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

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. METHODS PubMed and Embase databases were systematically searched by two independent investigators (last search 18 February 2021) for studies concerning TGCs published in the past two decades. RESULTS Totally, 144 articles, including 135 case reports and 9 case series, met our inclusion criteria. One hundred eighty-four patients were included (3:1/female:male) with an age of 42.3 ± 18.7 years (mean, SD), while 5 cases concerned new-born infants. Pain was the prevailing clinical manifestation (41.8%), whereas 16.8% were asymptomatic. MRI and CT were utilized for diagnosis in 58.7% and 54.7% of cases, respectively. The majority of cysts were multilocular, while ciliated columnar epithelium and smooth muscles of the cyst wall were the prevailed histological findings. Malignant degeneration of TGCs was reported in 32.1% of cases, while carcinoid tumours were the most frequent malignancies. Surgical resection was performed in 155 cases, while laparoscopic and robotic approach was preferred in 18 and 2 cases, respectively. A posterior approach was implemented in 80.9%, anterior technique in 9% and combined technique in 6.7% of cases. Postoperative complications and recurrence of the cystic lesion were reported in 17.4% and 7.6% of cases, respectively. CONCLUSIONS TGCs constitute a dilemma for the physicians. Surgical resection comprises the ultimate treatment to avoid complications or malignant transformation of the cyst. Concrete follow-up strategies and optimal therapeutic options should be outlined through consensus guidelines and at the time being, such decisions can be made only on the basis of extrapolation and on a case-by-case approach.