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Abdomen » Spleen
Splenic pseudotumor
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Presentation 81 year old female with an incidentally discovered splenic mass.
Caption: Transverse image of the spleen
Description: A well-defined, rounded, predominantly hypoechoic mass is seen within the spleen. The mass causes a bulge in the splenic contour.
Caption: Sagittal view of the spleen
Description: The mass is demonstrated here again in the sagittal plane.
Caption: Transverse image of the spleen
Description: In this view the internal heterogeneity is better appreciated, as is its relationship to the splenic hilus.
Caption: Color (Power) Doppler image.
Description: The splenic vessels are seen coursing around the periphery of the mass. Several feeder vessels are seen entering the mass but the central region displays poor vascularity.
Caption: Contrast enhanced CT scan of the abdomen
Description: The heterogeneous splenic mass is noted in this image. Incidentally noted is a simple hepatic cyst in the left lobe.
Caption: Contrast enhanced CT scan of the abdomen
Description: The splenic mass is seen again. Its enhancement pattern is not specific for a hemangioma, thus, ruling out that diagnosis.
Differential Diagnosis Benign or malignant splenic neoplasm.
Final Diagnosis Splenic Pseudotumor.

Pseudotumors are rare mass lesions.  They have been reported in various organs including the orbit, respiratory tract, gastrointestinal tract, lymph nodes, soft tissues, heart, and liver.  Pseudotumors of the spleen are particularly uncommon with fewer than 70 cases reported in the medical literature.  Cotelingam and Jaffe were the first to report on two cases of splenic pseudotumor in 1984.

The exact pathogenesis and etiology of splenic pseudotumor remains obscure. Various hypothesis have been proposed regarding its development and include autoimmune, infectious (especially with Epstein-Barr virus), vascular, inflammatory, and post-traumatic causes.  To date, none of the theories has been conclusively proven.  The term inflammatory pseudotumor, however, is frequently applied to these lesions since they are composed primarily of plasma cells and lymphocytes in a fibroblastic stroma. 

Clinical presentation: Splenic pseudotumor can occur over a large age range, but is most often seen in the middle and older age populations.  No gender predisposition has been identified.  Patients are typically asymptomatic, but may present with left flank pain or splenomegaly. No serum abnormalities have been shown to be consistently present in all patients with the disorder.

Imaging: On ultrasound, splenic pseudotumor typically demonstrates-
1. A large, well-circumscribed splenic mass. 
2. Central necrosis and partial calcification can also be seen with these lesions.
The imaging characteristics of splenic pseudotumor on CT, MRI, and US normally lack the specificity to differentiate this benign disorder from other benign and malignant neoplasms of the spleen. Franquet, et al., however, reported that the presence of a central stellate area corresponding to a fibrous plaque on CT after contrast administration is strongly suggestive of an inflammatory pseudotumor.

Differential diagnosis: The differential considerations during the radiographic evaluation of splenic pseudotumor include lymphoma, hamartoma, hemangioma, lymphangioma, plasmocytoma, and splenic hydatid cyst. The definitive diagnosis of a splenic pseudotumor is made histopathologically.

Management: The prognosis of pseudotumor of the spleen is generally considered favorable.  No reports of metastatic disease, local invasion, or recurrence following splenectomy have been reported in the literature in association with the disorder.

Case References 1. Wiernik P, et al.  Inflammatory pseudotumor of the spleen. Cancer 1990; 66:597-600.
2. Hirofumi N, et al. J Clin Oncl 2000; 30(4): 196-203.
3. Cotelingam J, Jaffe ES.  Inflam. pseudotumor of the spleen. Am J Surg Path 1984;8: 375-380.
4. Sheahan K, et al. Hum Path 1988;19: 1024-1029.
5. Dalal BI, et al.  Inflam. pseudotumor of the spleen. Arch Path Lab Med 1991; 115: 1062-1064.
6. Franquet T, et al.  Inflam. pseudotumor of the Spleen: Ultrasound and CT findings. GI Radiol 1989; 14: 181-183.
7. Glazer M, et al. J of CAT 1992; 16(6): 980-983.
8. Gray EF.  Calcifications of the spleen. AJR 1944; 51: 336-351.
9. Ozkara SK, et al.  Inflam. pseudotumor of the spleen. Virchows Arch 2001; 438: 692-631.
10. Neuhauser TS, et al.  Splenic inflammatory myofibroblastic tumor.. Arch Pathol Lab Med 2001; 125(3): 379-385.
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