A case of ruptured splenic cyst with elevated serum levels of CEA treated by laparoscopic unroofing

  • Masataka OkunoEmail author
  • Norihiro Yuasa
  • Eiji Takeuchi
  • Yasutomo Goto
  • Hideo Miyake
  • Hidemasa Nagai
  • Yuichiro Yoshioka
  • Kanji Miyata
Case Report


Splenic cysts are rare and tend to have elevated tumor markers, of which carbohydrate antigen (CA) 19-9 is the most frequently elevated. Therefore, splenic cysts with elevated serum carcinoembryonic antigen (CEA) levels and without CA19-9 elevation are extremely rare. A 26-year-old woman presented with sudden upper abdominal pain while sleeping. Contrast-enhanced computed tomography (CT) showed an 85-mm simple splenic cyst in the upper pole and a moderate amount of ascites around the spleen. The serum levels of CEA, but not CA19-9, were elevated. Spontaneous rupture of a splenic cyst was diagnosed. We performed elective laparoscopic unroofing. The histological findings revealed a stratified squamous epithelium on the inner surface of the cystic wall. On immunohistochemical examination, the squamous epithelium was found to be positive for cytokeratin (CK)7, negative for calretinin, and positive for CEA. The histological diagnosis was an epidermoid cyst. Three months after the operation, the elevated serum tumor marker levels of CEA were normalized. Splenic cysts with high levels of CEA and low levels of CA19-9 are extremely rare. Laparoscopic unroofing is a useful operative procedure for ruptured splenic cysts with elevated levels of serum tumor markers.


Epidermoid cyst Carcinoembryonic antigen Carbohydrate antigen 19-9 Tumor markers Laparoscopy 



We would like to thank American Journal Experts for their English language editing service.

Compliance with ethical standards

Conflict of interest

Masataka Okuno, Norihiro Yuasa, Eiji Takeuchi, Yasutomo Goto, Hideo Miyake, Hidemasa Nagai, Yuichiro Yoshioka and Kanji Miyata declare that they have no conflict of interest.

Human rights

All procedures followed have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

Informed consent was obtained from the patient for the publication of this report.


  1. 1.
    Sardi A, Ojeda HF, King D. Laparoscopic resection of a benign true cyst of the spleen with the harmonic scalpel producing high levels of CA 19-9 and carcinoembryonic antigen. Am Surg. 1998;64:1149–54.Google Scholar
  2. 2.
    Paksoy M, Karabicak I, Kusaslan R, et al. Laparoscopic splenic total cystectomy in a patient with elevated CA 19-9. JSLS. 2006;10:507–10.Google Scholar
  3. 3.
    Kubo M, Yamane M, Miyatani K, et al. Familial epidermoid cysts of the spleen: report of two cases. Surg Today. 2006;36:853–6.CrossRefGoogle Scholar
  4. 4.
    Maeda E, Okano K, Suto H, et al. Hybrid approach to laparoscopic decapsulation combined with splenic artery balloon occlusion in a patient with carbohydrate antigen 19-9 producing splenic cysts. Asian J Endosc Surg. 2017;10:459–62.CrossRefGoogle Scholar
  5. 5.
    Uludag M, Yetkin G, Citgez B, et al. Giant true cyst of the spleen with elevated serum markers, carbohydrate antigen 19-9 and cancer antigen 125. BMJ Case Rep. 2009. Scholar
  6. 6.
    Bresadola V, Pravisani R, Terrosu G, et al. Elevated serum CA 19-9 level associated with a splenic cyst: which is the actual clinical management? Review of the literature. Ann Ital Chir. 2015;86:22–9.Google Scholar
  7. 7.
    Chiarugi M, Galatioto C, Battini A, et al. Giant epidermoid cyst of the spleen with carbohydrate and cancer antigen production managed laparoscopically. Ann Ital Chir. 2006;77:443–6.Google Scholar
  8. 8.
    Chen YY, Shyr YM, Wang SE. Epidermoid cyst of the spleen. J Gastrointest Surg. 2013;17:555–61.CrossRefGoogle Scholar
  9. 9.
    Karasakalides A, Ganas E, Triantafillidou S, et al. Spontaneous rupture of a true splenic cyst diagnosed by laparoscopy. Dig Dis Sci. 2006;51:1829–32.CrossRefGoogle Scholar
  10. 10.
    Inokuma T, Minami S, Suga K, et al. Spontaneously ruptured giant splenic cyst with elevated serum levels of CA 19-9, CA 125 and carcinoembryonic antigen. Case Rep Gastroenterol. 2010;4:191–7.CrossRefGoogle Scholar
  11. 11.
    Kiriakopoulos A, Tsakayannis D, Papadopoulos S, et al. Laparoscopic management of a ruptured giant epidermoid splenic cyst. JSLS. 2005;9:349–51.Google Scholar
  12. 12.
    Tassopoulos A, Wein M, Segura A. Traumatic rupture of a giant congenital splenic cyst presenting as peritonitis. Radiol Case Rep. 2017;12:401–4.CrossRefGoogle Scholar
  13. 13.
    Ingle SB, Hinge Ingle CR, Patrike S. Epithelial cysts of the spleen: a minireview. World J Gastroenterol. 2014;20:13899–903.CrossRefGoogle Scholar
  14. 14.
    Ito Y, Shimizu E, Miyamoto T, et al. Epidermoid cysts of the spleen occurring in sisters. Dig Dis Sci. 2002;47:619–23.CrossRefGoogle Scholar
  15. 15.
    Robbins FG, Yellin AE, Lingua RW, et al. Splenic epidermoid cysts. Ann Surg. 1978;187:231–5.CrossRefGoogle Scholar
  16. 16.
    Poulose BK, Holzman MD. Chapter 56—The spleen. In: Townsend CM Jr. et al., editors. Sabiston textbook of surgery, 20th edn. Elsevier, Amsterdam; 2017. pp. 1556–71.Google Scholar
  17. 17.
    Shanthi V, Reddy VC, Rao NM, et al. Epithelial cyst of the spleen with squamous metaplasia: a rare entity. J Clin Diagn Res. 2014;8:FD05–FD06.Google Scholar
  18. 18.
    Matsubayashi H, Kuraoka K, Kobayashi Y, et al. Ruptured epidermoid cyst and haematoma of spleen: a diagnostic clue of high levels of serum carcinoembryonic antigen, carbohydrate antigen 19-9 and Sialyl Lewis x. Dig Liver Dis. 2001;33:595–9.CrossRefGoogle Scholar
  19. 19.
    Narimatsu H, Iwasaki H, Nakayama F, et al. Lewis and secretor gene dosages affect CA19-9 and DU-PAN-2 serum levels in normal individuals and colorectal cancer patients. Cancer Res. 1998;58:512–8.Google Scholar
  20. 20.
    Parra-Robert M, Santos VM, Canis SM, et al. Relationship between CA 19.9 and the Lewis phenotype: options to improve diagnostic efficiency. Anticancer Res. 2018;38:5883–8.CrossRefGoogle Scholar
  21. 21.
    Vestergaard EM, Hein HO, Meyer H, et al. Reference values and biological variation for tumor marker CA 19-9 in serum for different Lewis and secretor genotypes and evaluation of secretor and Lewis genotyping in a Caucasian population. Clin Chem. 1999;45:54–61.Google Scholar
  22. 22.
    Papadopoulos IN, Davatzikos A, Kasabalis G, et al. Primary epithelial splenic cyst with micro-rupture and raised carbohydrate antigen CA 19-9: a paradigm of management. BMJ Case Rep. 2010. Scholar
  23. 23.
    Weledji EP. Benefits and risks of splenectomy. Int J Surg. 2014;12:113–9.CrossRefGoogle Scholar
  24. 24.
    King H, Shumacker HB. Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg. 1952;136:239–42.CrossRefGoogle Scholar
  25. 25.
    Elit L, Aylward B. Splenic cyst carcinoma presenting in pregnancy. Am J Hematol. 1989;32:57–60.CrossRefGoogle Scholar
  26. 26.
    Hirota M, Hayashi N, Tomioka T, et al. Mucinous cystadenocarcinoma of the spleen presenting a point mutation of the Kirsten-ras oncogene at codon 12. Dig Dis Sci. 1999;44:768–74.CrossRefGoogle Scholar
  27. 27.
    Morgenstern L. Nonparasitic splenic cysts: pathogenesis, classification, and treatment. J Am Coll Surg. 2002;194:306–14.CrossRefGoogle Scholar
  28. 28.
    Garza-Serna U, Ovalle-Chao C, Martinez D, et al. Laparoscopic partial splenectomy for congenital splenic cyst in a pediatric patient: case report and review of literature. Int J Surg Case Rep. 2017;33:44–7.CrossRefGoogle Scholar
  29. 29.
    Breitenstein S, Scholz T, Schäfer M, et al. Laparoscopic partial splenectomy. J Am Coll Surg. 2007;204:179–81.CrossRefGoogle Scholar
  30. 30.
    Salky B, Zimmerman M, Bauer J, et al. Splenic cyst–definitive treatment by laparoscopy. Gastrointest Endosc. 1985;31:213–5.CrossRefGoogle Scholar
  31. 31.
    Cianci P, Tartaglia N, Altamura A, et al. A recurrent epidermoid cyst of the spleen: report of a case and literature review. World J Surg Oncol. 2016;14:98.CrossRefGoogle Scholar
  32. 32.
    Mertens J, Penninckx F, DeWever I, et al. Long-term outcome after surgical treatment of nonparasitic splenic cysts. Surg Endosc. 2007;21:206–8.CrossRefGoogle Scholar
  33. 33.
    Kimber C, Spitz L, Drake D, et al. Elective partial splenectomy in childhood. J Pediatr Surg. 1998;33:826–9.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Gastroenterology 2019

Authors and Affiliations

  1. 1.Department of SurgeryJapanese Red Cross Nagoya Daiichi HospitalNagoyaJapan

Personalised recommendations