Epidemiology, diagnostic approach and therapeutic management of tailgut cysts: A systematic review

Abstract

Background: Tailgut cysts (TGCs) are benign congenital abnormalities that usually present with non-specific symptoms, constituting a diagnostic dilemma for physicians. The aim of this study was to systematically review the literature concerning clinical manifestations, diagnostic modalities and histologic findings of TGCs and highlight current knowledge on therapeutic management of this rare entity.

Mucinous adenocarcinoma arising from a tailgut cyst: A case report

“…Primary retrorectal tumours include congenital (55%-65% of all tumours in this region), neurogenic (10%-12%), osteogenic (5%-11%), inflammatory (5%), and other tumour types (5%-11%). According to their embryonic origin, cysts are classified into epidermal, dermal, neural, teratoma, enteric, rectal duplication, mucous-secreting, enterogenous, simplex, gland anal, rectal, hamartoma, and TCs[ 5 , 8 ]. TCs are found in the presacral space and are typically thin-walled cysts that may be single or multiloculated, branched, and may contain green opalescent colloid fluid[ 2 ].…”

“…Hjermstad and Helwig reported the largest case series of TCs, which included 53 patients with an age range of 4 days to 73 years and average age of 36 years[ 10 ]. Based on the current literature, TCs may be asymptomatic or present with non-specific symptoms owing to the large size of the pelvic mass[ 2 , 5 , 8 ]. They can also lead to several complications including a neurogenic bladder, haemorrhage, faecal incontinence, faecal fistula, intestinal obstruction, infections, or malignant transformation as observed in the present case[ 11 , 12 ].…”

“…TCs are congenital lesions that develop from the residual posterior remnant of the intestine, which retains its structure and architecture regarding the mature ectodermal, endodermal, and mesodermal tissue elements. The lining epithelia may vary, including squamous, ciliated columnar, pseudostratified, columnar, transitional, goblet columnar, and cuboidal epithelia[ 8 , 14 ]. Additionally, it is characterised by the presence of a smooth muscle layer and connective tissue, which may be disarrayed and do not encompass the nerve plexus or differentiated neuronal cells[ 14 ].…”

“…Factors that determine prognosis include the stage during diagnosis, tumour histology and grade, and completeness of resection[ 3 ]. Compared with neuroendocrine tumours, adenocarcinomas arising from TCs have a poorer prognosis and carry a risk of local recurrence and metastasis[ 8 ]. Follow-up of the patient is also recommended, including monitoring for signs of recurrence with periodic positron emission tomography-CT and MRI scans in addition to serum CEA levels that serve as an indicator of the tumour’s response to treatment and development of recurrence[ 19 ].…”

The tail of the giant tailgut cyst

“…Sixty percent of all congenital presacral lesions are tailgut cysts. Patients typically present between the third and fifth decades of life with a female to male predilection (3:1) 1 . Embryologically, they can develop from all three germ layers 2 .…”