Diagnostic Ultrasound - Abdomen and Pelvis
Splenic Tumors TERMINOLOGY Definitions • Space occupying benign or malignant tumor(s) of spleen IMAGING General Features • Best diagnostic clue ○ Solitary or multiple, solid or cystic splenic masses • Key concepts ○ Classification based on pathology and histology; overlap of imaging appearances ○ Benign tumors – Hemangioma, hamartoma, lymphangioma, littoral cell angioma ○ Hemangioma – #1 primary benign neoplasm of spleen (up to 14%) – Typically small, incidental, asymptomatic – Multiple in diffuse splenic hemangiomatosis or syndromes (Klippel-Trenaunay-Weber and Beckwith- Wiedemann syndrome) ○ Hamartoma – Rare; incidentally detected at autopsy or imaging; no age/gender predilection – Contains anomalous mixture of normal elements of splenic tissue – Syndromic associations: Tuberous sclerosis and Wiskott-Aldrich syndrome ○ Lymphangioma – Rare; most occur in childhood; variable size – Uni- or multilocular; typically subcapsular in location – Solitary or multiple (as in systemic lymphangiomatosis) ○ Littoral cell angioma (LCA) – Rare 1° vascular tumor; commonly considered benign, though malignant LCAs have been reported – Solitary or multiple; usually presents with splenomegaly, hypersplenism ○ Malignant tumors – Lymphoma, leukemia, myeloproliferative disorders – Metastases – Rare 1° splenic malignancies: Angiosarcoma, leiomyosarcoma, malignant fibrous histiocytoma ○ Lymphoma – #1 malignant tumor of spleen: Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL) – Spleen: Nodal organ in HD and extranodal organ in NHL – Manifest: Focal lesions (> 1 cm) or diffuse (typical) – Primary splenic lymphoma: Typically NHL (B-cell origin) ○ Metastases – Relatively uncommon; may be multiple (60%), solitary (31%), nodular, and diffuse (9%) – Common route: Hematogenous spread (splenic artery) □ Retrograde (less common): Via splenic vein and lymphatics □ Direct extension (uncommon): From gastric, renal, pancreatic, colonic – Common primary sites for splenic metastases: Breast (21%), lung (18%), ovary (8%), stomach (7%), melanoma (6%), prostate (6%) – "Cystic" splenic metastases: Melanoma; adenocarcinoma of breast, ovary, and endometrium ○ Angiosarcoma – Very rare malignant tumor of spleen; seen in patients with previous exposure to Thorotrast – Poor prognosis with early, widespread metastases Ultrasonographic Findings • Grayscale ultrasound ○ Benign tumors ○ Hemangioma – Typically well defined, hyperechoic – Echogenicity can be variable (range from solid to cystic to mixed); ± calc when complex – Rarely, can be large and involve entire spleen with atypical features: Heterogeneous echotexture, areas of necrosis and hemorrhage ○ Hamartoma – Typically well defined, homogeneous, hyperechoic – Depending on histologic subtype, echogenicity and vascularity can be variable; can have cystic component or calc ○ Lymphangioma – Normal or enlarged spleen depending on number, size, loculations – Well-defined hypoechoic mass ± internal septations and intralocular debris; ± wall calc – Avascular on color Doppler, unless along cyst walls ○ Littoral cell angioma – Variable echogenicity and vascularity; solitary or multiple; splenomegaly ○ Malignant tumors ○ Lymphoma – US pattern corresponds to 3 macroscopic patterns; diffuse/infiltrative, miliary/nodular, focal hypoechoic – "Indistinct boundary" echo pattern – Anechoic/mixed echoic, small or large nodules; hyperechoic lesion uncommon (< 10%) – Lymphadenopathy: Abdominal or retroperitoneal ○ Leukemia and myeloproliferative disorders – Diffuse enlargement of spleen with variable echogenicity, very rarely focal hypoechoic nodular lesions ○ Metastases – Multiple focal lesions with variable size and appearance; iso-/hypo-/hyperechoic – "Target" lesions with hypoechoic "halo" ○ Angiosarcoma: Very rare, solid, mixed echogenicity mass; associated metastasis in liver (70%) CT Findings • Benign tumors ○ Hemangioma – Homogeneous, hypodense, solid or cystic masses – Concentric rim of C+ with uniform delayed fill-in; classic "peripheral nodular C+" associatedwith liver hemangioma is less common in spleen ○ Hamartoma Diagnoses: Spleen 409
Splenic Tumors Diagnoses: Spleen – Isodense (typically) or hypodense;variable early C+; uniform C+ on delayed scans ○ Lymphangioma – Low-attenuation lesion(s); sharp margins; typically subcapsular; no enhancement;± wall calcification • Malignant tumors ○ Lymphoma – Solitary, multifocal or diffuse; Hypodense lesions with minimal enhancement ○ Metastases – Multiple, solid (common) or cystic, hypodense, central or peripheral enhancement ○ Angiosarcoma – Solitary or multiple, irregular margins, heterogeneous density; variable enhancement;± calcification – Usually liver or distant metastases MR Findings • Benign tumors ○ Hemangioma: T1 ↓, T2 ↑; C+: uniform or heterogeneous centripetal ○ Hamartoma: T1 iso; T2 hetero; C+: similar to CT ○ Lymphangioma: T1 ↓ or ↑ (depending on protein/blood content) , T2 ↑↑; No C+ • Malignant tumors ○ Lymphoma – MR not reliable due to similar T1, T2 relaxation times and proton densities of spleen/lymphoma ○ Metastases – T1WI: Isointense to hypointense, T2WI: Hyperintense – T1 C+: Enhancement depends on type of primary Nuclear Medicine Findings • PET/CT ○ 18F-FDG avidity can be a helpful feature to distinguish between benign and malignant solid splenic lesions – In patients with known malignancy, SUV > 2.3 can differentiate benign and malignant with 100% sens/spec – High NPV in patients without known malignancy – False-positives can be due to granulomatous diseases Image-Guided Biopsy • Splenic biopsy is gaining increased acceptance ○ Risks include hemorrhage (leading to hypotensive shock), pneumothorax, or colonic injury • Ultrasound or CT used; US may be preferred due to real time assessment of the vessels with color Doppler • Splenic FNA: Low complication rate but lower success rate • Splenic core biopsy: Higher diagnostic success rate (> 88%) but higher complication rate (up to 12% reported) Imaging Recommendations • Best imaging tool ○ US initially, CT/MR for further characterization • Protocol advice ○ Patient is best scanned in supine or right decubitus position for intercostal acoustic window DIFFERENTIAL DIAGNOSIS Splenic Infarct • Wedge-shaped, well-defined, hyperechoic/hypoechoic area (depending on the age of infarct), avascular Splenic Infection/Abscess • Pyogenic abscess:Solitary or multiple, small or large, hypoechoic lesions with ± thick irregular walls • Fungal microabscesses; mycobacterial (TB, MAC) granulomas: Multiple hypoechoic avascular lesions Splenic Cyst • Anechoic/hypoechoic, sharp margins, posterior acoustic enhancement, ± peripheral rim calcification, avascular Splenic Hematoma • Subcapsular of intrasplenic; hyper/hypoechoic (depending on stage of liquefaction) fluid collection/lesion Hepatosplenic Sarcoidosis • Splenic involvement uncommon; SMG; multiple hypoechoic nodules (or iso/hyper) CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Varies from asymptomatic incidental finding to symptomatic depending on size, type of tumor ○ Left upper quadrant pain, palpable mass, splenomegaly, weight loss Natural History & Prognosis • Complications: Hemorrhage, rupture • Prognosis: Good (benign tumors); poor (malignant) Treatment • Splenectomy depending on type (can also be diagnostic if image guided biopsy not possible) DIAGNOSTIC CHECKLIST Consider • 1° splenic malignancies are rare; biggest diagnostic dilemma is usually an indeterminate splenic lesion in patients with extrasplenic malignancy (i.e., is it metastasis) Image Interpretation Pearls • Considerable overlap in US findings; reliable differentiation on imaging is not always possible, requires histology SELECTED REFERENCES 1. Gaetke-Udager K et al: Multimodality imaging of splenic lesions and the role of non-vascular, image-guided intervention. Abdom Imaging. 39(3):570-87, 2014 2. Thipphavong S et al: Nonneoplastic, benign, and malignant splenic diseases: cross-sectional imaging findings and rare disease entities. AJR Am J Roentgenol. 203(2):315-22, 2014 3. Kamaya A et al: Multiple lesions of the spleen: differential diagnosis of cystic and solid lesions. Semin Ultrasound CT MR. 27(5):389-403, 2006 4. Bachmann C et al: Color Doppler sonographic findings in focal spleen lesions. Eur J Radiol. 56(3):386-90, 2005 410
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Splenic Tumors<br />
Diagnoses: Spleen<br />
– Isodense (typically) or hypodense;variable early C+;<br />
uniform C+ on delayed scans<br />
○ Lymphangioma<br />
– Low-attenuation lesion(s); sharp margins; typically<br />
subcapsular; no enhancement;± wall calcification<br />
• Malignant tumors<br />
○ Lymphoma<br />
– Solitary, multifocal or diffuse; Hypodense lesions with<br />
minimal enhancement<br />
○ Metastases<br />
– Multiple, solid (common) or cystic, hypodense, central<br />
or peripheral enhancement<br />
○ Angiosarcoma<br />
– Solitary or multiple, irregular margins, heterogeneous<br />
density; variable enhancement;± calcification<br />
– Usually liver or distant metastases<br />
MR Findings<br />
• Benign tumors<br />
○ Hemangioma: T1 ↓, T2 ↑; C+: uniform or<br />
heterogeneous centripetal<br />
○ Hamartoma: T1 iso; T2 hetero; C+: similar to CT<br />
○ Lymphangioma: T1 ↓ or ↑ (depending on protein/blood<br />
content) , T2 ↑↑; No C+<br />
• Malignant tumors<br />
○ Lymphoma<br />
– MR not reliable due to similar T1, T2 relaxation times<br />
<strong>and</strong> proton densities of spleen/lymphoma<br />
○ Metastases<br />
– T1WI: Isointense to hypointense, T2WI: Hyperintense<br />
– T1 C+: Enhancement depends on type of primary<br />
Nuclear Medicine Findings<br />
• PET/CT<br />
○ 18F-FDG avidity can be a helpful feature to distinguish<br />
between benign <strong>and</strong> malignant solid splenic lesions<br />
– In patients with known malignancy, SUV > 2.3 can<br />
differentiate benign <strong>and</strong> malignant with 100%<br />
sens/spec<br />
– High NPV in patients without known malignancy<br />
– False-positives can be due to granulomatous diseases<br />
Image-Guided Biopsy<br />
• Splenic biopsy is gaining increased acceptance<br />
○ Risks include hemorrhage (leading to hypotensive<br />
shock), pneumothorax, or colonic injury<br />
• <strong>Ultrasound</strong> or CT used; US may be preferred due to real<br />
time assessment of the vessels with color Doppler<br />
• Splenic FNA: Low complication rate but lower success rate<br />
• Splenic core biopsy: Higher diagnostic success rate (> 88%)<br />
but higher complication rate (up to 12% reported)<br />
Imaging Recommendations<br />
• Best imaging tool<br />
○ US initially, CT/MR for further characterization<br />
• Protocol advice<br />
○ Patient is best scanned in supine or right decubitus<br />
position for intercostal acoustic window<br />
DIFFERENTIAL DIAGNOSIS<br />
Splenic Infarct<br />
• Wedge-shaped, well-defined, hyperechoic/hypoechoic area<br />
(depending on the age of infarct), avascular<br />
Splenic Infection/Abscess<br />
• Pyogenic abscess:Solitary or multiple, small or large,<br />
hypoechoic lesions with ± thick irregular walls<br />
• Fungal microabscesses; mycobacterial (TB, MAC)<br />
granulomas: Multiple hypoechoic avascular lesions<br />
Splenic Cyst<br />
• Anechoic/hypoechoic, sharp margins, posterior acoustic<br />
enhancement, ± peripheral rim calcification, avascular<br />
Splenic Hematoma<br />
• Subcapsular of intrasplenic; hyper/hypoechoic (depending<br />
on stage of liquefaction) fluid collection/lesion<br />
Hepatosplenic Sarcoidosis<br />
• Splenic involvement uncommon; SMG; multiple hypoechoic<br />
nodules (or iso/hyper)<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Varies from asymptomatic incidental finding to<br />
symptomatic depending on size, type of tumor<br />
○ Left upper quadrant pain, palpable mass, splenomegaly,<br />
weight loss<br />
Natural History & Prognosis<br />
• Complications: Hemorrhage, rupture<br />
• Prognosis: Good (benign tumors); poor (malignant)<br />
Treatment<br />
• Splenectomy depending on type (can also be diagnostic if<br />
image guided biopsy not possible)<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• 1° splenic malignancies are rare; biggest diagnostic dilemma<br />
is usually an indeterminate splenic lesion in patients with<br />
extrasplenic malignancy (i.e., is it metastasis)<br />
Image Interpretation Pearls<br />
• Considerable overlap in US findings; reliable differentiation<br />
on imaging is not always possible, requires histology<br />
SELECTED REFERENCES<br />
1. Gaetke-Udager K et al: Multimodality imaging of splenic lesions <strong>and</strong> the role<br />
of non-vascular, image-guided intervention. Abdom Imaging. 39(3):570-87,<br />
2014<br />
2. Thipphavong S et al: Nonneoplastic, benign, <strong>and</strong> malignant splenic diseases:<br />
cross-sectional imaging findings <strong>and</strong> rare disease entities. AJR Am J<br />
Roentgenol. 203(2):315-22, 2014<br />
3. Kamaya A et al: Multiple lesions of the spleen: differential diagnosis of cystic<br />
<strong>and</strong> solid lesions. Semin <strong>Ultrasound</strong> CT MR. 27(5):389-403, 2006<br />
4. Bachmann C et al: Color Doppler sonographic findings in focal spleen lesions.<br />
Eur J Radiol. 56(3):386-90, 2005<br />
410