Clinical Journal of Gastroenterology

, Volume 12, Issue 6, pp 539–551 | Cite as

Neuroendocrine tumour developing within a long-standing tailgut cyst: case report and review of the literature

  • Alice Lee
  • Thomas Surya SuhardjaEmail author
  • Thang Chien Nguyen
  • William Meng-Keat Teoh
Case Report


A tailgut cyst is a rare congenital lesion that can develop in the presacral space from the remnants of an embryonic hindgut. It is unusual for malignant change to occur in a tailgut cyst. We report a case of a large long-standing tailgut cyst, which was removed during a laparotomy. Histopathology showed a well-differentiated neuroendocrine tumour (primary carcinoid tumour) arising in a tailgut cyst. We reviewed the English literature for all adult cases with this condition. All original articles were reviewed, and data were compiled and tabulated. Including this report, 29 cases of NET developing in a tailgut cyst were found in the English literature. Tailgut cysts have been reported as more common in females, with a mean age of presentation in the fifth decade (Devine, in: Zbar A, Wexner S (eds) Coloproctology. Springer specialist surgery series, Springer, London, 2010; Hjermstad and Helwig in Am J Clin Pathol 89:139–147, 1988). Tailgut cysts may undergo malignant change including adenocarcinoma, sarcoma, and NET (Mathis et al. Br J Surg 97:575–579, 2010; Messick in Dis Colon Rectum 61:151–153, 2018; Patsouras et al. in Colorectal Dis 17:724–729, 2015; Chereau et al in Colorectal Dis 15:e476–e482, 2013). It is difficult to estimate the true incidence of malignant change in a tailgut cyst, with the literature reports only limited to case reports and small-case series. Although rare, our case confirms need to consider the possibility of a malignant component, even in a benign process such as a tailgut cyst. This prompts consideration for upfront definitive management.


Neuroendocrine Tailgut cyst Presacral cyst 



We would like to thank Dr Sukhpal Dayan (Anatomical Pathology, Monash Health) for her contribution of histopathology slides and information to the case report.

Compliance with ethical standards

Conflict of interest

Authors Alice Lee, Thomas Surya Suhardja, Thang Chien Nguyen and William Meng-Keat Teoh 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 all patients for being included in the study.


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Copyright information

© Japanese Society of Gastroenterology 2019

Authors and Affiliations

  1. 1.Colorectal Surgery Unit, Monash HealthDandenong HospitalDandenongAustralia
  2. 2.Department of Surgery, School of Clinical Sciences at Monash HealthMonash UniversityClaytonAustralia

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