Yonsei Med J.  2005 Aug;46(4):555-561. 10.3349/ymj.2005.46.4.555.

A Carcinoembryonic Antigen-Secreting Adenocarcinoma Arising in Tailgut Cyst : Clinical Implications of Carcinoembryonic Antigen

• Cho BC
• Kim NK
• Lim BJ
• Kang SO
• Sohn JH
• Roh JK
• Choi ST
• Kim SA
• Park SE

• KMID: 1716527
• DOI: http://doi.org/10.3349/ymj.2005.46.4.555

Abstract

Tailgut cysts (TGCs) are rare congenital cysts that occur in the retrorectal or presacral spaces. Although most tailgut cysts have been reported as benign, there have been at least 9 cases associated with malignant change. We report herein on an unusual case of a 40-year-old woman with a carcinoembryonic antigen (CEA) -producing adenocarcinoma arising within a TGC who underwent surgical resection and local radiation therapy. Despite the complete resection, metastatic adenocarcinoma developed five months after surgery. CEA-producing adenocarcinoma from a TGC is extremely rare and only two cases, including this case, have been reported in the English medical literature. Besides CEA, the serum levels of CA 19-9 became markedly elevated in this patient. Given that the serum CEA level decreased to the normal range after complete resection of tumor and that the tumor recurrence was associated with a rebound of the CEA serum level, our case shows that serial measurements of serum CEA can be used for treatment planning and for assessing the patient's treatment response for this rare disease.

Keyword

Tailgut cyst; developmental cyst; presacral space; adenocarcinoma; carcinoembryonic antigen

MeSH Terms

Adenocarcinoma/blood/pathology/*therapy
Adult
CA-19-9 Antigen/blood
Carcinoembryonic Antigen/*blood
Cysts/blood/pathology/*therapy
Female
Hamartoma/blood/pathology/*therapy
Humans
Rectal Neoplasms/blood/pathology/*therapy
Sacrococcygeal Region

Figure

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  Fig. 1 Barium enema study showing left-anterior displacement of the posterior wall of the rectum (arrows).

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  Fig. 2 (A) Saggital T1-weighted MRI scan showing presacral lesion with intermediate signal intensity invading surrounding structures (arrow). (B & C) Axial T1- and T2-weighted MRI scan showing multicystic mass with intermediate signal intensity (thick arrow). The cyst shows hypointense and hyperintense signal, respectively (thin arrow).

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  Fig. 3 On sectioning, the mass displays a firm gray-white appearance (thick arrow) and contains several, discrete cysts (thin arrow) filled with yellow gelatinous material. The ill-defined mass impinges on the outer wall of rectum, presacral soft tissue and coccygeal bone.

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  Fig. 4 (A) The tumor consisits of many scattered malignant glandular components with infiltrative growth to surrounding tissue (× 100 H&E stain). (B & C) The wall of cysts are lined by benign-looking ciliated columnar (B), columnar (C) and focal cuboidal epithelium (not shown), which is consistent with TGC (× 200 H&E stain).

Reference

1. Hjermstad BM, Helwig EB. Tailgut cysts, report of 53 cases. Am J Clin Pathol. 1998. 89:139–147.

2. Maruyama A, Murabayashi K, Hayashi M, Nakano H, Isaji S, Uehara S, et al. Adenocarcinoma arising in a tailgut cyst: report of a case. Surg Today. 1998. 28:1319–1322.

3. Moreira AL, Scholes JV, Boppana S, Melamed J. p53 Mutation in adenocarcinoma arising in retrorectal cyst hamartoma (tailgut cyst): report of 2 cases-an immuohistochemistry/immunoperoxidase study. Arch Pathol Lab Med. 2001. 125:1361–1364.

4. Schwarz RE, Lyda M, Lew M, Paz IB. A carcinoembryonic antigen-secreting adenocarcinoma arising within a retrorectal tailgut cyst: clinicopathologic considerations. Am J Gastroenterol. 2000. 95:1344–1347.

5. Graadt van Roggen JF, Welvaart K, de Roos A, Offerhaus GJ, Hogendoorn PC. Adenocarcinoma arising within a tailgut cyst: clinicopathological description and follow up of an unusual case. J Clin Pathol. 1999. 52:310–312.

6. Lim KE, Hsu WC, Wang CR. Tailgut cyst with malignancy: MR imaging findings. Am J Roentgenol. 1998. 170:1488–1490.

7. Horenstein MG, Erlandson RA, Gonzalez-Cueto DM, Rosai J. Presacral carcinoid tumors: report of three cases and review of the literature. Am J Surg Pathol. 1998. 22:251–255.

8. Edelstein PS, Wong WD, La Valleur J, Rothenberger DA. Carcinoid tumor: an extremely unusual presacral lesion. Report a case. Dis Colon Rectum. 1996. 39:928–942.

9. Mourra N, Caplin S, Parc R, Flejou JF. Presacral neuroendocrine carcinoma developed in a tailgut cyst: report of a case. Dis Colon Rectum. 2003. 46:411–413.

10. Umar T, Mikel JJ, Poller DN. Carcinoma arising in a tailgut cyst diagnosed on fine needle aspiration (FNA) cytology. Cytopathology. 2000. 11:129–132.

11. Song DE, Park JK, Hur B, Ro JY. Carcinoid tumor arising in a tailgut cyst of the anorectal junction with distant metastasis: a case report and review of the literature. Arch Pathol Lab Med. 2004. 05. 128:578–580.

12. Chung KJ, Lee YL. Adenocarcinoma arising from the tailgut cyst (in Korean). Korean Radiol Society. 1995. 33:399–402.

13. Kang JW, Kim SH, Kim KW, Moon SK, Kim CJ, Chi JG. Unusual perirenal location of a tailgut cyst. Korean J Radiol. 2002. 3:267–270.

14. Ahn BY, Jeong CS, Lee DH, Yu CS, Lee HJ, Lee MK, et al. Tailgut cyst- A case report (in Korean). J Korean Soc Coloproctol. 1998. 14:617–620.

15. Lee H, Oh JH, Cho SY, Yang DM, Ha SY. Two cases of Tailgut cyst (in Korean). J Korean Soc Coloproctol. 2001. 17:209–212.

16. Lim MJ, Lee SN, Kim SS, Koo H, Kim OK. Tailgut cyst with Glomus Coccygeum (in Korean). Korean J Pathol. 1996. 30:643–645.

17. Johnson AR, Ros PR, Hjermstad BM. Tailgut cyst: Diagnosis with CT and Sonography. Am J Roentgenol. 1986. 147:1309–1311.

18. Marchena J, Acosta MA, Garcia-Anguiano F, Simpson H, Cruz F. Use of the preoperative levels of CEA in patients with colorectal cancer. Hepatogastroenterology. 2003. 50:1017–1020.

19. Jao SW, Beart RW Jr, Spencer RJ, Reiman HM, Ilstrup DM. Retrorectal tumors. Dis Colon Rectum. 1985. 28:644–652.

20. Bohm B, Milsom JW, Fazio VW, Lavery IC, Church JM, Oakley JR. Our approach to the management of congenital presacral tumors in adults. Int J Colorectal Dis. 1993. 8:134–138.

21. Wang JY, Hsu CH, Changchien CR, Chen JS, Hsu KC, You YT, et al. Presacral tumor: A review of forty-five cases. Am Surg. 1995. 61:310–315.