multisystem disease 2 lymphoma

Multisystem Disease 2 Multisystem Disease 2 Lymphoma Lymphoma

A SwartbooiA Swartbooi

Clinical Imaging SciencesClinical Imaging Sciences

28 September 201228 September 2012

Extranodal LymphomaExtranodal Lymphoma

Lymphomatous infiltration of Lymphomatous infiltration of anatomic sites other than the anatomic sites other than the lymph nodeslymph nodes

Primary and secondary Primary and secondary involvementinvolvement

Occur in both HD and NHLOccur in both HD and NHL– More common in NHLMore common in NHL


Primary involvementPrimary involvement– 30-40% of cases30-40% of cases– Lymph nodal involvement limited to Lymph nodal involvement limited to regional nodesregional nodes– Stage I or II diseaseStage I or II disease– 11 extranodal HD ext rare – disease ⁰ extranodal HD ext rare – disease ⁰involvement elsewhere for exclusioninvolvement elsewhere for exclusion– Increased incidence noted as frequency of Increased incidence noted as frequency of NHL is seenNHL is seen

Seen esp of CNS and OrbitsSeen esp of CNS and Orbits


Primary involvementPrimary involvement– Incidence of NHLIncidence of NHL

Age of pt (Age of pt (⇧ children⇧ children)) Immune status (Immune status (⇧ with ⇩ CD4⇧ with ⇩ CD4))

– More aggressive subtypeMore aggressive subtype Pathological subtypePathological subtype

– Mantle cell lymphomaMantle cell lymphoma– Lymphoblastic lymphomaLymphoblastic lymphoma– Burkitt lymphomaBurkitt lymphoma– MALT lymphomaMALT lymphoma


Secondary involvementSecondary involvement– Presence of widespread advanced Presence of widespread advanced

disease elsewheredisease elsewhere– Both HD and NHLBoth HD and NHL

NHL more commonNHL more common

– Adverse prognostic factorAdverse prognostic factor Stage III or IV diseaseStage III or IV disease

ThoraxThorax

Pulmonary parenchymaPulmonary parenchyma– 22 Involvement⁰ Involvement⁰

HD more common 12% than NHL 4% HD more common 12% than NHL 4% Intrathoracic LNIntrathoracic LN Extrathoracic LNExtrathoracic LN

– Direct ext of nodal diseaseDirect ext of nodal disease Paramediastinal / Perihilar lung Paramediastinal / Perihilar lung

involvementinvolvement

ThoraxThorax

ThoraxThorax

Pulmonary parenchymaPulmonary parenchyma– Recurrence after previously Rx Recurrence after previously Rx

(radiation) mediastinal HD often confined (radiation) mediastinal HD often confined to lungsto lungs

– In compar pt with NHL and pulmonary or In compar pt with NHL and pulmonary or pleural diseasepleural disease

No mediastinal nodes in 50%No mediastinal nodes in 50%

– As disease progress/relapseAs disease progress/relapse Lung disease become commonLung disease become common Pt with HD 30-40% have pulmon involvementPt with HD 30-40% have pulmon involvement

ThoraxThorax


< 1% of lymhoma< 1% of lymhoma Primary NHLPrimary NHL– Usually low grade B-cell NHL (80-85%)Usually low grade B-cell NHL (80-85%)

Commonly single lung noduleCommonly single lung nodule Multiple nodules or segm consolidation may Multiple nodules or segm consolidation may occuroccur Pleural eff in 20%Pleural eff in 20%– High grade NHL (15-20%)High grade NHL (15-20%) Rapidly enlarging single or multiple nodulesRapidly enlarging single or multiple nodules

ThoraxThorax


Primary HDPrimary HD– Extremely rareExtremely rare– Single or multiple nodulesSingle or multiple nodules– Upper lobeUpper lobe– CavitateCavitate– Pleural eff in 20%Pleural eff in 20%

ThoraxThorax

Pleural diseasePleural disease– Pleural effusionsPleural effusions– Usually accompanied by mediastinal Usually accompanied by mediastinal lymphadenopathylymphadenopathy– 10% of patients with NHL and 7% of patients 10% of patients with NHL and 7% of patients with HD with HD – Secondary to central lymphatic or venous Secondary to central lymphatic or venous obstructionobstruction– Rx of mediastinal diseaseRx of mediastinal disease– Pleural masses more common in relapse than Pleural masses more common in relapse than presentationpresentation

ThoraxThorax

ThoraxThorax

Heart & PericardiumHeart & Pericardium– Can occur with high-grade peripheral T-Can occur with high-grade peripheral T-cell and large B-cell lymphomascell and large B-cell lymphomas– Direct spread rareDirect spread rare Except in AIDS related lymphoma (ARL) and Except in AIDS related lymphoma (ARL) and post-transplant lymphoproliferative disorders post-transplant lymphoproliferative disorders (PTLD)(PTLD)– Pericardial effusions occur in 6% of Pericardial effusions occur in 6% of patients with HD at presentationpatients with HD at presentation Associated mass adjacent to heartAssociated mass adjacent to heart

ThoraxThorax

ThymusThymus– 1 Thymic HD rare⁰1 Thymic HD rare⁰ Involvement noted in assoc with Involvement noted in assoc with mediastinal nodes in 30-50%mediastinal nodes in 30-50% Often large B-cell lymphomaOften large B-cell lymphoma Young woman (25-40yrs)Young woman (25-40yrs) Typically rapidly growing with SVC obstr.Typically rapidly growing with SVC obstr.(40%)(40%) Thymus homogenous or heterogenous; Thymus homogenous or heterogenous; may be nodular (lobulated) or smoothmay be nodular (lobulated) or smooth

ThoraxThorax

ThymusThymus

ThoraxThorax

Chest wallChest wall– From direct extensionFrom direct extension Ant Mediastinal nodesAnt Mediastinal nodes Ax & Supra clav nodesAx & Supra clav nodes– No bony destructionNo bony destruction Infection or MalignancyInfection or Malignancy

BreastBreast

Widespread disease elsewhereWidespread disease elsewhere NHL of breast rareNHL of breast rare– 2% lymphomas; 0.5% breast 2% lymphomas; 0.5% breast malignancymalignancy BimodalBimodal– Pregnancy and lactationPregnancy and lactation

High grade or burkittHigh grade or burkitt Billat with inflammatory pictureBillat with inflammatory picture

BreastBreast

BreastBreast

BimodalBimodal– 50yrs50yrs UnilateralUnilateral Discrete often solitary massDiscrete often solitary mass No archicteral distortion/skin thickeningNo archicteral distortion/skin thickening No calcificationsNo calcifications

2 involvement characterized by ⁰2 involvement characterized by ⁰multiple nodules and enlarged LN’smultiple nodules and enlarged LN’s

AbdomenAbdomen

Hepatobiliary systemHepatobiliary system– Liver involvementLiver involvement

15 % in NHL15 % in NHL– ⇧ ⇧ in Paeds and with recurrencein Paeds and with recurrence 5% in HD5% in HD Primary hepatic disease rare (< 1%)Primary hepatic disease rare (< 1%)– Increased in pt HepA or HepC + (25%)Increased in pt HepA or HepC + (25%) Infiltration around portal tracts most Infiltration around portal tracts most commoncommon Hepatomegaly suggest diffuse involvementHepatomegaly suggest diffuse involvement

AbdomenAbdomen

Hepatobiliary systemHepatobiliary system– Liver involvementLiver involvement

5-10% focal infiltration5-10% focal infiltration– Resemble metastasesResemble metastases

Hypoechoiec and well defined on USHypoechoiec and well defined on US

– GBGB NHL Rare, but NHL Rare, but ⇧ frequency in patients ⇧ frequency in patients with ARLwith ARL

AbdomenAbdomen

SpleenSpleen– Affected in 20% of patients with NHLAffected in 20% of patients with NHL– 30–40% of patients with HD at 30–40% of patients with HD at presentation, considered a nodal organpresentation, considered a nodal organ– Presence of nodal disease above and Presence of nodal disease above and below the diaphragm (stage III), but in a below the diaphragm (stage III), but in a small proportion it is the sole focus of small proportion it is the sole focus of intra-abdominal disease.intra-abdominal disease.– Microscopic diffuse infiltration with only Microscopic diffuse infiltration with only splenomegaly (not indicate involvement)splenomegaly (not indicate involvement)

AbdomenAbdomen

SpleenSpleen– 33% splenomeg – no infiltration33% splenomeg – no infiltration– 33% normal – infiltration33% normal – infiltration– Volume + indices not usedVolume + indices not used– Focal lesions 10-25% > 1 cm Focal lesions 10-25% > 1 cm

detected by cross sect imagingdetected by cross sect imaging– 40% of NHL has involvement some 40% of NHL has involvement some

timetime

AbdomenAbdomen

SpleenSpleen– ImagingImaging

Solitary, miliary, multiple massesSolitary, miliary, multiple masses Ddx: infection, granulomatous diseaseDdx: infection, granulomatous disease CT & US sensitive (us >3mm)CT & US sensitive (us >3mm) MRI (not routine)MRI (not routine) FDG–PET > CT or ScintigraphyFDG–PET > CT or Scintigraphy

– 11 splenic lymphoma rare (1%)⁰ splenic lymphoma rare (1%)⁰

AbdomenAbdomen

SpleenSpleen

AbdomenAbdomen

GITGIT– Most common site of 1 EN NHL⁰Most common site of 1 EN NHL⁰ 30-45% of all30-45% of all 10% adults + 30% children10% adults + 30% children– 1 HD of GIT rare⁰1 HD of GIT rare⁰ 2 involvement common⁰2 involvement common⁰ Direct extension, multiple sitesDirect extension, multiple sites– 1 lymphoma from lamina propria + ⁰1 lymphoma from lamina propria + ⁰submucosa submucosa

AbdomenAbdomen

GITGIT– Frequently below the age of 10 Frequently below the age of 10 years years (BL)(BL)– Sixth decade (MALT type Sixth decade (MALT type enteropathy-associated T-cell type)enteropathy-associated T-cell type)– Stomach 50%, Small bowel 35%, Stomach 50%, Small bowel 35%, large bowel 15%large bowel 15%

AbdomenAbdomen

GITGIT– StomachStomach 2–5% of all gastric tumours2–5% of all gastric tumours Submucosa, affecting the antrum moreSubmucosa, affecting the antrum more– ImagingImaging Multiple nodules, some with central Multiple nodules, some with central ulceration, or a large, fungating lesion with ulceration, or a large, fungating lesion with or without ulceration,wall thickening, or without ulceration,wall thickening, luminal narrowing, enlargement of the luminal narrowing, enlargement of the gastric folds (barium studies or gastric folds (barium studies or endoscopically)endoscopically)

AbdomenAbdomen

GITGIT– ImagingImaging Gastric wall Gastric wall thickening and thickening and accompanying nodal accompanying nodal involvement, which involvement, which is well shown on CTis well shown on CT Low grade – shallow Low grade – shallow ulcerationulceration High grade – High grade – massive infiltration, massive infiltration, massesmasses

AbdomenAbdomen

GITGIT– Small bowelSmall bowel 50% of all primary tumours of the small bowel50% of all primary tumours of the small bowel Term ileum frequent, duodenal rareTerm ileum frequent, duodenal rare Multifocal in 50%Multifocal in 50% Obstructive symptomsObstructive symptoms Bowel wall thickening is well demonstrated Bowel wall thickening is well demonstrated on CTon CT With infiltration alternating areas of With infiltration alternating areas of dilatation and constriction dilatation and constriction

AbdomenAbdomen

GITGIT– Submucosal infiltration with multiple Submucosal infiltration with multiple nodules or polyps of varying size nodules or polyps of varying size result in intususception (most result in intususception (most common cause < 6yrs)common cause < 6yrs)– Barium studies typically show Barium studies typically show multiple polypoid filling defects, with multiple polypoid filling defects, with or without central ulceration and or without central ulceration and irregular thickening of the valvulae.irregular thickening of the valvulae.

AbdomenAbdomen

GITGIT– Colon and rectumColon and rectum Primary colonic lymphomas are usually of Primary colonic lymphomas are usually of Burkitt or MALT subtypesBurkitt or MALT subtypes <0.1% of all colonic neoplasms<0.1% of all colonic neoplasms caecum and rectumcaecum and rectum diffuse or segmental distribution of small diffuse or segmental distribution of small nodules 0.2–2.0 cm in diameternodules 0.2–2.0 cm in diameter Mucosa intactMucosa intact Polypoid mass uncommon, mimic ca unless Polypoid mass uncommon, mimic ca unless term ileum involvementterm ileum involvement

AbdomenAbdomen

GITGIT– Advanced disease, there may be Advanced disease, there may be marked thickening of the colonic or marked thickening of the colonic or rectal folds resulting in focal rectal folds resulting in focal strictures, fissures or ulcerative strictures, fissures or ulcerative masses with fistula formationmasses with fistula formation– Strictures longer than caStrictures longer than ca– Obstruc and rectal bleedingObstruc and rectal bleeding

AbdomenAbdomen

GITGIT– Primary gastrointestinal lymphomaPrimary gastrointestinal lymphoma An absence of superficial or An absence of superficial or intrathoracic lymph node enlargement intrathoracic lymph node enlargement No involvement of the liver or spleenNo involvement of the liver or spleen A normal white cell countA normal white cell count No more than local regional lymph No more than local regional lymph node enlargementnode enlargement

AbdomenAbdomen

GITGIT

AbdomenAbdomen

PancreasPancreas– 1 cases <1.3% of pancreas tumours⁰1 cases <1.3% of pancreas tumours⁰– 2% of NHL2% of NHL– Indistinguishable from primary Indistinguishable from primary adenocarcinoma on US, CT, or MRIadenocarcinoma on US, CT, or MRI– 2 with disease elsewhere⁰2 with disease elsewhere⁰ Direct infiltration Direct infiltration

Genito-urinary TractGenito-urinary Tract

Not commonly involved at the Not commonly involved at the time of presentation (<5%)time of presentation (<5%) 50% of patients will have 50% of patients will have involvement of some part of the involvement of some part of the genitourinary tract at autopsygenitourinary tract at autopsy Testicle is the most commonly Testicle is the most commonly involved organ, followed by the involved organ, followed by the kidney and the perirenal spacekidney and the perirenal space


Rarely are the bladder, prostate, Rarely are the bladder, prostate, uterus, vagina, or ovaries uterus, vagina, or ovaries involvedinvolved 1 cases rare, little lymphoid ⁰1 cases rare, little lymphoid ⁰tissuetissue


KidneysKidneys– 90% of cases are in association with high-90% of cases are in association with high-grade NHL (BL)grade NHL (BL)– Often in recurrence, normal functionOften in recurrence, normal function– Multiple masses 60%Multiple masses 60% US hypoechoicUS hypoechoic Density reversal pattern on CTDensity reversal pattern on CT Solitary mass in 5-15%, indistinguishable RCCSolitary mass in 5-15%, indistinguishable RCC NHL renal mass often no retroperit nodesNHL renal mass often no retroperit nodes Direct infil in 25% Direct infil in 25% Vessel encasement, hilar extensionVessel encasement, hilar extension


KidneysKidneys


TestisTestis– 5% of primary testicular tumours overall and 5% of primary testicular tumours overall and 25–50% of those in patients over 50 years25–50% of those in patients over 50 years– Commonest primary tumour over the age of Commonest primary tumour over the age of 60 years60 years– 1% of all patients of NHL, usually with 1% of all patients of NHL, usually with DLBCL or BLDLBCL or BL– Association with lymphoma of Waldeyer's Association with lymphoma of Waldeyer's ring, the skin and central nervous systemring, the skin and central nervous system– Painless testicular swelling Painless testicular swelling


TestisTestis– Ultrasonically, the lesions usually Ultrasonically, the lesions usually have a nonspecific appearancehave a nonspecific appearance– Staging must always include Staging must always include ultrasonic evaluation of the ultrasonic evaluation of the contralateral testis and whole body CTcontralateral testis and whole body CT

Adrenal GlandsAdrenal Glands

25% of patients with NHL will 25% of patients with NHL will develop secondary involvement develop secondary involvement of the adrenal glandof the adrenal gland Primary adrenal lymphoma is Primary adrenal lymphoma is extremely rare, usually extremely rare, usually occurring in men over the age of occurring in men over the age of 60.60. Secondary involvement detected Secondary involvement detected in about 6% undergoing routine in about 6% undergoing routine abdominal staging CT, in the abdominal staging CT, in the presence of widespread presence of widespread retroperitoneal diseaseretroperitoneal disease Indistinguishable from Indistinguishable from metastases metastases

Central Nervous Central Nervous SystemSystem

1 CNS Lymphoma⁰1 CNS Lymphoma⁰– Brain and spine onlyBrain and spine only– 3% of brain tumours3% of brain tumours– 30% of NHL – immunodeficiency states30% of NHL – immunodeficiency states 6% AIDS pt get PCNSL6% AIDS pt get PCNSL– Peaks 40-60yrsPeaks 40-60yrs– Lesions often frontal (personality)Lesions often frontal (personality)– White matter close to CC (spread across)White matter close to CC (spread across)– Isointense on CT&MRI, Hom enhancementIsointense on CT&MRI, Hom enhancement


2 CNSL⁰2 CNSL⁰– 15% of NHL15% of NHL– In HD brain lesions often second dxIn HD brain lesions often second dx– Often extra-axial and spinal spacesOften extra-axial and spinal spaces– MRI more sensitive (enhancing MRI more sensitive (enhancing plaques)plaques)– Spinal presentation often from Spinal presentation often from extension of prevertebral mass (dum-extension of prevertebral mass (dum-bell tumour)bell tumour)


CNSLCNSL


Orbital lymphomaOrbital lymphoma– Pt’s 40-70yrsPt’s 40-70yrs– Slow growing, infiltratingSlow growing, infiltrating– NHL most commonNHL most common 10-15% of primary 10-15% of primary orbital tumour in adultsorbital tumour in adults 4% primary EN NHL4% primary EN NHL– 2 cases 3.5 – 5% of NHL & ⁰2 cases 3.5 – 5% of NHL & ⁰HDHD– Can involve any part, Can involve any part, symptoms acc to sitesymptoms acc to site

Head & NeckHead & Neck

Waldeyer’s ringWaldeyer’s ring– Commonest site (1/3 of EN NHL)Commonest site (1/3 of EN NHL)– Lymphoid tissue in nasopharynx, Lymphoid tissue in nasopharynx,

oropharynx, faucial palatine and lingual oropharynx, faucial palatine and lingual tonsilstonsils

– Circumferential or multifocal involvementCircumferential or multifocal involvement– 8% Paranasal sinus tumours are NHL8% Paranasal sinus tumours are NHL– Assoc with GIT involvementAssoc with GIT involvement– Most are DLBCLMost are DLBCL– MRI preferred imaging methodMRI preferred imaging method


ThyroidThyroid– NHL 2% malignant thyroid tumoursNHL 2% malignant thyroid tumours– MALT type associated with MALT type associated with Hashimoto’sHashimoto’s– Can extend beyond glandCan extend beyond gland– Hypoechoic on USHypoechoic on US– Low density on CTLow density on CT


Salivary glandsSalivary glands– All may be involvedAll may be involved Parotid most commonParotid most common– MALT most commonMALT most common– Single or multiple massesSingle or multiple masses– High density on CTHigh density on CT– Hypoechoic on USHypoechoic on US– Common in middle aged Common in middle aged womanwoman– Assoc with Sjogren’s Assoc with Sjogren’s diseasedisease

Musculoskeletal Musculoskeletal SystemSystem

Involvement of the bone, bone Involvement of the bone, bone marrow and skeletal muscles can marrow and skeletal muscles can occur in both HD and NHLoccur in both HD and NHL NB sites of disease relapse NB sites of disease relapse


Bone marrowBone marrow– Lymphoma may arise within the marrow as true Lymphoma may arise within the marrow as true primary diseaseprimary disease– Stage 1E (present in 50-80% low grade NHL)Stage 1E (present in 50-80% low grade NHL)– Stage 4 if with widespread diseaseStage 4 if with widespread disease– In high grade NHL, marrow involvement is In high grade NHL, marrow involvement is present in 20–40% - poorer prognosis than liver present in 20–40% - poorer prognosis than liver and lungand lung– BM biopsy NB in all stagingBM biopsy NB in all staging– In HD, marrow involvement at presentation is In HD, marrow involvement at presentation is rare (5-15% during course)rare (5-15% during course)


Bone marrowBone marrow– MRI – extremely sensitive in MRI – extremely sensitive in detecting bone marrow involvementdetecting bone marrow involvement Low signal intensity on T1-weighted Low signal intensity on T1-weighted images and high signal on STIR images and high signal on STIR sequencessequences – FDG-PET is also very sensitive for FDG-PET is also very sensitive for bone marrow involvement at bone marrow involvement at presentationpresentation


BoneBone– Primary lymphoma of bone is nearly all NHL Primary lymphoma of bone is nearly all NHL (1% of NHL) - DLBCL(1% of NHL) - DLBCL– < 5% of bone malignancy< 5% of bone malignancy– Appendicular skeleton in 1 cases – eg. femur⁰Appendicular skeleton in 1 cases – eg. femur⁰– Most cases secondary and HD (10-25%)Most cases secondary and HD (10-25%)– Average age at presentation is 24 yearsAverage age at presentation is 24 years– Males>femalesMales>females– NHL lytic, HD sclerotic(skull, spine – ivory NHL lytic, HD sclerotic(skull, spine – ivory verteb)verteb)


BoneBone– Criteria for dx of primary lymphomaCriteria for dx of primary lymphoma Only a single bone is involvedOnly a single bone is involved There is unequivocal histological There is unequivocal histological evidence of lymphomaevidence of lymphoma Other disease is limited to regional Other disease is limited to regional areas at the time of presentationareas at the time of presentation The primary tumour precedes The primary tumour precedes metastases by at least 6 monthsmetastases by at least 6 months


BoneBone– Imaging features of osseous lymphoma at conventional Imaging features of osseous lymphoma at conventional radiography, CT, and MR imaging are nonspecificradiography, CT, and MR imaging are nonspecific– Radionuclide radiology has a sensitivity of close to 95% Radionuclide radiology has a sensitivity of close to 95% in the detection of bone involvement, far greater than in the detection of bone involvement, far greater than plain radiographyplain radiography– FDG PET has proved to be more specific and sensitive FDG PET has proved to be more specific and sensitive than conventional bone scintigraphy in identifying than conventional bone scintigraphy in identifying osseous involvement by malignant lymphoma osseous involvement by malignant lymphoma – MRI extraosseous and marrow involvement (1 bone ⁰MRI extraosseous and marrow involvement (1 bone ⁰Lymphoma staging)Lymphoma staging)


BoneBone


Cutaneous LymphomaCutaneous Lymphoma– Primary cutaneous lymphoma is the second Primary cutaneous lymphoma is the second most prominent group of NHLs, whereas most prominent group of NHLs, whereas primary or secondary skin involvement is very primary or secondary skin involvement is very rare in patients with Hodgkin disease.rare in patients with Hodgkin disease.– Almost 65% of primary cutaneous lymphomas Almost 65% of primary cutaneous lymphomas are T-cell lymphomas, with the remainder being are T-cell lymphomas, with the remainder being B-cell lymphomasB-cell lymphomas– About 25% of primary cutaneous lymphomas About 25% of primary cutaneous lymphomas demonstrate extracutaneous involvement at the demonstrate extracutaneous involvement at the time of diagnosistime of diagnosis

Mucosa Associated Mucosa Associated Lymphoid Tissue LymphomaLymphoid Tissue Lymphoma

From mucosal sites that normally have no From mucosal sites that normally have no organized lymphoid tissueorganized lymphoid tissue– Lymphoid tissue arise as a result of Lymphoid tissue arise as a result of chronic inflammation or autoimmunitychronic inflammation or autoimmunity– Hashimoto's thyroiditisHashimoto's thyroiditis 70x increased risk for thyroid lymphoma70x increased risk for thyroid lymphoma– Sjögren's syndromeSjögren's syndrome 44x increased risk for lymphoma44x increased risk for lymphoma– HelicobacterHelicobacter-induced chronic follicular -induced chronic follicular gastritisgastritis

Mucosa Associated Mucosa Associated Lymphoid Tissue LymphomaLymphoid Tissue Lymphoma

Often Adults - median age 60 Often Adults - median age 60 BM involved in 20%BM involved in 20% Multiple sites in 10%Multiple sites in 10% Most common siteMost common site– GIT (50%)GIT (50%)

Stomach (85%)Stomach (85%)– OthersOthers lung, head & neck, ocular adnexae, lung, head & neck, ocular adnexae, skin, and breastskin, and breast

Burkitt’s LymphomaBurkitt’s Lymphoma

Highly aggressive B-cell variant of Highly aggressive B-cell variant of NHLNHL Associated with Epstein–Barr virus Associated with Epstein–Barr virus Extremely aggressive, rapidly growing Extremely aggressive, rapidly growing but potentially curablebut potentially curable 2–3% of NHL2–3% of NHL 30–50% of all childhood lymphoma30–50% of all childhood lymphoma


1.1. Endemic formEndemic form– Jaws and orbit are involved in 50% of casesJaws and orbit are involved in 50% of cases– Ovaries, kidneys and breast may be involvedOvaries, kidneys and breast may be involved2.2. Sporadic formSporadic form– Predilection for the ileocaecal region (present Predilection for the ileocaecal region (present with intussusception)with intussusception)– Also ovaries, kidneys and breastsAlso ovaries, kidneys and breasts– Retroperitoneal and paraspinal disease can Retroperitoneal and paraspinal disease can cause paraplegiacause paraplegia– Thoracic disease rareThoracic disease rare


3.3. Immunodeficiency associated BLImmunodeficiency associated BL– Association with HIV infectionAssociation with HIV infection– Initial manifestation of AIDSInitial manifestation of AIDS– EBV is identified in up to 40% of casesEBV is identified in up to 40% of cases Extranodal disease is common and all three Extranodal disease is common and all three variants are at risk for CNS diseasevariants are at risk for CNS disease

ImmunosuppressionImmunosuppression

Four WHO categoriesFour WHO categories– Primary immunodeficiencyPrimary immunodeficiency– Infection with HIVInfection with HIV– Immunosuppresion following Immunosuppresion following transplanttransplant– Immunosupression for autoimmune Immunosupression for autoimmune disordersdisorders


Lymphoma and HIVLymphoma and HIV– AIDS defining in 5%AIDS defining in 5%– Incidence of all subtypes of NHL is Incidence of all subtypes of NHL is increased 60–200xincreased 60–200x– Incidence of HD increased up to 8xIncidence of HD increased up to 8x– Tumours are aggresive, advancedTumours are aggresive, advanced– Involve GIT, CNS(multiple), Liver & BMInvolve GIT, CNS(multiple), Liver & BM– Multiple EN sites in 75%Multiple EN sites in 75%


Post transplant Post transplant lymphoproliferative disorderlymphoproliferative disorder– Low risk 1% ( renal and BM Low risk 1% ( renal and BM transplant )transplant )– Highest 5% (heart-lung, liver-bowel)Highest 5% (heart-lung, liver-bowel)– Onset and site of EN disease Onset and site of EN disease correlate to transplant type and type correlate to transplant type and type of suppressionof suppression Eg. Cyclosporine affect GIT > CNSEg. Cyclosporine affect GIT > CNS

RelapseRelapse

10-40% of pt’s with HD10-40% of pt’s with HD– Within 1Within 1stst 2 yrs 2 yrs 50% of pt’s with NHL50% of pt’s with NHL Follow up CT according to Follow up CT according to institution guideliness, pt’s institution guideliness, pt’s symptomssymptoms Functional imaging can detect Functional imaging can detect relapse earlier than CTrelapse earlier than CT

Post Treatment Post Treatment EvaluationEvaluation

Imaging plays a critical role in monitoring Imaging plays a critical role in monitoring response to treatment and in assessing the response to treatment and in assessing the degree of responsedegree of response Lesions to be measured before, during and Lesions to be measured before, during and after treatmentafter treatment Well defined lesions either side of the Well defined lesions either side of the diaphragmdiaphragm


Response criteriaResponse criteria– HDHD Complete response when no clinical or Complete response when no clinical or radiological evidence of diseaseradiological evidence of disease Complete remissionComplete remission– No clinical evidenceNo clinical evidence– Radiologcal abn not related to RxRadiologcal abn not related to Rx– Additional imaging or biopsyAdditional imaging or biopsy


Reponse criteriaReponse criteria– NHLNHL Comple remissionComple remission– No clinical or Rad evidence of diseaseNo clinical or Rad evidence of disease– Nodes of >1.5cm now <1.5cm or 1-1.5 cm Nodes of >1.5cm now <1.5cm or 1-1.5 cm nodes <1cmnodes <1cm– Spleen normal sized with resolution of Spleen normal sized with resolution of lesionslesions– Marrow if involved, now cleared (BM biopsy Marrow if involved, now cleared (BM biopsy proven)proven)


Response CriteriaResponse Criteria– NHLNHL Complete remission – unconfirmedComplete remission – unconfirmed– Residual mass >1.5cm reduction of 75% in SPDResidual mass >1.5cm reduction of 75% in SPD Partial responsePartial response– > 50% reduction in SPD of all lesions> 50% reduction in SPD of all lesions– No progressionNo progression ProgressionProgression– > 50% increase in SPD of mass> 50% increase in SPD of mass– New lesionNew lesion


CTCT– Imaging characteristics of extranodal involvement can Imaging characteristics of extranodal involvement can be subtle or absent at conventional computed be subtle or absent at conventional computed tomography tomography – Response criteriaResponse criteria RemmisionRemmision Residual diseaseResidual disease ProgressionProgression– Unable to diff Rx changes from residual on densityUnable to diff Rx changes from residual on density– Limited sensitivity in detecting lymphomatous Limited sensitivity in detecting lymphomatous involvement of normal-sized lymph nodes, bone involvement of normal-sized lymph nodes, bone marrow, spleen, and extranodal tissues.marrow, spleen, and extranodal tissues.


MRIMRI– Differentiate active tumour from Differentiate active tumour from necrosisnecrosis– Tumours often high signal intensity Tumours often high signal intensity on T2on T2 Residual or recurrent disease can be IDResidual or recurrent disease can be ID– Difficult in small lesionsDifficult in small lesions– False positive early with inflammation and False positive early with inflammation and necrosisnecrosis


FDG-PETFDG-PET– Has facilitated the identification of affected Has facilitated the identification of affected extranodal sites, even when CT has extranodal sites, even when CT has demonstrated no lesionsdemonstrated no lesions– Predict eventual outcome of NHL (early – after Predict eventual outcome of NHL (early – after 1-3 Cycles of Chemo) more accurately than 1-3 Cycles of Chemo) more accurately than PETPET– Ineffective Rx can be changed earlyIneffective Rx can be changed early– Sensitivity and specificity for the detection of residual Sensitivity and specificity for the detection of residual disease after completion of first-line therapy are 84% disease after completion of first-line therapy are 84% and 90%, respectively, for Hodgkin disease, and 72% and 90%, respectively, for Hodgkin disease, and 72% and 100%, respectively, for aggressive NHand 100%, respectively, for aggressive NH


FDG UptakeFDG Uptake


PET-CTPET-CT– Certain PET/CT patterns are suggestive of Certain PET/CT patterns are suggestive of extranodal disease and can help differentiate extranodal disease and can help differentiate tumor from normal physiologic FDG activity, tumor from normal physiologic FDG activity, particularly in the mucosal tissues, bone particularly in the mucosal tissues, bone marrow, and organs of the gastrointestinal marrow, and organs of the gastrointestinal tracttract– Standard imaging modality for initial staging, Standard imaging modality for initial staging, follow-up, and treatment response in some follow-up, and treatment response in some centre’scentre’s– NHL & HD upstaged in 31%NHL & HD upstaged in 31%


Radionuclide ImagingRadionuclide Imaging– Gallium-67 can be usedGallium-67 can be used Taken up by tumour and not in fibrotic Taken up by tumour and not in fibrotic or necrotic tissueor necrotic tissue ⇩ ⇩ uptake – good response and the uptake – good response and the reversereverse False +, in thymic hyperplasia, False +, in thymic hyperplasia, inflammation and benign uptakeinflammation and benign uptake

PET-CT vs CE CTPET-CT vs CE CT

Take Home PointsTake Home Points

Most common extranodal sites of Most common extranodal sites of involvement are the stomach, spleen, involvement are the stomach, spleen, Waldeyer ring, central nervous system, lung, Waldeyer ring, central nervous system, lung, bone, and skinbone, and skin Diagnosis of primary versus secondary Diagnosis of primary versus secondary extranodal lymphoma remains challengingextranodal lymphoma remains challenging FDG PET/CT is now the proposed imaging FDG PET/CT is now the proposed imaging modality of choice for staging and follow-up modality of choice for staging and follow-up in Hodgkin disease and most NHLsin Hodgkin disease and most NHLs

Take Home PointsTake Home Points

It is important to determine whether It is important to determine whether extranodal involvement represents a extranodal involvement represents a primary manifestation or primary manifestation or dissemination of systemic disease, dissemination of systemic disease, which has a poorer prognosiswhich has a poorer prognosis At any stage during the course of the At any stage during the course of the disease, the potential presence of disease, the potential presence of extranodal involvement should be extranodal involvement should be consideredconsidered

ReferencesReferences

Grainger & Allison – Diagnostic Radiology 5Grainger & Allison – Diagnostic Radiology 5thth Ed; Ed; pages 1740-1757pages 1740-1757 Paes, Kalkanis, Sideras, Serafini. FDG PET/CT of Paes, Kalkanis, Sideras, Serafini. FDG PET/CT of Extranodal Involvement in Non-Hodgkin Extranodal Involvement in Non-Hodgkin Lymphoma and Hodgkin Disease. January 2010 Lymphoma and Hodgkin Disease. January 2010 RadioGraphics, 30, 269-291. RadioGraphics, 30, 269-291. Raanani P, Shasha Y, Perry C, et al. Is CT scan Raanani P, Shasha Y, Perry C, et al. Is CT scan still necessary for staging in Hodgkin and non-still necessary for staging in Hodgkin and non-Hodgkin lymphoma patients in the PET/CT era? Hodgkin lymphoma patients in the PET/CT era? Ann Oncol 2006;17:117–122.Ann Oncol 2006;17:117–122.

ReferencesReferences

Guermazi, Brice, DebKerviler, Fermé. Guermazi, Brice, DebKerviler, Fermé. Extranodal Hodgkin Disease: Spectrum of Extranodal Hodgkin Disease: Spectrum of Disease January 2001 RadioGraphics, 21, Disease January 2001 RadioGraphics, 21, 161-179161-179 Kwee TC, Kwee RM, Nievelstein RA. Imaging Kwee TC, Kwee RM, Nievelstein RA. Imaging in staging of malignant lymphoma: a in staging of malignant lymphoma: a systematic review. Blood 2008;111:504–516systematic review. Blood 2008;111:504–516

multisystem disease 2 lymphoma

Documents

hd secondary

rx radiation mediastinal

multisystem disease

frequency of nhl

high grade nhl

mediastinal lymphadenopathy10

large bcell lymphomasdirect

hdprimary hepatic disease