|Year : 2019 | Volume
| Issue : 4 | Page : 605-607
|Primary epithelial splenic cyst: A rare encounter
Pooja Sharma Kala1, Sheenam Azad2, Trisha Sharma2, Seema Acharya2
1 Department of Pathology, Government Doon Medical College, Dehradun, Uttarakhand, India
2 Department of Pathology, Sri Guru Ram Rai Institute of Medical and Health Sciences, Dehradun, Uttarakhand, India
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|Date of Web Publication||14-Oct-2019|
| Abstract|| |
Splenic cysts are rare with an overall incidence of 0.07% in a large autopsy series and 0.5% among all the splenectomies done. The parasitic splenic cysts, usually caused by Echinococcus granulosus, account for 60% of all primary splenic cysts. The primary epithelial cysts account for 10% of all splenic cysts. We report a case of 30-year-old female presenting with left upper abdominal pain and heaviness. Computed tomography revealed a multiloculated cyst in spleen. Hydatid serology was negative. Total splenectomy was done. Histopathological evaluation was done and a diagnosis of primary epithelial splenic cyst was given.
Keywords: Epithelial, primary, splenic cyst
|How to cite this article:|
Kala PS, Azad S, Sharma T, Acharya S. Primary epithelial splenic cyst: A rare encounter. Indian J Pathol Microbiol 2019;62:605-7
| Introduction|| |
Splenic cysts are rare with an overall incidence of 0.07% in a large autopsy series. The parasitic splenic cysts, usually caused by Echinococcus granulosus, account for 60% of all primary splenic cysts. The primary epithelial cysts account for 10% of all splenic cysts. Primary splenic cysts are generally asymptomatic and are identified incidentally on radiological imaging. Wide range of treatment modalities including open and laparoscopic procedures, e.g. total splenectomy, partial splenectomy, total cystectomy, marsupialization, or cyst decapsulation can be adopted depending on the size and location of the cyst.
| Case Summary|| |
A 30-year-old female presented to the surgical outpatient department with chief complaints of pain and heaviness in left upper quadrant of abdomen since 6 months. On examination, moderate splenomegaly was present. Contrast-enhanced computed tomography (CT) revealed a multiloculated cyst measuring 7.3 × 5.2 × 5.1 cm in spleen, giving an impression of multiloculated complex splenic cyst, possibly Hydatid cyst. Hydatid serology was negative. Open total splenectomy was done. The specimen was sent for histopathological examination.
Grossly, the splenectomy specimen measured 11 × 9 × 4 cm [Figure 1]a. Outer surface showed a cystic mass measuring 6.5 × 5 × 3.4 cm in the hilar region. On serial section, three cysts filled with serous fluid were seen [Figure 1]b. The cyst wall was creamy white and wall thickness varied from 0.2 to 0.5 cm.
|Figure 1: (a) Splenectomy specimen showing a cystic mass near hilum. (b) Cut surface of spleen showing multilocular cyst with thick creamy white cyst wall. (c) Section shows two cysts with adjacent splenic parenchyma (arrow heads). One of the cyst is lined by epithelium (long arrow) while the other is filled with cholesterol clefts and has a thick eosinophilic cyst wall without any epithelial lining (H and E; x40). (d) Section showing cyst lining of cuboidal to columnar epithelium (H and E; x100)|
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Microscopically, the cysts were lined by cuboidal to low columnar epithelium [Figure 1]c and [Figure 1]d. The lumen was filled with eosinophilic material and entrapped inflammatory cells. There was dense fibrosis and hyalinization in the wall which extended into the surrounding parenchyma. Additional findings were cholesterol clefts, foci of calcification, and collections of foamy histiocytes. The surrounding splenic parenchyma showed congestion and Gamna--Gandy bodies at places.
A histological diagnosis of primary (epithelial) Splenic cyst with associated venous congestion was given.
| Discussion|| |
Cysts in spleen are unusual. Splenic cysts have been classified differently by different authors.
Martin as well as Hansen and Moller classified splenic cysts as primary (type I) and secondary/false/pseudocysts (type II)., The latter are usually posttraumatic and lack the epithelial lining. Primary cysts can be further classified into parasitic and nonparasitic types. The nonparasitic primary cysts can be congenital or neoplastic. Congenital cysts can be further classified according to the type of epithelial lining, e.g., epidermoid, dermoid, and simple by Hansen and Moller.
Morgenstern proposed a slightly different classification for the nonparasitic cysts according to which these were categorized into congenital, neoplastic, traumatic, necrotic, and degenerative types.
Parasitic cysts comprise 60% of all splenic cysts. Echinococcus granulosus is the commonest causative agent for parasitic cysts. These are more common in Africa and Central America. Nonparasitic cysts are common in Europe and North America. Epithelial splenic cysts are considered congenital. Their actual origin is still unclear. Various theories have been proposed – developmental epithelial displacement and metaplasia from surrounding structures during embryogenesis, invagination of peritoneal mesothelium during development, and metaplasia of heterotopic endodermal inclusions in the spleen., These cysts are quite uncommon and comprise only 10% of all splenic cysts and 25% of nonparasitic cysts.
The nonparasitic cysts are commonly encountered in young adults around second and third decades. The patients are asymptomatic in up to 60% cases. At times, the patients may present with left upper quadrant pain (due to splenomegaly/stretching of splenic capsule), nausea, and vomiting (due to pressure effect on stomach). Symptoms because of development of complications, e.g., rupture, intracapsular hemorrhage, or infections can also occur. A similar presentation was seen in the present case. Radiology especially CT scan is helpful in diagnosing splenic cysts; however, its exact categorization is not possible without histopathological examination. Serum or cyst fluid CA 19-9 and Carcinoembryonic antigen (CEA) levels can be increased in cases of nonparasitic cysts, but these cysts do not have malignant potential.
The identification of lining epithelium of a splenic cyst plays a major role in differentiating a primary splenic cyst from a pseudocyst. The lining epithelium is lacking in pseudocyst because of desquamation/degeneration. Immunohistochemistry using Epithelial-membrane antigen (EMA) and CEA may help in identification of epithelial lining in difficult cases.
Symptomatic cysts and large cysts of size >5 cm are surgically managed. Smaller cysts may undergo spontaneous resolution or remain asymptomatic; hence, a regular radiological follow-up is more logical in these cases. The cysts located at hilum may lead to intractable bleeding, hence should be surgically managed. Total splenectomy was done in the present case considering the large size of the cyst and its hilar location. Vaccination against S. pneumoniae, N. meningitidis, and H influenza virus type b was also given to the patient.
Splenectomy was once thought to be the sole surgical modality. But now, open as well as laparoscopic techniques are available. Spleen preserving surgical techniques are preferred so as to avoid lifelong risk of overwhelming post splenectomy sepsis. Preservation of 25% of splenic parenchyma helps in prevention of overwhelming postsplenectomy infection. The options are partial splenectomy, cystectomy, and cyst decapsulation. Other methods are radiologically guided drainage of cyst fluid; however, the chances of recurrence are very high with this procedure. Laparoscopic techniques for nonparasitic splenic cysts include aspiration, marsupialization, fenestration, partial cystectomy (decapsulation), and laparoscopic partial splenectomy with the resection of the cyst and a portion of the contiguous splenic parenchyma. Laparoscopic intervention has obvious benefits of less pain and faster recovery but is associated with a recurrence rate of 22%.
| Conclusion|| |
Cysts in spleen are a rare encounter in surgical practice especially the primary epithelial splenic cysts and histopathological examination remains the gold standard for the diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Pooja Sharma Kala
Assistant Professor, Department of Pathology, Government Doon Medical College, Dehradun, Uttarakhand - 248 001
Source of Support: None, Conflict of Interest: None
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