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CASE REPORT Table of Contents   
Year : 2014  |  Volume : 11  |  Issue : 1  |  Page : 67-70
A report of a giant epidermoid splenic cyst

Department of Medical and Surgical Sciences, Pediatric Surgery Unit, University of Foggia, Foggia, Italy

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Date of Web Publication20-Mar-2014


Splenic cysts are uncommon lesions and are classified as true or pseudocysts based on the presence of an epithelial lining. True congenital cysts can be epidermoid, dermoid or endodermoid and require surgery when large, sympthomatic or complicated. Ultrasonography and computerised tomography scan are useful for diagnosis and serum test for echinococcosis should be done. Several procedures, open or laparoscopic, have been described, with emphasis on the spleen-preserving surgery, when possible. We present a boy with a giant symptomatic epidermoid cyst in whom total splenectomy by open technique was carried out.

Keywords: Children, epidermoid cyst, management, splenic cyst

How to cite this article:
Pastore V, Bartoli F. A report of a giant epidermoid splenic cyst. Afr J Paediatr Surg 2014;11:67-70

How to cite this URL:
Pastore V, Bartoli F. A report of a giant epidermoid splenic cyst. Afr J Paediatr Surg [serial online] 2014 [cited 2022 Jan 23];11:67-70. Available from:

   Introduction Top

Splenic cysts are unusual in everyday surgical practice, especially in western countries and less than 1000 cases have been described in the literature. [1] They are classified as true (primary, 25%) lined by epithelium or false cysts (pseudocysts or secondary, 75%) without epithelial or endothelial lining (often secondary to trauma, haemorrhage or infarction). [2] True cysts can be further subdivided into parasitic (caused by Echinococcus granulosus) and nonparasitic [3] according to their aetiology. Nonparasitic cysts are neoplastic (lymphangioma and haemangioma) or congenital which are classified as epidermoid (90%), dermoid and endodermoid depending on the type of lining [1],[2] [Table 1]. Usually, congenital cysts are benign, sporadic, with a slight female preponderance, present at younger age and their pathogenesis is unclear. Most of the cysts are asymptomatic presenting as an incidental finding during the abdominal ultrasonography (USG). Some patients present symptoms related to complications or bulky size. In fact, cysts larger than 5 cm are susceptible to haemorrhage, rupture and infection and surgery is recommended for congenital cysts >4 cm. [1] USG and computerised tomography (CT) are useful investigations but diagnosis of an epidermoid cyst is only made by the pathologist. Controversies exist regarding optimal surgical management (aspiration, marsupialisation, cystectomy, decapsulation, partial splenectomy, splenectomy). In fact, the increased risk of overwhelming post-splenectomy infection in children [4] has prompted organ-saving procedures. We report a case of a 9-year-old boy with a giant epidermoid splenic cyst with discussion on surgical strategies.
Table 1: Splenic cysts classifications according to Fowler, Martin and Morgenstern

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   Case Report Top

The present case report is about a 9-year-old boy who was admitted to our Unit because of abdominal pain localised in the left upper quadrant that had started a day before. The patient's medical history was unremarkable, including previous abdominal trauma. The physical examination revealed only a distended and asymmetric abdomen with a palpable mass in the left hypochondrium. Routine blood tests were within normal ranges. Chest and abdominal X-rays showed the elevation of the left hemidiaphragm and asymmetric intestinal gas distribution. The abdominal USG revealed a giant, round hypoechoic and multilocular cystic lesion (maximum diameter of the cyst was 20 cm) with internal echoes without Doppler flow. The abdominal CT scan demonstrated a well-limited, large multilocular (20 cm × 13 cm × 21 cm), non-enhancing and hypoattenuated cystic lesion occupying all the splenic parenchyma causing medial displacement of the stomach, pancreas and left kidney [Figure 1]. The cyst was septed with smooth wall and fluid content. These findings oriented for the diagnostic hypothesis of hydatidosis or congenital cyst of the spleen. However, serology for E. granulosus was negative so that surgery was planned. After pre-treatment with meningococcal, pneumococcal and haemophilus influenza vaccines, antibiotic and steroid prophylaxis, the child underwent open splenectomy. Splenectomy was indicated due to giant size, the location at hilum and the residual atrophic splenic tissue [Figure 2]; at surgery, we observed that most of splenic blood flow was originating from a branch (right gastroepyploic) of gastroduodenal artery while the splenic artery was completely hypoplasic. The post-operative course was uneventful and he was discharged 5 days later. Pathological examination showed an epidermoid congenital cyst with fibrous tissue covered by stratified squamous epithelium [Figure 3] without evidence of epithelial atypia or malignancy. At immunostaining, the epithelium was positive for cytokeratines AE1 and AE8 [Figure 4]. The fluid was turbid and yellow with some cholesterol crystals and lipids. At 12 months follow-up, the child is doing well with normal blood or immunological tests.
Figure 1: Computerized tomography scans showing the giant splenic cyst

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Figure 2: Surgical specimen: Spleen with epidermoid cyst

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Figure 3: Epidermoid cyst with squamous epithelium (EE ×40/×100)

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Figure 4: Positivity at immunostaining for cytokeratines AE1 and AE8 ( ×200)

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   Discussion Top

The incidence of splenic cysts is low and congenital ones are very uncommon. [5] These are usually large, solitary and sporadic (few familial cases have been reported without identifying genetic predisposing factors). [6],[7] The physiopathology is still controversial especially, about their origin from developmental displacement of epithelial tissue, with subsequent metaplasia, or whether they arise from the peritoneal mesothelium. The types of epithelial tissues hypothesized are germinal cells from gonads, [8] the dorsal mesogastrium [9] and the Wolffian duct. [10] Bürrig [11] and Ough et al. [12] have reported that epithelial cysts originate from invaginations of capsular peritoneal mesothelium or collections of peritoneal mesothelial cells trapped into splenic sulci, which subsequently undergo to squamous metaplasia (possibly as consequences of chronic inflammation). Furthermore, Authors suggested that epithelial cysts have a teratomatous derivation [13] while Others [14] considered splenic epithelial cysts the result of secondary fluid collection after injury or spontaneous intrasplenic bleeding. In addition, communication between the pancreatic duct system and the intrapancreatic accessory spleen might lead to cyst growth. [15] The clinical manifestation differs according to size and the symptoms are non-specific and are related to compression of the adjacent organs. Malignant degeneration of primary splenic cyst is rare. Serum carbohydrate antigen 19-9 and carcinoembryonic antigen have been reported to be elevated in serum and cyst fluid [16] but these antigens were not investigated in our patient. USG and CT are the most frequently used diagnostic methods helpful in determining whether the cyst is multi- or unilocular, the location of the cyst and relationship to the surrounding structures. [17] USG is a sensitive, cheap and non-invasive tool in the diagnosis and epidermoid cyst appears as a round anechoic area with thin wall. Septations and calcifications may be seen. On CT, cysts are spherical without rim enhancement. The conventional treatment of splenic symptomatic, large or complicated cysts [18] has been total splenectomy, open or laparoscopic. In recent years, a spleen-preserving surgical approach is recommended since it is well-known that the spleen plays an important role in normal homeostasis (regulation of the circulating blood volume, haematopoiesis, immunity and protection against infections and malignancies). In fact, the risk of post-splenectomy sepsis is about 4%, with a mortality rate of 1.5%. [19] However, conservative approaches such as percutaneous aspiration or sclerosis (by using tetracycline or alcohol) are associated with high recurrence rate and potential risk of bleeding and infection. [20],[21] In addition, dense inflammatory response around the spleen makes subsequent surgery difficult. Today the suggested treatment options are partial splenectomy (preserving >25%), cystectomy, decapsulation and marsupialization, with open or laparoscopic approach. Partial splenectomy is the best known spleen-preserving procedure and is based on the segmentation of the splenic vascularization. The first successful partial splenectomy for an epidermoid cyst was carried out in 1980 [22] but it is a difficult procedure with higher risks of major intraoperative and post-operative blood loss. Cystectomy is the removal of the cyst wall with lining epithelium and a portion of the contiguous splenic parenchyma, but it can be attempted only in superficial cysts that are not close to the ilum. [1],[17] Furthermore this technique may be complicated by major intra-operative blood loss. Some Authors have proposed decapsulation of the cyst, leaving the portion of cyst wall contiguous with the splenic parenchyma in situ. [23],[24] Its advantages include a simpler and more rapidly performed procedure, with less blood loss, simplified haemostasis and preservation of splenic tissue; [25] disadvantage is higher recurrence rate because a portion of the cyst lining is left intact. Marsupialisation is creation of an opening on the cyst wall for internal or external drainage [26] with placation of cyst edges to prevent collapse of the cyst wall and recurrence. Recently, advances in operative techniques, haemostasis with fibrin glue, radiofrequency ablation and stapler techniques have made spleen-preserving procedures safe and feasible. [27],[28],[29],[30] However, total splenectomy remains the treatment of choice if the cyst is big enough to involve all the spleen which is reduced to a small and atrophic remnant, as it was in our case, or if the cyst involves the hilum. In addition, complete removal of the cyst is needed since recurrence has been reported after open and laparoscopic surgery. [31] Total splenectomy remains a safe procedure, associated with few complications. Laparoscopic approach has recently gained increasing acceptance in splenic surgery and was first reported in the adult literature. [32] In the paediatric experience it showed good short-term results [33],[24] but the largest retrospective studies showed that it is associated with a high recurrence rate. [5],[34],[35],[36] The significance of splenic cyst recurrence after laparoscopic treatment remains unclear and it ranges from 50% to 88% and may depend on the type of laparoscopic resection performed. Furthermore, laparoscopic surgery provides a minimal access method to approach small true cysts, but in our patient the cyst was giant (maximum diameter 20 cm) and the splenic parenchyma was completely occupied and substituted by the mass. Despite the pre-operative serology for E. granulosus was negative the cyst was multiloculated and the risk of eventual dissemination of cyst fluid could not be excluded, that is why we decided to perform a laparotomy.

   Conclusion Top

In summary, the epidermoid cyst is a rare splenic lesion and usually presents as a large cystic mass producing abdominal symptoms. There is no reliable pre-operative diagnostic method to surely identify an epidermoid cyst. The laparoscopic management offers the benefits of minimally invasive surgery (minimal post-operative pain, faster recovery, shorter hospital stay, better aesthetic result) but has an higher recurrence rate, especially in partial splenectomy. The spleen-preserving techniques should be attempted when nonparasitic cysts are relatively small and peripheric. We believe that in our patient, total splenectomy was the safest and the most effective procedure to treat giant epidermoid splenic cyst.

   References Top

1.Morgenstern L. Nonparasitic splenic cysts: Pathogenesis, classification, and treatment. J Am Coll Surg 2002;194:306-14.  Back to cited text no. 1
2.Fowler RH. Nonparasitic benign cystic tumors of the spleen. Int Abstr Surg 1953;96:209-27.  Back to cited text no. 2
3.Martin JW. Congenital splenic cysts. Am J Surg 1958;96:302-8.  Back to cited text no. 3
4.Eraklis AJ, Kevy SV, Diamond LK, Gross RE. Hazard of overwhelming infection after splenectomy in childhood. N Engl J Med 1967;276:1225-9.  Back to cited text no. 4
5.Czauderna P, Vajda P, Schaarschmidt K, Kalman A, Jainsch M, Engelis A, et al. Nonparasitic splenic cysts in children: A multicentric study. Eur J Pediatr Surg 2006;16:415-9.  Back to cited text no. 5
6.Younger KA, Hall CM. Epidermoid cyst of the spleen: A case report and review of the literature. Br J Radiol 1990;63:652-3.  Back to cited text no. 6
7.Ragozzino MW, Singletary H, Patrick R. Familial splenic epidermoid cyst. AJR Am J Roentgenol 1990;155:1233-4.  Back to cited text no. 7
8.Lurbasch O. Pathologie des angiomas. Ergebn Allg Path 1905;10:815.  Back to cited text no. 8
9.Harding HE. Large inclusion cyst in spleen. J Path Bact 1933;36:485.  Back to cited text no. 9
10.Santy P. Splenectomy for a true cyst of the spleen in a child. Lyon Chir 1930;27:101-4.  Back to cited text no. 10
11.Bürrig KF. Epithelial (true) splenic cysts. Pathogenesis of the mesothelial and so-called epidermoid cyst of the spleen. Am J Surg Pathol 1988;12:275-81.  Back to cited text no. 11
12.Ough YD, Nash HR, Wood DA. Mesothelial cysts of the spleen with squamous metaplasia. Am J Clin Pathol 1981;76:666-9.  Back to cited text no. 12
13.Lifschitz-Mercer B, Open M, Kushnir I, Czernobilsky B. Epidermoid cyst of the spleen: A cytokeratin profile with comparison to other squamous epithelia. Virchows Arch 1994;424:213-6.  Back to cited text no. 13
14.Touloukian RJ, Maharaj A, Ghoussoub R, Reyes M. Partial decapsulation of splenic epithelial cysts: Studies on etiology and outcome. J Pediatr Surg 1997;32:272-4.  Back to cited text no. 14
15.Horn AJ, Lele SM. Epidermoid cyst occurring within an intrapancreatic accessory spleen. A case report and review of the literature. JOP 2011;12:279-82.  Back to cited text no. 15
16.Arda IS, Tüzün M, Hicsönmez A. Epidermoid cyst of the spleen with elevated levels of CA125 and carcino-embryonic antigen. Eur J Pediatr 2005;164:108.  Back to cited text no. 16
17.Rescorla FJ. Spleen. In: Grosfeld JL, O'Neill JA Jr, Coran AG, Fonkalsrud EW, editors. Pediatric Surgery. Philadelphia: Mosby-Elsevier; 2006. p. 1692.  Back to cited text no. 17
18.Hansen MB, Moller AC. Splenic cysts. Surg Laparosc Endosc Percutan Tech 2004;14:316-22.  Back to cited text no. 18
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20.Fahel E, Amaral PC, Filho EM, Ettinger JE, Souza EL, Fortes MF, et al. Videolaparoscopic approach of the splenic cyst: A case report. JSLS 2000;4:23-6.  Back to cited text no. 20
21.Moir C, Guttman F, Jequier S, Sonnino R, Youssef S. Splenic cysts: Aspiration, sclerosis, or resection. J Pediatr Surg 1989;24:646-8.  Back to cited text no. 21
22.Morgenstern L, Shapiro SJ. Partial splenectomy for nonparasitic splenic cysts. Am J Surg 1980;139:278-81.  Back to cited text no. 22
23.Touloukian RJ, Seashore JH. Partial splenic decapsulation: A simplified operation for splenic pseudocyst. J Pediatr Surg 1987;22:135-7.  Back to cited text no. 23
24.van der Zee DC, Kramer WL, Ure BM, Mokhaberi B, Bax NM. Laparoscopic management of a large posttraumatic splenic cyst in a child. Surg Endosc 1999;13:1241-2.  Back to cited text no. 24
25.Mahomed A, Youngson G. Cyst decapsulation: An alternative option in the conservative management of benign splenic cysts. Pediatr Surg Int 1998;14:153.  Back to cited text no. 25
26.Palanivelu C, Rangarajan M, Madankumar MV, John SJ. Laparoscopic internal marsupializaton for large nonparasitic splenic cysts: Effective organ-preserving technique. World J Surg 2008;32:20-5.  Back to cited text no. 26
27.Yavorski CC, Greason KL, Egan MC. Splenic cysts: A new approach to partial splenectomy - Case report and review of the literature. Am Surg 1998;64:795-8.  Back to cited text no. 27
28.Pampaloni F, Valeri A, Mattei R, Presenti L, Noccioli B, Tozzini S, et al. Laparoscopic decapsulation of a large epidermoid splenic cyst in a child using the UltraCision LaparoSonic Coagulating Shears. Pediatr Med Chir 2002;24:59-62.  Back to cited text no. 28
29.Sellers GJ, Starker PM. Laparoscopic treatment of a benign splenic cyst. Surg Endosc 1997;11:766-8.  Back to cited text no. 29
30.Khelif K, Maassarani F, Dassonville M, Laet MH. Laparoscopic partial splenectomy using radiofrequency ablation for nonparasitic splenic cysts in two children. J Laparoendosc Adv Surg Tech A 2006;16:414-7.  Back to cited text no. 30
31.Ganti AL, Sardi A, Gordon J. Laparoscopic treatment of large true cysts of the liver and spleen is ineffective. Am Surg 2002;68:1012-7.  Back to cited text no. 31
32.Heidenreich A, Canero A, di Pasquo A. Laparoscopic approach for treatment of a primary splenic cyst. Surg Laparosc Endosc 1996;6:243-6.  Back to cited text no. 32
33.Till H, Schaarschmidt K. Partial laparoscopic decapsulation of congenital splenic cysts. A medium-term evaluation proves the efficiency in children. Surg Endosc 2004;18:626-8.  Back to cited text no. 33
34.Fisher JC, Gurung B, Cowles RA. Recurrence after laparoscopic excision of nonparasitic splenic cysts. J Pediatr Surg 2008;43:1644-8.  Back to cited text no. 34
35.Mertens J, Penninckx F, DeWever I, Topal B. Long-term outcome after surgical treatment of nonparasitic splenic cysts. Surg Endosc 2007;21:206-8.  Back to cited text no. 35
36.Schier F, Waag KL, Ure B. Laparoscopic unroofing of splenic cysts results in a high rate of recurrences. J Pediatr Surg 2007;42:1860-3.  Back to cited text no. 36

Correspondence Address:
Valentina Pastore
Pediatric Surgery, University of Foggia, Viale Pinto 1, 71122, Foggia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-6725.129239

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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