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Year : 2012  |  Volume : 55  |  Issue : 4  |  Page : 602-604
Multilocular epithelial cyst of spleen: A rare occurrence


Department of Pathology and Biochemistry, AFMC, Department of Pathology, CH (SC), Pune, India

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Date of Web Publication4-Mar-2013
 

How to cite this article:
Manoj MG, Misra P, Kakkar S. Multilocular epithelial cyst of spleen: A rare occurrence. Indian J Pathol Microbiol 2012;55:602-4

How to cite this URL:
Manoj MG, Misra P, Kakkar S. Multilocular epithelial cyst of spleen: A rare occurrence. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Feb 25];55:602-4. Available from: http://www.ijpmonline.org/text.asp?2012/55/4/602/107850


Sir,

Splenic cysts are unusual occurrences in everyday practice. They can be parasitic (hydatid), caused by the parasite Echinococcus granulosus, or nonparasitic. [1] Nonparasitic cysts are classified as (a) primary (true, epithelial), lined by an epithelium (epidermoid, dermoid, and mesothelial) or endothelium (hemangioma, lymphangioma), and (b) secondary (pseudocysts, non-epithelial), which are usually post-traumatic in origin. [2] Among these, the primary ones are rare and appear as asymptomatic masses in the left hypochondrium. The pathogenesis of true cyst is conjectural and numerous hypotheses are given by different authors. [3] We present a multilocular epithelial cyst of spleen, which presented as a case of isolated splenomegaly.

A 47-year-old previously healthy female presented with a slowly enlarging mass in the left upper quadrant of the abdomen of 2 months duration accompanied with dull dragging pain. There was no past history of trauma. Physical examination revealed a nontender tense mass with smooth surface in the left hypochondrium which moved with respiration. It was freely mobile from side to side and was 3 cm below the left costal margin. Spleen could not be made out separately. Systemic examination, routine biochemical and hematological investigations were within normal limits. Hydatid serology and Casoni's tests were negative. Ultrasonography of abdomen showed an enlarged multicystic spleen and computed tomography (CT) scan confirmed the findings [Figure 1]. The patient underwent a total splenectomy and had an uneventful postoperative period. The surgical specimen of the spleen measured 13 × 10 × 6 cm weighed 450 g and had a smooth surface. The cut surface showed multiple cysts containing gelatinous material. The largest cyst measured 5 × 5 × 3 cm. The inner surface was smooth and glistening with normal appearing adjacent splenic parenchyma [Figure 2]. Microscopically, the fibrocollagenous cyst wall was lined by flattened mesothelium-like epithelium [Figure 3]a,b. There was no evidence of squamous epithelium in the numerous sections studied from different areas, hence ruling out an epidermoid cyst. Immunohistochemically, the epithelial cells were positive for pan-cytokeratin [Figure 3]c and negative for D240 (lymphatic epithelial cell marker) and CD34 (endothelial cell marker) [Figure 3]d.
Figure 1: Ultrasonography and CT scan images show multiple hypoechoic lesions in the splenic parenchyma

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Figure 2: Cut surface of spleen shows the multiloculated cyst with surrounding normal appearing splenic parenchyma

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Figure 3: (a) Cyst wall lined by benign flattened epithelial lining (Hematoxylin and eosin stain, ×100) (b). Cyst wall lined by benign flattened epithelial lining (Hematoxylin and eosin stain, ×400). (c) Cyst epithelial lining showing cytoplasmic positivity for Pancytokeratin (Immunohistochemistry, ×100) (d) Cyst epithelial lining is negative for cluster differentiation-34 with the endothelial lining of the capillaries (internal control) in the cyst wall being positive (Immunohistochemistry, ×100)

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Epithelial splenic cysts are uncommon and comprise only about 10% of all splenic cysts and 25% of nonparasitic cysts. Splenic epithelial cysts occur predominantly in the second and third decades of life but can occur in children too. Controversy still exists regarding aetiopathogenesis of these uncommon lesions. Primary cysts are thought to be congenital and have been attributed to misplaced epithelial tissue during embryogenesis with consequent metaplasia. William et al.[4] suggested that these cysts, excluding dermoid cysts, are a result of invagination of the splenic capsular mesothelium during development and fluid accumulation for unknown reasons, resulting in cyst formation.

These cysts are usually asymptomatic, or they may present with vague abdominal pain, abdominal mass or may be incidental findings during abdominal ultrasonography. These cysts become symptomatic only when they attain a size of more than 5 cm or more. The initial symptoms are primarily gastro-intestinal and include vague abdominal pain, early satiety, nausea, vomiting, and dysphagia. On rare occasions, these cysts can become infected or rupture resulting in an acute abdominal emergency. [5]

The cysts are usually unilocular with occasional foci of calcification. Though no associated cysts are noted in other viscera, a few case reports on familial true splenic cysts are reported. Depending on the type of lining, true splenic cysts are histologically classified as angiomatous (endothelium), epidermoid (stratified nonkeratinising squamous), simple congenital/mesothelial (low cuboidal to low columnar) and true dermoid (squamous lining with dermal structures) types. [2] Among these, the endothelial lined cyst is considered as a cystic vascular lesion composed of several ectatic vessels, and would be more correctly referred to as lymphangioma or hemangioma. Excluding these vascular cysts, the rest constitute true epithelial cysts among which epidermoid cyst is the most common. Some prefer to call epidermoid cysts "mesothelial cysts with focal squamous metaplasia," as in most cases the squamous epithelium is focal and in continuity with the mesothelial lining.

Burrig et al.[3] performed morphological investigation, electron microscopy, and Immunohistochemistry, and concluded that most epithelial cysts are derived from traumatic damage to the splenic capsule with resultant in-growth of surface mesothelium and subsequent cyst formation. Some, doubted this etiopathogenic association, and concluded that injury only leads to hemorrhage in an already existing cyst, resulting in clinical enlargement. The occurrence of various types of epithelium in true cysts may be explained simply by implicating the pleuripotent nature of mesothelial cells. Immunohistochemistry with a battery of monoclonal antibodies to cytokeratins and comparing it with other squamous epithelia, some authors have suggested that the epidermoid cyst is either of teratomatous derivation or originates from inclusion of foetal squamous epithelium rather than from squamous metaplasia of mesothelium. [4] The cystic nature and splenic origin of these lesions can be well established by various radiological studies such as ultrasonography, CT scan and magnetic resonance imaging. The benign nature of these cysts can be ascertained by radioisotope splenic scan and selective angiography; however the final characterization depends on the histological examination of the lining of the cyst. [4]

Due to the increased risk of complications, such as rupture, hemorrhage, and infection, splenic cysts with a diameter larger than 4-5 cm should be managed surgically, because conservative options, such as percutaneous aspiration or sclerosis, do not result in long-term control. There are different types of surgical treatment according to the patient's age and the size, location, and nature of the cyst. The classical approach to splenic cysts has been complete splenectomy. [6]

In conclusion, splenic cysts are uncommonly encountered lesions in surgical pathology; those larger than 5 cm or symptomatic ones should be treated surgically to avoid the risk of complications; histopathological examination should be done to confirm the subtype of splenic cyst and to rule out a possible, though rare, malignant transformation of the pleuripotent epithelial lining.

 
   References Top

1.Avital S, Kashtan H. A large epithelial splenic cyst. N Engl J Med 2003;349:2173-4.  Back to cited text no. 1
[PUBMED]    
2.Tsakraklides V, Hadley TW. Epidermoid cysts of the spleen. Arch Pathol 1973;96:251-4.  Back to cited text no. 2
[PUBMED]    
3.Burrig KF. Epithelial (true) splenic cysts. Am J Surg Pathol 1988;12:275-81.  Back to cited text no. 3
    
4.Williams RJ, Glazer G. Splenic cysts: Changes in diagnosis, treatment and etiological concepts. Ann R Coll Surg Engl 1993;75:87-9.  Back to cited text no. 4
[PUBMED]    
5.Qureshi MA, Hafner CD. Clinical manifestations of splenic cysts: Study of 75 cases. Am Surg 1965;31:605-8.  Back to cited text no. 5
[PUBMED]    
6.Schwartz SI. The spleen. In: Schwartz SI, Harold E, editors. Maingot's Abdominal Operations. Vol II. 9 th ed. Connecticut, USA: Appleton and Lange; 1990. p. 1679-80.  Back to cited text no. 6
[PUBMED]    

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Correspondence Address:
Madakshira Gopal Manoj
Department of Pathology, AFMC, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.107850

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    Figures

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