Diagnostic Ultrasound - Abdomen and Pelvis
Renal Lymphoma TERMINOLOGY Abbreviations • Primary renal lymphoma (PRL); secondary renal lymphoma (SRL) Definitions • Lymphoma: Malignant tumor of lymphocytes • Primary: Involvement of kidneys without evidence of other organ or nodal involvement ○ Extremely rare; < 1% of extranodal lymphoma • Secondary: Dissemination of extrarenal lymphoma by hematogenous spread (90%) or direct extension via retroperitoneal lymphatic channels ○ Non-Hodgkin lymphoma >> Hodgkin disease IMAGING General Features • Morphology ○ Multiple hypoenhancing/hypoechoic masses (50-60%) ○ Direct extension from retroperitoneal adenopathy (25- 30%), associated hydronephrosis ○ Solitary hypoenhancing/hypoechoic mass (10-25%) ○ Bilateral renal enlargement (nephromegaly) (20%) – More common in Burkitt lymphoma ○ Perinephric disease (< 10%); rind of homogeneous perinephric soft tissue – May be limited to thickening of Gerota fascia or plaques and nodules in perirenal space ○ Renal sinus predominant: Uncommon, no vascular invasion, milder hydronephrosis Ultrasonographic Findings • Grayscale ultrasound ○ Typically hypoechoic and homogeneous mass ○ Homogeneity of lymphoma results in few tissue interfaces to insonating beam; there may even be posterior acoustic enhancement ○ Solitary or multiple masses – Small lesions may be confused with medullary pyramids, renal cysts, or abscesses ○ Direct invasion: Hypoechoic mass extending from retroperitoneum or perirenal space into renal parenchyma or sinus ○ Nephromegaly: Globular enlargement with heterogeneous echotexture and loss of normal echogenic appearance of renal sinus fat – Diffuse uniform increase in echogenicity may simulate renal parenchymal disease ○ Perirenal lymphoma: Hypoechoic soft tissue of variable thickness surrounding kidney • Color Doppler ○ Displacement of normal renal cortical vessels with little vascularity within lesions ○ Soft tissue surrounding renal hilar vessels or vena cava without significant compromise CT Findings • Mildly hyperdense to normal kidney on unenhanced CT • Hypovascular mass, unlike clear cell renal cell carcinoma (RCC) ○ Note hypovascular subtypes of RCC, such as papillary and chromophobe • Despite large infiltrative retroperitoneal and perinephric space masses, vena cava and renal vein are rarely invaded, unlike RCC • Nephromegaly with diffuse infiltration and heterogeneous enhancement • Difficult to differentiate from transitional cell carcinoma (TCC) when epicenter of disease is in renal sinus • Splenomegaly or lymphadenopathy at other sites in SRL • Extranodal involvement of gastrointestinal tract, brain, liver, and bone marrow • Calcification and cystic change are rare MR Findings • Low to intermediate signal on both T1 and T2 • Heterogeneous high signal may be seen on T2WI • May show restricted diffusion • T1 C+: Heterogenous enhancement, less than that of cortex • Ideal for detecting synchronous osseous disease Image-Guided Biopsy • Important role to differentiate from other solid renal masses and determine medical or surgical management • US is ideal for guidance, but CT may be needed for deeper lesions or lesions not visible on US • Fine-needle aspiration supplemented by core biopsies: High sensitivity and specificity ○ Immunochemistry, flow cytometry, and histopathology drive specific therapies • Renal mass biopsy not necessary in widespread lymphoma unless renal mass is atypical or patient has 2nd malignancy Nuclear Medicine Findings • PET/CT ○ Important role in evaluation of both nodal and extranodal lymphoma ○ Used for initial staging, evaluation of treatment response, and detection of recurrence in some subtypes of lymphoma ○ More sensitive than conventional anatomic imaging ○ 18F-FDG uptake much higher on average in renal lymphoma (SUV mean 6.37) compared to RCC (SUV mean 2.58) Imaging Recommendations • Best imaging tool ○ CECT is modality of choice for initial diagnosis and staging of renal lymphoma – Combined with 18F-FDG PET for initial staging, assessment of response and detection of recurrence ○ MR is alternative in patients with impaired renal function; avoids radiation exposure DIFFERENTIAL DIAGNOSIS Renal Cell Carcinoma • Round or oval cortical solid or cystic mass ±central necrosis • Typically hypervascular, however, some subtypes are hypovascular • Propensity to vascular invasion Diagnoses: Urinary Tract 509
Renal Lymphoma Diagnoses: Urinary Tract Transitional Cell Carcinoma • Centered in collecting system; may be infiltrative and extend into renal cortex; more likely to cause collecting system obstruction Metastases • Lung, breast, gastric cancer, and melanoma Other Malignant Lymphoproliferative Diseases • Myeloma:Diffuse infiltration, solitary or multiple masses • Leukemia: Enlarged kidney from diffuse infiltration Renal Infection • Pyelonephritis, renal abscess, septic emboli • May present as single or multiple lesions; differentiated by clinical history and urinalysis Perirenal Hematoma,Extramedullary Hematopoiesis,Retroperitoneal Sarcoma • May simulate perirenal lymphoma PATHOLOGY General Features • GU system commonly affected by extranodal spread of lymphoma; kidney is most commonly involved • Usually non-Hodgkin lymphoma, typically B-cell or Burkitt type; involvement by Hodgkin disease very rare • Renal tissue is devoid of lymphoid tissue; lymphatics within renal capsule, perinephric fat, or lymphocytes in areas of chronic inflammation may account for renal involvement • Modes of renal spread include hematogenous and direct contiguous extension Gross Pathologic & Surgical Features • Enlarged kidney ± distortion of renal contour • Expansion of fat caused by homogeneous yellowish tumor infiltration CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Majority are asymptomatic and renal function unaffected ○ Rarely present with acute renal failure; rapid improvement in renal function post treatment ○ Hematuria, flank pain, palpable mass, or renal insufficiency • Other signs/symptoms ○ Fever, weight loss,↑ serum lactate dehydrogenase, lymphopenia Demographics • Age ○ Any (middle-aged to elderly more common) • Gender ○ Prevalence equal in both sexes • Epidemiology ○ SRL: 30-60% silent renal disease in case of widespread lymphoma based on autopsy series; imaging manifestation occurs only in 1-8% ○ PRL: More common in middle-aged men; may present with renal failure in absence of other causes of renal impairment • Predisposing factors ○ Immunocompromised (post organ transplantation, HIV), prior treatment for malignancy,autoimmune disorders, viruses such as Epstein-Barr virus Natural History & Prognosis • Complications ○ Renal or perinephric hemorrhage, renal obstruction, renovascular hypertension, acute renal failure • Prognosis ○ Good prognosis with early diagnosis and chemotherapy ○ Tumor size > 10 cm, involvement of renal hilum and diffuse infiltration associated with poorer prognosis ○ Involvement of kidneys at time of initial presentation of B-cell lymphoma may be associated with high incidence of CNS relapse; diagnosis may alter treatment regimen Treatment • Chemotherapy ± radiation therapy DIAGNOSTIC CHECKLIST Consider • Clinical history, any other malignancy, extent of disease • Overlapping features of renal metastases, renal lymphoma, and primary renal carcinoma • Ultrasound-guided renal biopsy for definitive diagnosis Image Interpretation Pearls • Always look for evidence of multisystem involvement in liver, lung, CNS, bone marrow and gastrointestinal tract SELECTED REFERENCES 1. Ganeshan D et al: Imaging of primary and secondary renal lymphoma. AJR Am J Roentgenol. 201(5):W712-9, 2013 2. Kostakoglu L et al: State-of-the-Art Research on "Lymphomas: Role of Molecular Imaging for Staging, Prognostic Evaluation, and Treatment Response". Front Oncol. 3:212, 2013 3. Bach AG et al: Prevalence and patterns of renal involvement in imaging of malignant lymphoproliferative diseases. Acta Radiol. 53(3):343-8, 2012 4. Ye XH et al: 18F-FDG PET/CT evaluation of lymphoma with renal involvement: comparison with renal carcinoma. South Med J. 103(7):642-9, 2010 5. El-Sharkawy MS et al: Renal involvement in lymphoma: prevalence and various patterns of involvement on abdominal CT. Int Urol Nephrol. 39(3):929-33, 2007 6. Sheth S et al: Imaging of renal lymphoma: patterns of disease with pathologic correlation. Radiographics. 26(4):1151-68, 2006 510
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Renal Lymphoma<br />
Diagnoses: Urinary Tract<br />
Transitional Cell Carcinoma<br />
• Centered in collecting system; may be infiltrative <strong>and</strong><br />
extend into renal cortex; more likely to cause collecting<br />
system obstruction<br />
Metastases<br />
• Lung, breast, gastric cancer, <strong>and</strong> melanoma<br />
Other Malignant Lymphoproliferative Diseases<br />
• Myeloma:Diffuse infiltration, solitary or multiple masses<br />
• Leukemia: Enlarged kidney from diffuse infiltration<br />
Renal Infection<br />
• Pyelonephritis, renal abscess, septic emboli<br />
• May present as single or multiple lesions; differentiated by<br />
clinical history <strong>and</strong> urinalysis<br />
Perirenal Hematoma,Extramedullary<br />
Hematopoiesis,Retroperitoneal Sarcoma<br />
• May simulate perirenal lymphoma<br />
PATHOLOGY<br />
General Features<br />
• GU system commonly affected by extranodal spread of<br />
lymphoma; kidney is most commonly involved<br />
• Usually non-Hodgkin lymphoma, typically B-cell or Burkitt<br />
type; involvement by Hodgkin disease very rare<br />
• Renal tissue is devoid of lymphoid tissue; lymphatics within<br />
renal capsule, perinephric fat, or lymphocytes in areas of<br />
chronic inflammation may account for renal involvement<br />
• Modes of renal spread include hematogenous <strong>and</strong> direct<br />
contiguous extension<br />
Gross Pathologic & Surgical Features<br />
• Enlarged kidney ± distortion of renal contour<br />
• Expansion of fat caused by homogeneous yellowish tumor<br />
infiltration<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Majority are asymptomatic <strong>and</strong> renal function<br />
unaffected<br />
○ Rarely present with acute renal failure; rapid<br />
improvement in renal function post treatment<br />
○ Hematuria, flank pain, palpable mass, or renal<br />
insufficiency<br />
• Other signs/symptoms<br />
○ Fever, weight loss,↑ serum lactate dehydrogenase,<br />
lymphopenia<br />
Demographics<br />
• Age<br />
○ Any (middle-aged to elderly more common)<br />
• Gender<br />
○ Prevalence equal in both sexes<br />
• Epidemiology<br />
○ SRL: 30-60% silent renal disease in case of widespread<br />
lymphoma based on autopsy series; imaging<br />
manifestation occurs only in 1-8%<br />
○ PRL: More common in middle-aged men; may present<br />
with renal failure in absence of other causes of renal<br />
impairment<br />
• Predisposing factors<br />
○ Immunocompromised (post organ transplantation, HIV),<br />
prior treatment for malignancy,autoimmune disorders,<br />
viruses such as Epstein-Barr virus<br />
Natural History & Prognosis<br />
• Complications<br />
○ Renal or perinephric hemorrhage, renal obstruction,<br />
renovascular hypertension, acute renal failure<br />
• Prognosis<br />
○ Good prognosis with early diagnosis <strong>and</strong> chemotherapy<br />
○ Tumor size > 10 cm, involvement of renal hilum <strong>and</strong><br />
diffuse infiltration associated with poorer prognosis<br />
○ Involvement of kidneys at time of initial presentation of<br />
B-cell lymphoma may be associated with high incidence<br />
of CNS relapse; diagnosis may alter treatment regimen<br />
Treatment<br />
• Chemotherapy ± radiation therapy<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• Clinical history, any other malignancy, extent of disease<br />
• Overlapping features of renal metastases, renal lymphoma,<br />
<strong>and</strong> primary renal carcinoma<br />
• <strong>Ultrasound</strong>-guided renal biopsy for definitive diagnosis<br />
Image Interpretation Pearls<br />
• Always look for evidence of multisystem involvement in<br />
liver, lung, CNS, bone marrow <strong>and</strong> gastrointestinal tract<br />
SELECTED REFERENCES<br />
1. Ganeshan D et al: Imaging of primary <strong>and</strong> secondary renal lymphoma. AJR<br />
Am J Roentgenol. 201(5):W712-9, 2013<br />
2. Kostakoglu L et al: State-of-the-Art Research on "Lymphomas: Role of<br />
Molecular Imaging for Staging, Prognostic Evaluation, <strong>and</strong> Treatment<br />
Response". Front Oncol. 3:212, 2013<br />
3. Bach AG et al: Prevalence <strong>and</strong> patterns of renal involvement in imaging of<br />
malignant lymphoproliferative diseases. Acta Radiol. 53(3):343-8, 2012<br />
4. Ye XH et al: 18F-FDG PET/CT evaluation of lymphoma with renal<br />
involvement: comparison with renal carcinoma. South Med J. 103(7):642-9,<br />
2010<br />
5. El-Sharkawy MS et al: Renal involvement in lymphoma: prevalence <strong>and</strong><br />
various patterns of involvement on abdominal CT. Int Urol Nephrol.<br />
39(3):929-33, 2007<br />
6. Sheth S et al: Imaging of renal lymphoma: patterns of disease with<br />
pathologic correlation. Radiographics. 26(4):1151-68, 2006<br />
510