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Multisystem Disease 2 Lymphoma

Multisystem Disease 2 Lymphoma. A Swartbooi Clinical Imaging Sciences 28 September 2012. Extranodal Lymphoma. Lymphomatous infiltration of anatomic sites other than the lymph nodes Primary and secondary involvement Occur in both HD and NHL More common in NHL. Extranodal Lymphoma.

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Multisystem Disease 2 Lymphoma

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  1. Multisystem Disease 2 Lymphoma A Swartbooi Clinical Imaging Sciences 28 September 2012

  2. Extranodal Lymphoma • Lymphomatous infiltration of anatomic sites other than the lymph nodes • Primary and secondary involvement • Occur in both HD and NHL • More common in NHL

  3. Extranodal Lymphoma • Primary involvement • 30-40% of cases • Lymph nodal involvement limited to regional nodes • Stage I or II disease • 1⁰ extranodal HD ext rare – disease involvement elsewhere for exclusion • Increased incidence noted as frequency of NHL is seen • Seen esp of CNS and Orbits

  4. Extranodal Lymphoma • Primary involvement • Incidence of NHL • Age of pt (⇧ children) • Immune status (⇧ with ⇩ CD4) • More aggressive subtype • Pathological subtype • Mantle cell lymphoma • Lymphoblastic lymphoma • Burkitt lymphoma • MALT lymphoma

  5. Extranodal Lymphoma • Secondary involvement • Presence of widespread advanced disease elsewhere • Both HD and NHL • NHL more common • Adverse prognostic factor • Stage III or IV disease

  6. Thorax • Pulmonary parenchyma • 2⁰ Involvement • HD more common 12% than NHL 4% • Intrathoracic LN • Extrathoracic LN • Direct ext of nodal disease • Paramediastinal / Perihilar lung involvement

  7. Thorax

  8. Thorax • Pulmonary parenchyma • Recurrence after previously Rx (radiation) mediastinal HD often confined to lungs • In compar pt with NHL and pulmonary or pleural disease • No mediastinal nodes in 50% • As disease progress/relapse • Lung disease become common • Pt with HD 30-40% have pulmon involvement

  9. Thorax • Pulmonary parenchyma • 1⁰ Involvement • < 1% of lymhoma • Primary NHL • Usually low grade B-cell NHL (80-85%) • Commonly single lung nodule • Multiple nodules or segm consolidation may occur • Pleural eff in 20% • High grade NHL (15-20%) • Rapidly enlarging single or multiple nodules

  10. Thorax • Pulmonary parenchyma • 1⁰ Involvement • Primary HD • Extremely rare • Single or multiple nodules • Upper lobe • Cavitate • Pleural eff in 20%

  11. Thorax • Pleural disease • Pleural effusions • Usually accompanied by mediastinallymphadenopathy • 10% of patients with NHL and 7% of patients with HD • Secondary to central lymphatic or venous obstruction • Rx of mediastinal disease • Pleural masses more common in relapse than presentation

  12. Thorax

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

  14. Thorax • Thymus • 1⁰ Thymic HD rare • Involvement noted in assoc with mediastinal nodes in 30-50% • Often large B-cell lymphoma • Young woman (25-40yrs) • Typically rapidly growing with SVC obstr.(40%) • Thymus homogenous or heterogenous; may be nodular (lobulated) or smooth

  15. Thorax • Thymus

  16. Thorax • Chest wall • From direct extension • Ant Mediastinal nodes • Ax & Supra clav nodes • No bony destruction • Infection or Malignancy

  17. Breast • Widespread disease elsewhere • NHL of breast rare • 2% lymphomas; 0.5% breast malignancy • Bimodal • Pregnancy and lactation • High grade or burkitt • Billat with inflammatory picture

  18. Breast

  19. Breast • Bimodal • 50yrs • Unilateral • Discrete often solitary mass • No archicteral distortion/skin thickening • No calcifications • 2⁰ involvement characterized by multiple nodules and enlarged LN’s

  20. Abdomen • Hepatobiliary system • Liver involvement • 15 % in NHL • ⇧ in Paeds and with recurrence • 5% in HD • Primary hepatic disease rare (< 1%) • Increased in pt HepA or HepC + (25%) • Infiltration around portal tracts most common • Hepatomegaly suggest diffuse involvement

  21. Abdomen • Hepatobiliary system • Liver involvement • 5-10% focal infiltration • Resemble metastases • Hypoechoiec and well defined on US • GB • NHL Rare, but ⇧ frequency in patients with ARL

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

  23. Abdomen • Spleen • 33% splenomeg – no infiltration • 33% normal – infiltration • Volume + indices not used • Focal lesions 10-25% > 1 cm detected by cross sect imaging • 40% of NHL has involvement some time

  24. Abdomen • Spleen • Imaging • Solitary, miliary, multiple masses • Ddx: infection, granulomatous disease • CT & US sensitive (us >3mm) • MRI (not routine) • FDG–PET > CT or Scintigraphy • 1⁰ splenic lymphoma rare (1%)

  25. Abdomen • Spleen

  26. Abdomen • GIT • Most common site of 1⁰ EN NHL • 30-45% of all • 10% adults + 30% children • 1⁰ HD of GIT rare • 2⁰ involvement common • Direct extension, multiple sites • 1⁰ lymphoma from lamina propria + submucosa

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

  28. Abdomen • GIT • Stomach • 2–5% of all gastric tumours • Submucosa, affecting the antrum more • Imaging • Multiple nodules, some with central ulceration, or a large, fungating lesion with or without ulceration,wall thickening, luminal narrowing, enlargement of the gastric folds (barium studies or endoscopically)

  29. Abdomen • GIT • Imaging • Gastric wall thickening and accompanying nodal involvement, which is well shown on CT • Low grade – shallow ulceration • High grade – massive infiltration, masses

  30. Abdomen • GIT • Small bowel • 50% of all primary tumours of the small bowel • Term ileum frequent, duodenal rare • Multifocal in 50% • Obstructive symptoms • Bowel wall thickening is well demonstrated on CT • With infiltration alternating areas of dilatation and constriction

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

  32. Abdomen • GIT • Colon and rectum • Primary colonic lymphomas are usually of Burkitt or MALT subtypes • <0.1% of all colonic neoplasms • caecum and rectum • diffuse or segmental distribution of small nodules 0.2–2.0 cm in diameter • Mucosa intact • Polypoid mass uncommon, mimic ca unless term ileum involvement

  33. Abdomen • GIT • Advanced disease, there may be marked thickening of the colonic or rectal folds resulting in focal strictures, fissures or ulcerative masses with fistula formation • Strictures longer than ca • Obstruc and rectal bleeding

  34. Abdomen • GIT • Primary gastrointestinal lymphoma • An absence of superficial or intrathoracic lymph node enlargement • No involvement of the liver or spleen • A normal white cell count • No more than local regional lymph node enlargement

  35. Abdomen • GIT

  36. Abdomen • Pancreas • 1⁰ cases <1.3% of pancreas tumours • 2% of NHL • Indistinguishable from primary adenocarcinoma on US, CT, or MRI • 2⁰ with disease elsewhere • Direct infiltration

  37. Genito-urinary Tract • Not commonly involved at the time of presentation (<5%) • 50% of patients will have involvement of some part of the genitourinary tract at autopsy • Testicle is the most commonly involved organ, followed by the kidney and the perirenal space

  38. Genito-urinary Tract • Rarely are the bladder, prostate, uterus, vagina, or ovaries involved • 1⁰ cases rare, little lymphoid tissue

  39. Genito-urinary Tract • Kidneys • 90% of cases are in association with high-grade NHL (BL) • Often in recurrence, normal function • Multiple masses 60% • US hypoechoic • Density reversal pattern on CT • Solitary mass in 5-15%, indistinguishable RCC • NHL renal mass often no retroperit nodes • Direct infil in 25% • Vessel encasement, hilar extension

  40. Genito-urinary Tract • Kidneys

  41. Genito-urinary Tract • Testis • 5% of primary testicular tumours overall and 25–50% of those in patients over 50 years • Commonest primary tumour over the age of 60 years • 1% of all patients of NHL, usually with DLBCL or BL • Association with lymphoma of Waldeyer's ring, the skin and central nervous system • Painless testicular swelling

  42. Genito-urinary Tract • Testis • Ultrasonically, the lesions usually have a nonspecific appearance • Staging must always include ultrasonic evaluation of the contralateral testis and whole body CT

  43. Adrenal Glands • 25% of patients with NHL will develop secondary involvement of the adrenal gland • Primary adrenal lymphoma is extremely rare, usually occurring in men over the age of 60. • Secondary involvement detected in about 6% undergoing routine abdominal staging CT, in the presence of widespread retroperitoneal disease • Indistinguishable from metastases

  44. Central Nervous System • 1⁰ CNS Lymphoma • Brain and spine only • 3% of brain tumours • 30% of NHL – immunodeficiency states • 6% AIDS pt get PCNSL • Peaks 40-60yrs • Lesions often frontal (personality) • White matter close to CC (spread across) • Isointense on CT&MRI, Hom enhancement

  45. Central Nervous System • 2⁰ CNSL • 15% of NHL • In HD brain lesions often second dx • Often extra-axial and spinal spaces • MRI more sensitive (enhancing plaques) • Spinal presentation often from extension of prevertebral mass (dum-bell tumour)

  46. Central Nervous System • CNSL

  47. Central Nervous System • Orbital lymphoma • Pt’s 40-70yrs • Slow growing, infiltrating • NHL most common • 10-15% of primary orbital tumour in adults • 4% primary EN NHL • 2⁰ cases 3.5 – 5% of NHL & HD • Can involve any part, symptoms acc to site

  48. Head & Neck • Waldeyer’s ring • Commonest site (1/3 of EN NHL) • Lymphoid tissue in nasopharynx, oropharynx, faucial palatine and lingual tonsils • Circumferential or multifocal involvement • 8% Paranasal sinus tumours are NHL • Assoc with GIT involvement • Most are DLBCL • MRI preferred imaging method

  49. Head & Neck • Thyroid • NHL 2% malignant thyroid tumours • MALT type associated with Hashimoto’s • Can extend beyond gland • Hypoechoic on US • Low density on CT

  50. Head & Neck • Salivary glands • All may be involved • Parotid most common • MALT most common • Single or multiple masses • High density on CT • Hypoechoic on US • Common in middle aged woman • Assoc with Sjogren’s disease

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