• tree-in-bud opacities,
• small or large nodules,
ground-glass opacities
• a good prognosis
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48. Lymphoid Interstitial Pneumonia
• diffuse interstitial infiltrate
lymphocytes and plasma cells.
• In patients with AIDS, LIP
usually occurs in children.
• Adult female with connective
tissue disorders (mean age,
50 yrs).
• reticular or reticulonodular
pattern lower lung zones.
• cystic airspaces
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49. Lymphoid Interstitial Pneumonia …
CT: key features
 Ground-glass opacity.
 Nodules—poorly defined, centrilobular
 Cystic airspaces.
 Peribronchovascular interstitial thickening.
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50. 2. malignant lymphoproliferative disorders
Primary malignant lymphoproliferative disorders.
• primary pulmonary lymphoma (PPL)
 monoclonal lymphoid proliferation affecting the lungs in a patient
with no detectable extrathoracic lymphoma for at least 3 months
after the initial diagnosis.
 Less than 1%.
 arising in pulmonary lymphoid tissue.
• The malignant LPDs that present with primary lung involvement include:
• MALT lymphoma
• diffuse large B-cell lymphoma (DLBCL)
• lymphomatoid granulomatosis (LYG).
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51. MALToma
• Bronchial MALT distributed along the bifurcation
of bronchioles and along lymphatic routes
inconspicuous in healthy adult individuals, but
may become hyperplastic secondary to antigenic
stimulation.
• MALToma accounts for 70% to 90% of primary
pulmonary lymphomas, but less than 0.5% of all
primary lung tumors.
• These are derived from MALT, hence the term
MALToma or MALT lymphoma.
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52. MALToma
CT: key features
• A positive ‘‘angiogram’’ sign,
• single or multiple nodules or
areas of consolidation(70%)
• air bronchograms.
• Peribronchovascular
distribution.
• bronchiectasis and bronchiolitis
• hilar/mediasitinal
lymphadenopathy(30%)
5/1/2023 52

53. lymphoid hyperplasia VS MALT
lymphoma
• There is a significant overlap.
1. NLH is more often seen as a solitary nodule
unlike MALT lymphoma, which usually
presents with multiple, bilateral nodules.
2. Pleural invasion favours MALT lymphoma
3. Prominent lymphangitic extension favours
MALT lymphoma.
 denser consolidation, larger nodules and
pleural effusion suggest lymphoma while
cyst likely go for LIP.
5/1/2023 53

54. Diffuse Large B-cell Lymphoma
• sheets of medium to large atypical
lymphocytes forming a solid lesion that
replaces the underlying lung architecture.
• necrosis is a common feature, occasionally
with central cavitation.
Key CT findings:
• solitary or multiple nodules.
• air bronchograms may be visible
• bilateral consolidation or a diffuse
reticulonodular pattern.
• Lymph node enlargement may be present.
5/1/2023 54

55. Lymphomatoid Granulomatosis
• rare Epstein–Barr virus (EBV)-associated
lymphoproliferative disorder with a propensity for blood
vessel destruction.
atypical reactive T cell infiltration
the lung is the most common primary site of
involvement,
it is often difficult to prove clonality, and
it has an angiocentric distribution
• a poor prognosis and a median survival of less than 2
years
5/1/2023 55

56. Lymphomatoid granulomatosis
• Nodules can disappear or
migrate spontaneously ,
and
• may display central
ground-glass opacity
surrounded by denser
consolidation -‘‘reversed
halo sign’’.
5/1/2023 56

57. Lymphomatoid Granulomatosis
• Key CT findings
 bilateral, poorly defined nodular
lesions.
 a basal predominance.
 Peribronchovascular distribution
 Can coalesce and cavitate,
 Reversed halo sign
 ‘‘Migratory’’ nodules
 mimicking granulomatosis with
polyangiitis (Wegener’s
granulomatosis).
5/1/2023 57

58. Primary Effusion Lymphoma(PEL)
• rare and aggressive B-cell NHL presents with
malignant effusions, without tumor masses.
• universally associated with human herpesvirus 8
(HHV-8, Kaposi sarcoma)
• Patients are in immunosuppressed states.
• Even with combination chemotherapy prognosis
is poor.
5/1/2023 58

59. Plasmacytoma
• focal neoplastic proliferation of
plasma cells unassociated with
multiple myeloma.
• usually originates in bone,
resulting in an expansile
osteolytic lesion.
• rare form of primary
pulmonary lymphoma.
• tracheal or endobronchial
mass or pulmonary nodules or
mass lesions.
5/1/2023 59

60. 3. Lymphoproliferative disorders in the immunocompromised patient
• Lymphoma is the second most common tumour occurring in
patients with AIDS , after Kaposi’s sarcoma.
• they are generally more aggressive and present with more
varied radiological appearance.
• AIDS- related lymphoma (ARL):DLBCL and Burkitt lymphoma
being the commonest.
• post-transplantation lymphoproliferative disorder (PTLD),
which includes reactive processes, neoplasms and lymphoid
proliferations of uncertain nature
5/1/2023 60

61. AIDS-Related Lymphoma
• 90% of these patients, it is a B-cell
NHL.
• characterized by advanced clinical
stage,often involves multiple sites,
high histologic grade, frequent
posttreatment relapse, and poor
survival.
CT: key features
• Well-defined pulmonary nodules
• Nodules + effusions + adenopathy +
HIV = highly suggestive of ARL.
5/1/2023 61

62. Posttransplantation Lymphoproliferative Disorder
• lymphocyte proliferation, known collectively as PTLD, can
occur after bone marrow or solid organ transplantation.
• The histologic patterns range from benign hyperplastic
proliferation of lymphocytes to malignant lymphoma, either
HL or NHL.
• Most cases of PTLD have been associated with EBV infection.
• first year after transplantation.
• PTLD can manifest as localized or disseminated disease.
• extranodal involvement.
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63. SUMMARY
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64. SUMMARY
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65. REFERENCES
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66. THANK YOU
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