Case Report
Splenic Epithelial Cyst with Elevated Ca 19-9 Levels
Authors
Sunil Kumar Shetty, Assistant Professor, Dept. of General Surgery,
Keshav Prasad, Assistant Professor, Dept. of General Surgery,
Sriram Bhat, Professor, Department of General Surgery,
Srikanth Soma, Junior Resident, Dept. of General Surgery,
Kausalya Kumari Sahu, Additional Professor, Department of Pathology,
Kasturba Medical College, Mangalore, Karnataka, India.
Address for Correspondence
Dr. Sunil Kumar Shetty,
Assistant Professor,
Dept. of General Surgery,
Kasturba Medical College,
Attavar, Mangalore,
Karnataka, India.
E-mail:
sunildr14@yahoo.co.in
Citation
Shetty SK, Prasad K, Bhat S, Soma S, Sahu KK. Splenic Epithelial Cyst With Elevated Ca 19-9 Levels. Online J Health Allied Scs.
2014;13(1):11. Available at URL:
http://www.ojhas.org/issue49/2014-1-11.html
Open Access Archives
http://cogprints.org/view/subjects/OJHAS.html
http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Dec 8,
2013; Accepted: Apr 15, 2014; Published: May 15, 2014 |
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Introduction:
Splenic cysts are very unusual in everyday surgical practice. They can be parasitic or non-parasitic. Nonparasitic cysts are classified as primary (true, epithelial), lined by an epithelial cover (epidermoid, dermoid and mesothelial) or endothelial cover (hemangioma, lymphangioma), and secondary (pseudocysts, nonepithelial), which are usually of post-traumatic origin.1,2 In the last decade, approximately 30 cases of epithelial splenic cysts associated with high serum concentrations of CA 19-9 were reported. We present a patient with a large epithelial splenic cyst accompanied by high levels of CA 19-9 in the serum.
Case Report
A 19 years old female presented to us with a palpable mass per abdomen in the left upper quadrant which has been gradually increasing in size over the past one year. The mass was associated with constant, dull aching and non radiating pain over the past one month duration. There were no other symptoms of note. The patient does not give any history of trauma. Patient does not have any comorbid illnesses or any previous surgical history. General physical examination was within normal limits. Per abdominal examination revealed a 18x10cm mass occupying her left upper abdominal quadrant. Mild tenderness could be elicited. There was no hepatomegaly. Rest of the abdominal and systemic examination was unremarkable. Her routine blood investigations were normal. CA 19-9 was very high (1118 units/ml) and CEA was normal.
Ultrasonography of the abdomen revealed a large cystic mass with papillary projections in epigastrium extending towards pancreatic tail region. Contrast enhanced computerized tomography of the abdomen confirmed the cystic mass arising from the spleen abutting the tail of pancreas.
At laparotomy, a huge cyst was found emerging from the hilum of the spleen and compressing the rest of the splenic parenchyma(Figure1). These findings resulted in total splenectomy(Figure 2) which was followed by an uneventful postoperative recovery.
During the 4 weeks after the operation, CA 19-9 dropped to normal value. Pathological examination of the specimen confirmed a benign, true, epithelial splenic cyst.
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Figure 1: Cyst from the hilum of the spleen, compressing the rest of the splenic parenchyma. |
Figure 2: After total splenectomy |
Discussion
Splenic epithelial cysts occur predominantly in the second and third decades of life but can occur in children and even in infants.3 Small cysts are usually asymptomatic. An asymptomatic painless abdominal mass is the presenting feature in 30-45% of the cases. Splenic cysts may present with localized or referred pain relating to splenomegaly, abdominal distension and mass effect. Rarely, these cysts may present with acute symptoms related to rupture, haemorrhage or infection.
Ultrasonography allows the distinction to be made between cysts (anechoic or hypoechoic) and solid masses (isoechoic or hyperechoic). An abdominal CT scan is helpful in determining both the cyst's site of origin and its relationship to surrounding structures. Epithelial splenic cysts are characteristically unilocular, anechoic lesions with smooth, well-defined margins. This is the most common pattern in small (<5 cm) cysts. The echogenicity, which is the most common finding in large (>5 cm) cysts, is due to cholesterol crystals or other breakdown products of hematoma. The smaller size of the anechoic cysts compared with the echogenic ones suggests that large cysts are more prone to episodes of intracystic hemorrhage.4,5
Histologically, epidermoid cysts have a squamous epithelial lining with intracellular bridges and a thick collagenous wall. The interior cyst wall may be composed of thick trabeculated fibrous bands covered by epithelium. The cystic fluid may contain cholesterol crystals, protein particles or breakdown products of hemorrhage. The cellular lining of congenital cysts is thought to arise from infolding of the peritoneal mesothelium following splenic capsule rupture or from mesothelial cells trapped in the splenic sulci. The mesothelium undergoes metaplasia to squamous epithelium secondary to chronic irritation.
True cysts are characterized by a squamous epithelial lining, and many are considered congenital. These epithelial cells are often positive for CA 19-9 and CEA by immunochemistry, and patients with epidermoid cysts of the spleen may have elevated serum levels of one or both of these tumor-associated antigens.6 Despite the presence of these tumor markers, these cysts are benign and apparently do not have malignant potential greater than any other native tissue.
An association between serum levels of CA 19-9 and splenic cysts was first reported in 1994. More than 30 cases of benign true splenic cysts associated with elevated CA 19-9 serum levels have been published. For the majority, the origin of this tumor marker is the inner epithelial lining of the cyst, as demonstrated by histochemical analysis. Our patient had an elevated CEA level as well, which dropped to normal after surgery. The elevation of CA 19-9 is supposedly due to the variability of the cellular differentiation of the cells in the cyst wall.
Operative intervention is indicated for symptomatic cysts and for large cysts. Symptomatic splenic cysts have been considered as high-risk lesions for future automatic rupture, which explains why most authors include them in the indications for surgical treatment (either splenectomy or more conservative operations).7-9 Rupture is more likely in lesions exceeding 4-5 cm in diameter, thus setting this size as the minimal indication for asymptomatic cyst operative treatment.10 Today the optimal treatment options are partial splenectomy, total cystectomy, marsupialization, or cyst decapsulation (unroofing), accessed either by open laparotomy or laparoscopy.
However, any type of conservative procedure is difficult to perform, if the cyst is very large, is located in the splenic hilum, or is covered completely by the splenic parenchyma (intrasplenic cyst), or if there are multiple cysts (polycystic cases). In these cases, a complete splenectomy should be performed.
References
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- Williams RJ, Glazer G. Splenic cysts: changes in diagnosis, treatment and aetiological concepts. Ann R Coll Surg Engl. 1993;75(2):87-89.
- Sakamoto Y, Yunotani S, Edakuni G, Mori M, Iyama A, Miyazaki K. Laparoscopic splenectomy for a giant splenic epidermoid cyst: report of a case. Surg Today. 1999;29:1268-1272.
- Siniluoto TMJ, Paivansalo MJ, Lahde ST, Alavaikko MJ, Lohela PK, Typpo ABT, Suramo IJI. Nonparasitic splenic cyst. Ultrasonographic features and follow-up. Acta Radiologica. 1994;35:447-451.
- Kaufman RA, Silver TM, Wesley JR. Preoperative diagnosis of splenic cysts in children by gray scale ultrasonography. J Pediatr Surg. 1979;14:450-454.
- Yigitbasi R, Karabicak I, Aydogan F, Erturk S, Bican O, Aydin O, Kantarci F. Benign splenic epithelial cyst accompanied by elevated Ca 19-9 level: a case report. Mt Sinai J Med. 2006;73:871-873.
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- Hansen M, Moller A. Splenic cysts. Surg Laparosc Endosc Percutan Tech. 2004;14:316-322.
- Gianom D, Wildisen A, Hotz T, Goti F, Decurtins M. Open and laparoscopic treatment of nonparasitic splenic cysts. Dig Surg.2003;20:74-78
- Mirilas P, Demetriades DM, Siatitsas YS. Epithelial (epidermoid) splenic cysts in childhood: surgical management of eight cases. Am Surg. 2002;68:134-138.
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