Extranodal  lymphoma      

Rising  Incidence  and  Pa7erns  in  the  Abdomen  

Cynthia  Walsh    FRCP(C)  Department  of  Radiology,  The  O7awa  Hospital  

O7awa,  Canada  

I  HAVE  NO  DISCLOSURES  

“THE  RISING  INCIDENCE  OF  LYMPHOMA  IN  THE  PAST  2  DECADES  HAS  BEEN  CHARACTERIZED  BY  AN  

 

INCREASE  IN  EXTRANODAL  LYMPHOMA”  

 1.  Immunosuppression  •  HIV  •  Solid  organ  transplants  (esp.  cyclosporine)  

2.  Indolent  viral  infecWons  (EBV)  3.  Increase  in  skin  lymphomas  may  be  related  to  sunlight  

 

Learning  ObjecWves  

1.  To  recognize  the  increasing  incidence  of  extranodal  lymphoma  

2.  To  review  the  most  common  pa7erns  of  involvement  in  the  abdomen  

3.  To  review  potenWal  pi\alls  and  differenWal  diagnoses  in  extranodal  lymphoma  

Outline:  Extranodal  Lymphoma  •  DefiniWon  •  Primary  vs.  Secondary  •  Types  of  Lymphoma  most  likely  to  manifest  as  extranodal  

disease  

•  Most  Common  Involvement  In  The  Abdomen  –  Spleen  –  Liver  –  GI  tract  –  Kidneys  

Extranodal  Lymphoma  DEFINITION:    •   Lymphoma  located  outside  of  lymph  nodes,  thymus,  tonsils,  Waldeyer  ‘s  ring    

SPLEEN:  •  Nodal  disease  in  HL  •  Extranodal  disease  in  NHL  

 PROGNOSIS:    •  Usually  intermediate  to  high-­‐grade  •  Typically  indicates  a  more  advanced  stage  of  disease  •  Poor  prognosWc  factor  

FREQUENCY:  •  More  common  in  NHL  than  HL  (40%  vs.    5%)  •  More  common  in  recurrent  disease  or  immunodeficiency  

Extranodal  Lymphoma  DEFINITION:    •   Lymphoma  located  outside  of  lymph  nodes,  thymus,  tonsils,  Waldeyer  ‘s  ring    

SPLEEN:  •  Nodal  disease  in  HL  •  Extranodal  disease  in  NHL  

 PROGNOSIS:    •  Usually  intermediate  to  high-­‐grade  •  Typically  indicates  a  more  advanced  stage  of  disease  •  Poor  prognosWc  factor  

FREQUENCY:  •  More  common  in  NHL  than  HL  (40%  vs.    5%)  •  More  common  in  recurrent  disease  or  immunodeficiency  

Extranodal  Lymphoma  DEFINITION:    •   Lymphoma  located  outside  of  lymph  nodes,  thymus,  tonsils,  Waldeyer  ‘s  ring    

SPLEEN:  •  Nodal  disease  in  HL  •  Extranodal  disease  in  NHL  

 PROGNOSIS:    •  Usually  intermediate  to  high-­‐grade  •  Typically  indicates  a  more  advanced  stage  of  disease  •  Poor  prognosWc  factor  

FREQUENCY:  •  More  common  in  NHL  than  HL  (40%  vs.    5%)  •  More  common  in  recurrent  disease  or  immunodeficiency  

Extranodal  Lymphoma  DEFINITION:    •   Lymphoma  located  outside  of  lymph  nodes,  thymus,  tonsils,  Waldeyer  ‘s  ring    

SPLEEN:  •  Nodal  disease  in  HL  •  Extranodal  disease  in  NHL  

 PROGNOSIS:    •  Usually  intermediate  to  high-­‐grade  •  Typically  indicates  a  more  advanced  stage  of  disease  •  Poor  prognosWc  factor  

FREQUENCY:  •  More  common  in  NHL  than  HL  (40%  vs.    5%)  •  More  common  in  recurrent  disease  or  immunodeficiency  

 Primary  vs.  Secondary  Extranodal  

Lymphoma     Secondary  Extranodal  

lymphoma  

Ø  Involves  nodes  other  than  those  immediately  adjacent  to  the  primary  organ    

Ø OR  more  than  one  extranodal  site    

•  Stage  III  or  IV      •  More  common  than  

primary    

Primary  Extranodal  lymphoma  

Ø  Confined  to  a  single  organ  and  immediately  adjacent  lymph  nodes  

•  Can  be  Stage  I  or  II  

PET/CT  and  Extranodal  Disease  

Advantages  of  FDG  PET/CT  are  largely  due  to:    

1.   Extranodal  sites  not  iden0fied  at  CT    2.  DetecWon  of  FDG-­‐avid,  normal-­‐sized  lymph  

nodes  (usually  <1  cm)  

Subtypes  of  Lymphoma  involving  Extranodal  sites  

•  Most  common  subtypes:    

•  Diffuse  large  B-­‐cell    •  Follicular  lymphoma    

•  Most  LIKELY  to  affect  extranodal  sites:  •  Mucosa-­‐associated  lymphoid  Wssue  (MALT)  lymphoma  •  Mantle  cell  lymphoma  •  LymphoblasWc  lymphoma  •  Burki7's  lymphoma  

Splenic  lymphoma  

SPLENIC  LYMPHOMA  

•  Most  common  splenic  malignancy  

•  PET/CT:    – Accuracy  of  almost  100%  for  primary  splenic  involvement  at  staging  (CT  accuracy  =  57%  )  

– EvaluaWon  more  limited  amer  treatment  due  to  splenic  acWvaWon  

Discrete  Nodules  

Normal    

Splenic  lymphoma:  Pa7erns  of  involvement  

Diffuse  infiltraWon    

Splenomegaly  

Pa7erns  of  Splenic  Lymphoma:    Diffuse  infiltraWon  

•  Most  common  pa7ern  •  May  look    normal  

Lymphadenopathy  in  the  splenic  hilum  is  suggesWve  of  splenic  disease  

Splenic  Lymphoma:  Splenomegaly  Size  is  a  poor  indicator  of  splenic  involvement  

Moderate  splenomegaly  •  ReacWve  splenomegaly  

occurs  in  30%    of  paWents    

Marked  splenomegaly  •  Almost  always  

indicates  infiltraWon  

Normal  size  •  Does  not  exclude  

involvement  

Patterns of Splenic Lymphoma: Discrete nodules

•  CT:  Low  a7enuaWon  and  hypovascular  •  MRI:  hypo/isointense  on  T1WI,  hyperintense  on  

T2WI,  hypovascular  

Pa7erns  of  Splenic  Lymphoma:    Discrete  nodules    

On  Ultrasound:    hypoechoic    with  no  through  transmission  

HepaWc  lymphoma  

•  At  presentaWon,  the  liver  is  involved  in  – 15%  of  paWents  with  NHL    – 10%  of  with  HD  

•  Primary  disease  is  rare:  – Most  common  in  immunocompromised  paWents  

HepaWc  lymphoma  

Pa7erns  of  HepaWc  lymphoma  

Pa7erns  of  HepaWc  Lymphoma:  Hepatomegaly  and  Diffuse  InfiltraWon  

1.  Diffuse  infiltraWon  is  the  most  common  pa7ern    2.  Hepatomegaly:  nonspecific.    

Pa7erns  of  HepaWc  lymphoma:  Discrete  nodules  

•  HepaWc  NHL  lymphoma  may  occur  in  the  absence  of  splenic  disease    •  Hodgkin’s  Lymphoma  of  the  liver  is  almost  always  associated  with  splenic  

disease  

•  Single  dominant  mass  •  Heterogeneous  

•  Typically  mulWple,  smaller  masses  •  Homogenous  

Secondary  HepaWc  Lymphoma  

Primary    HepaWc  Lymphoma  

HepaWc  lymphoma  

HepaWc  lymphoma  

CT  

MRI:  +  Gado    MRI:  T2WI  

MRI:  T1WI  

HepaWc  lymphoma  

1.  Homogenous  2.  Bulky  lymphadenopathy  3.  Spleen  omen  involved    

1.  Heterogeneous  2.  Spleen  usually  normal  

Metastases  vs.  Lymphoma  

Metastases  vs.  Lymphoma  

MetastaWc  Melanoma  Lymphoma  

Microabcesses  

Disseminated  fungal  infecWon  in  immunocompromised  paWents    

•  Typically  smaller  and  more  heterogeneous    •  Peripheral  enhancement  •  No  lymphadenopathy    •  MRI  most  sensiWve  in  differenWaWng  lymphoma  from  fungus  

Focal  Lymphoma  

Lymphoma  of  the  GI  Tract  

Lymphoma  of  the  GI  tract  

•  Primary  GI  lymphoma  is  the  most  common  extra-­‐nodal  manifestaWon  of  non-­‐Hodgkin  lymphoma  (20%  of  all  cases)  

•  MulWple  sites  omen  involved  

ObstrucWon  is  uncommon  …  EXCEPTION  …T  cell  lymphoma      

 PerforaWon  is  uncommon…  EXCEPTION  …Post  chemotherapy  

Imaging  appearance  of  GI  Lymphoma    

1.  Nodular  2.  Polypoid    3.  InfiltraWve  4.  Aneurysmal    5.  UlceraWve  

CT  staging  has  important  therapeuWc  implicaWons:    

•  Stage  I  and  II  disease  may  be  excised  •  Stage  III  and  IV  disease  must  be  treated  with  radiaWon,  chemotherapy,  

or  both.  

Ø  GI  tract  lymphoma  may  be  missed  at  endoscopy    

Risk  factors  for  GI  Lymphoma:  1.  HIV  2.   Helicobacter  pylori  3.  Celiac  disease  (Enteropathy  Type  T-­‐cell  

Lymphoma)  

4.  IBD  –  2-­‐3  x  increase  –  Further  5x  increased  risk  with  immunosuppression  

5.  Immunosuppression  amer  solid  organ  transplantaWon  

Gastric  lymphoma  

Most  common  histologic  subtypes:    

•  H.  Pylori  –associated  low-­‐grade  MALT  lymphoma  •  High-­‐grade  diffuse  large  B-­‐cell  lymphoma.    

Gastric  MALT  Lymphoma  •  Chronic  H  pylori  infecWon  is  associated  with  development  of  mucosa  

associated  lymphoid  Wssue  =  MALT  

Ø  Most  low-­‐grade  primary  gastric  lymphomas  arise  from  MALT  

Ø  Low-­‐grade  MALT  lymphoma  diagnosed  early  has  a  good  prognosis  (5-­‐year  survival  75%–91%)  

Ø  High  grade  MALT  lymphoma:  survival  rate    <  50%    

Therefore,  early  diagnosis  is  crucial    

Ø   detecWon  challenging  as  the  clinical,  endoscopic,  and  radiologic  findings    can  simulate  gastriWs  and  gastric  carcinoma.    

MALT  LYMPHOMA  (High  Grade)  

•  Normal  CT  shown  to  be  highly  predicWve  of  low-­‐grade  MALT  lymphoma    •  Greater  than  minimal  thickening  suggests  transformaWon  to  a  higher  grade  •  Lymphadenopathy  less  common  in  low-­‐grade  MALT  Lymphoma    

Gastric  B  Cell  Lymphoma  

Gastric  Lymphoma  vs.  Adenocarcinoma  

Lymphoma    

•  Diffuse  wall  thickening  •  Hypovascular  •  Gastric  distensibility  •  Lymphadenopathy  below  the  renal  pedicle  

Adenocarcinoma    

•  More  focal  wall  thickening  •  Greater  tumour  enhancement  •  Tumour  infiltraWon  beyond  gastric  wall  •  LiniWs  plasWca  •  Gastric  outlet  obstrucWon    

Lymphadenopathy  extending  below  the  renal  

pedicle  =  Lymphoma  

Gastric  Lymphoma  vs.  Adenocarcinoma  

DDx:  GIST  

Occasionally,  a  GIST  may  mimic  a  gastric  lymphoma  

Lymphoma  of  the  Small  Bowel  

•  Rising  incidence  related  to  B-­‐cell  hyperacWvaWon  in  HIV  •  Distal  ileum  is  the  most  frequently  affected  site    

Small  Bowel  Lymphoma  

Small  Bowel  Lymphoma  

Mantle  cell  lymphoma:    Most  common  subtype  

Enteropathy  Associated  T  Cell  Lymphoma:  ObstrucWon  

Enteropathy  Associated  T  Cell  Lymphoma:  ObstrucWon  

•  50  x  increased  risk  with  untreated  Celiac  Disease  

•  Less  wall  thickening  than  B-­‐cell  lymphomas    

•  Imaging  features  similar  to  IBD  with  extensive  ulcers,  refractory  to  treatment  

   

***  Death  is  usually  due  to  perfora0on  caused  by  refractory  malignant  ulcers.    

 

Post  Transplant  ProliferaWve  Disorder  

Post  Transplant  ProliferaWve  Disorder:  Perforated  Esophagus  

SB  lymphoma  vs.  Adenocarcinoma  

•  Cavitary  mass  •  MulWfocal  •  Bulky  lymphadenopathy  

•  Solid  mass  •  ObstrucWng  

Duodenal  lymphoma  

Lymphoma  of  the  Large  Bowel    

Rectal  mass   Bulky  Lymphadenopathy  

Most  common  in  the  cecum  and  rectum  

Mantel  Cell  Lymphoma  of  the  Cecum  with  intussuscepWon  

Mantel  Cell  Lymphoma  of  the  Cecum  with  intussuscepWon  

Colonic  lymphoma  presenWng  with  innumerable  polypoidal  lesions  

T2   T1  +  Gado  

Rectal  Lymphoma  

T2   T1  +  Gado  

Cecal  lymphoma  with  lymphadenopathy  

Cecal  Mass   Bulky  Lymphadenopathy  

Renal  Lymphoma  

 Renal  Lymphoma    

 •  Usually  clinically  silent    •  Acute  renal  failure  is  rare    •  Incidence:    

–  60  %  at  autopsy  – …BUT  only  8%  of  all  pa0ents  at  staging  CT    

Omen  minimal  mass  effect  on  renal  contour    

Ø  Imaging  findings  can  be  subtle  Ø  IV  contrast  is  essenWal  for  diagnosis.  

Pa7erns  of  Renal  Lymphoma  

•  MulWple  masses  •  Single  mass  •  Invasion  from  retroperitoneal  disease  •  Perirenal  disease  •  InfiltraWve  

Pa7erns  of  Renal  Lymphoma:    MulWple  Lesions  =  widespread  disease  

•  Most  common  pa7ern    •  Usually  bilateral  

Pa7erns  of  Renal  Lymphoma:    MulWple  Lesions  =  widespread  disease  

MulWfocal    Lymphoma  

Metastases    (Lung  Cancer)  

Pa7erns  of  Renal  Lymphoma:    Solitary  Lesion  

Ultrasound  •  hypoechoic  (reflects  Wssue  

homogeneity)    

CT  •  Nephrographic-­‐phase  is  the  most  

sensiWve  for  lesion  detecWon    

•  Typically  vascular  (cauWon…papillary  RCC)  

•   Omen  heterogeneous  

•  Typically  hypovascular    •  Homogeneous  

Lymphoma   Renal  Cell  Carcinoma  

•  Varying  appearances  •  Typically  more  echogenic  and  

heterogeneous  

•  Homogenously  hypoechoic…PITFALL  =  CYST  

•  Hypovascular    

Lymphoma   Renal  Cell  Carcinoma  

Diagnosis:  PyelonephriWs  

3  months  later…  

January  2008   April  2008  

3  months  later…Bilateral  Disease    -­‐  Biopsy  proven  renal  lymphoma  

January  2008   April  2008  

Transplant  kidney  mass  

DDx:  Renal  cell  carcinoma,  PTLD  

 Pa7erns  of  Renal  Lymphoma:    

Renal  invasion  from  con0guous  retroperitoneal  disease    

•  May  surround  kidney  without  parenchymal  compression    •  Renal  vessels  remain  patent  despite  encasement  •  Can  cause  obstrucWon  with  hydronephrosis    

Pa7erns  of  Renal  Lymphoma:    Perirenal  Disease  

Pa7erns  of  Renal  Lymphoma:    Diffuse  infiltra0on    

•  Almost  always  bilateral    •  Renal  funcWon  poor,  but  usually  sufficient  to  remain  clinically  

silent.    

Diffuse  InfiltraWon  

Nephromegaly  and  infiltrated  parenchyma  

Diffuse  InfiltraWon  

Nephromegaly   4  years  earlier  

PaWent  with  AIDS    

•  Nephromegaly  (17  cm)  •  Echogenic  Kidney  

Ø  DDx:  Aids  Nephropathy  vs.                InfiltraWve  Lymphoma    

Ø Biopsy:  Lymphoma  

Pa7erns  of  Renal  Lymphoma:    Focal  Infiltra0on  

DDx:  TCC  or  pyelonephriWs  

InfiltraWng  hilar  mass  causing    hydronephrosis  

Learning  ObjecWves  

1.  To  recognize  the  increasing  incidence  of  extranodal  lymphoma  

2.  To  review  the  most  common  pa7erns  of  involvement  in  the  abdomen  

3.  To  review  potenWal  pi\alls  and  differenWal  diagnoses  in  extranodal  lymphoma  

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Thank  You  

Acknowledgements  All  of  my  Radiology  Colleagues  at  The  O7awa  Hospital  for  contribuWng  cases