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Year : 2009  |  Volume : 52  |  Issue : 4  |  Page : 564-565
Inflammatory psuedo-tumor of the spleen


Department of Pathology, Pt. B.D. Sharma PGIMS, Rohtak, Haryana- 124 001, India

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Date of Web Publication1-Oct-2009
 

   Abstract 

Inflammatory pseudo-tumor of spleen, a benign reactive lesion of unknown etiology and pathogenesis, is extremely rare with isolated case reports in literature. These are usually misdiagnosed preoperatively, both clinically and radiologically; metastasis or lymphoproliferative disorders with pathological studies allow reliable diagnosis of the disease. We report the unusual occurrence of this lesion in the spleen.

Keywords: Inflammatory pseudo-tumor, spleen

How to cite this article:
Singh S, Chhabra S, Modi S, Marwah N, Rawal A, Arora B. Inflammatory psuedo-tumor of the spleen. Indian J Pathol Microbiol 2009;52:564-5

How to cite this URL:
Singh S, Chhabra S, Modi S, Marwah N, Rawal A, Arora B. Inflammatory psuedo-tumor of the spleen. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Nov 1];52:564-5. Available from: http://www.ijpmonline.org/text.asp?2009/52/4/564/56169



   Introduction Top


Inflammatory pseudo-tumor of spleen is a benign rare tumorous lesion of uncertain etiopathogenesis with histological features of nonspecific inflammation and mesenchymal repair. Only a few cases have been reported to date. [1] Such a lesion has occasionally been observed in several tissues like orbit, spinal meanings, digestive system, heart, soft tissues, mesothelial membranes and respiratory tract. Its occurrence in spleen is extremely rare and is usually not diagnosed clinically and radiologically. Only histopathological and immunohistochemical (IHC) studies provide a reliable diagnosis of the disease. [2]


   Case Report Top


A 21-year-old male presented with pain epigastrium and left hypochondrium for one year. He was a known case of stage-III Wilm's tumor right kidney (tumor measured 15x10x8cm with preaotrtic and paraaortic lymphadenopathy) for which right sided nephrectomy was done 20 years back followed by radiotherapy and chemotherapy. On ultrasonography, spleen showed single hypoechoic lesion measuring 2.3x2.2cm. Septated collections were seen in small areas in splenic fossa. Computed tomography (CT) revealed a hypo dense lesion with peripheral enhancement suggestive of metastasis. Right kidney was not visualized. A clinical diagnosis of metastatic deposits from Wilm's tumor was done.

The patient underwent laparotomy followed by splenectomy. Preoperatively, splenic adhesions were present and tumor was present deep in the spleen. Spleen was submitted for pathological examination.

Pathological findings

Spleen weighed 150gm and measured 8x7x4cm. A circumscribed firm tan white nodule measuring two cm in diameter was seen [Figure 1] on serial sectioning.

Histological examination of mass revealed it to be composed of admixture of inflammatory cellular elements, predominantly lymphocytes, plasma cells, histiocytes along with neutrophils, some eosinophils and spindle cells (myofibroblasts) [Figure 2] and [Figure 3]. A histopathological diagnosis of inflammatory pseudo-tumor of spleen was made.


   Discussion Top


Inflammatory pseudo-tumors are benign mass like lesions formed in the course of an inflammatory reparative process characterized histologically by nonspecific inflammation and mesenchymal repair. [3] These are frequently encountered and described in a wide variety of anatomical sites like lungs, heart, gastrointestinal tract, pancreas, liver, urinary bladder, kidney, central nervous system, orbit, thyroid, soft tissues, serious membranes and lymph nodes. Inflammatory pseudo-tumors of spleen are rare and present as well circumscribed, enlarging, space-occupying lesions which may be symptomatic or asymptomatic. Symptomatic patients present, most frequently, with pain usually involving the left upper quadrant or epigastric region. [3]

The cause of inflammatory pseudo-tumor of spleen is unknown. Various hypotheses have been put forward in the pathogenesis including infectious (EBV, mycobacterium in immunocompromised patients, human immunodeficiency virus, auto immune and vascular causes. However, the exact cause remains uncertain. [4],[5]

Inflammatory pseudo-tumors of spleen are usually misdiagnosed as malignant process clinically and radiologically. [5] However, histopathological examination is the diagnostic tool for this entity. Constant findings are the presence of polymorphic inflammatory cell infiltrate and proliferation of spindle cells. Inflammatory cells present mainly include lymphocytes, plasma cells, histiocytes along with neutrophils and eosinophils. [6] Majority of cells are T-cells, lesser being B-cells and polyclonal plasma cells expressing polytypic cytoplasmic immunoglobulin light chain. [5] Spindle cells are positive for vimentin confirming their myofibroblastic origin. [4]

Clinical and radiological differential diagnosis of splenic inflammatory pseudo-tumor, presenting as solitary nodule and seen as hypovascular/ hypo dense mass includes metastatic neoplasms, lymphoproliferative disorders, granulomatous infections, vascular malformations, infarction, cysts, localized reactive hyperplasia [3] and splenic hamartoma. [5] However, the lack of atypia in lymphocytes, polymorphous nature of infiltrate accompanied by plump spindle cells suggests a reactive process and the diagnosis can be made readily by identifying the reactive nature of the cells. [2] Splenectomy is both diagnostic and curative. [6]

 
   References Top

1.Chen WH, Liu TP, Liu CL, Tzen CY. Inflammatory psuedotumor of the spleen. J Clin Med Assoc 2004;67:533-6.  Back to cited text no. 1      
2.Venizelos I, Tamiolakis D, Simopoulos C, Nikolaidou S, Barbagadaki S, Alexiadis G, et al. Inflammatory pseudo-tumor of the spleen. Chirurgia (Buccu) 2004;99:265-8.  Back to cited text no. 2      
3.Dalel BI, Greenberg H, Quinonez GE, Gough JC. Inflammatory psuedotumor of spleen: Morphological, radiological, immunophenotypic, and ultrastructural features. Arch Pathol Lab Med 1991;115:1062-3.  Back to cited text no. 3      
4.Lewis JT, Gaffney RL, Casey MB, Farrel MA, Morice WG, Macon WR. Inflammatory pseudo-tumor of the spleen associated with a clonal Epstein-Barr virus genome: Case report and review of the literature. Am J Clin Pathol 2003;120:56-61.  Back to cited text no. 4      
5.Thomas RM, Jaffe ES, Osorno AZ, Medeiros J. Inflammatory pseudo-tumor of the spleen: A clinicopathologic and immunophenotypic study of eight cases. Arch Pathol Lab Med 1993;117:921-6.  Back to cited text no. 5      
6.Spivach A, Martinolli S, Adornetto R, Melato M. Inflammatory pseudo-tumor of the spleen: A case report and review of the literature. Chir Ital 2005;57:655-60.  Back to cited text no. 6      

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Correspondence Address:
Sunita Singh
881/23, DLF Colony, Rohtak- 124 001
India
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DOI: 10.4103/0377-4929.56169

PMID: 19805975

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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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