Single-center Experience of 24 Cases of Tailgut Cyst

PURPOSE: Tailgut cysts are rare congenital or developmental lesions that arise from vestiges of the embryological hindgut. They are usually present in the presacral space. We report our single-center experience with managing tailgut cysts. METHODS: We conducted a retrospective analysis of 24 patients with tailgut cyst treated surgically at the Colorectal Surgery Department of Severance Hospital, Yonsei University, Seoul, South Korea, between 2007–2018. RESULTS: This study included 24 patients (18 females) with a median age of 51.5 years (range, 21–68 years). Ten cases were symptomatic and 14 were asymptomatic. Cysts were retrorectal in 21 patients. Cysts were below the coccyx level in 16 patients, opposite the coccyx in 6, and above the coccyx in 2. Cysts were supralevator in 5 patients, had a supra- and infralevator extension in 18 patients, and were infralevator in 1. Ten patients were managed using an anterior laparoscopic approach, 11 using a posterior approach, and 3 using a combined approach. Mean cyst size was 5.5 ± 2.7 cm. Postoperative complications were Clavien-Dindo (CD) classification grade II in 9 patients (37.5%) and CD grade III in 1 (4.2%). The posterior approach group showed the highest rate of complications (P = 0.021). Patients managed using a combined approach showed a larger cyst size (P < 0.001), longer operation times (P < 0.001), and a greater likelihood of tumor level above the coccyx (P = 0.002) compared to other approaches. The tumors of 2 male patients were malignant: 1 was a neuroendocrine tumor treated with radiotherapy, while the other was a closely followed adenocarcinoma. Median follow-up was 12 months (range, 1–66 months) with no recurrence. CONCLUSION: Tailgut cysts are uncommon but can cause perineal or pelvic pain. Complete surgical excision via an appropriate approach according to tumor size, location, and correlation with adjacent pelvic floor muscles is the key treatment.

Laparoscopic Resection of Presacral Tumor: A New Approach in the Era of the Minimally Invasive Surgery

Presacral tumors are rare; however, once diagnosed, surgical resection is recommended even in asymptomatic patients as there is potential risk for growth or malignant transformation. Many different types of surgical approaches to resect presacral tumors have been reported including posterior, anterior, and combined abdominosacral approaches. With introduction of the minimally invasive approach, laparoscopic or robotic approaches to resect presacral tumors are reported increasingly. We report a case of successful anterior laparoscopic resection for a presacral mass that was incidentally diagnosed during management of pancreatitis.

New surgical approach of retrorectal cystic hamartoma using transanal minimally invasive surgery (TAMIS) / Nova abordagem cirúrgica do hamartoma cístico retrorretal usando cirurgia transanal minimamente invasiva (TAMIS)

Introduction: Tailgut cysts or cystic hamartomas are rare developmental tumors of the pre-sacral space. Their true incidence is not well known but we must think on it when we have a patient with a multicystic uncapsulated tumor at presacral space. Tailgut cysts are often asymptomatic, and in other cases present with chronic perineal pain, constipation or rectal tenesmus. They must be treated to avoid complications such as infections (perianal fistula or abscess) and malignant degenerations (usually adenocarcinoma). Case report: One patient with anal pain was diagnosed with a perianal abscess. He was operated but at the second month review a MRI revealed a multilocular lesion in the presacral suggesting the presence of a retrorectal cystic hamartoma. An elective operation was performed by endoanal surgical approach using TAMIS. En bloc resection of the cyst was achieved with safe margins. The pathology result reported retrorectal cystic hamartoma. The patient was asymptomatic with no signs of recurrence in subsequent controls. Our experience, despite being based on one case, is evidence that TAMIS (Transanal Minimally Invasive Surgery) allows a minimally invasive dissection with similar benefits as the use of TEM/TEO devices. (AU)

Introdução: Tail gut cysts ou hamartomas císticos são tumores raros de desenvolvimento do espaço pré-sacral. Sua verdadeira incidência não é bem conhecida, mas devemos pensarnele quando temos um paciente com um tumor uncapsulated multicística no espaço présacral. Hamartomas císticos são muitas vezes assintomáticas, em outros casos, apresentase com dor perineal crônica, constipação ou tenesmo retal. Eles devem ser tratados para evitar complicações, como infecções (fístula perianal ou abscesso) e degenerações malignas (geralmente adenocarcinoma). Relato de Caso: Um paciente com dor anal foi diagnosticado com um abscesso perianal. Ele foi operado, mas na revisão do segundo mês uma ressonância magnética revelou uma lesão multilocular no pré-sacral, sugerindo a presença de um hamartoma cístico retrorretal. Uma operação eletiva foi realizada por abordagem cirúrgica endoanal usando TAMIS. A resseção em bloco do cisto foi conseguido com margens seguras. O resultado do exame patológico relatou hamartoma cístico retrorretal. O paciente encontrava-se assintomático, sem sinais de recidiva em controles posteriores. A nossa experiência, apesar de ser baseado em um dos casos, as provas que TAMIS (cirurgia transanal minimamente invasiva) permite um esvaziamento minimamente invasivo com benefícios similares como o uso de dispositivos de MET/TEO. (AU)

Tailgut cyst: An uncommonly diagnosed common entity - A case report and review of literature.

Tailgut cysts (TGC) though relatively common are rarely diagnosed and documented. Most of the times, they are misdiagnosed and mismanaged. When malignancy develops in TGC it is rarely attributed to it as it is undiagnosed earlier and later on there may not be any evidence left behind. We report one such case which was diagnosed though not preoperatively. Review of literature reveals only few such cases after the initial description by Hjermstad and Helwig in 1988. Surprisingly most of the reports especially the larger case series are by radiologists. Surgeons and pathologists have contributed very few cases. This case report is aimed at increasing the awareness about this entity so that it will be suspected, investigated properly and treated before development of complications like recurrent fistulae, ischioanal abscesses which increase the morbidity and life threatening complications like malignant change.

Clinical Study and Review of Articles (Korean) about Retrorectal Developmental Cysts in Adults

PURPOSE: A retrorectal developmental cyst (tailgut cyst, epidermoid cyst, dermoid cyst, teratoma, and duplication) is very rare disease, and the symptoms are not characteristic so that sometimes this disease is still misdiagnosed as a supralevator abscess or a complex anal fistula. We would like to present a clinical approach to this disease. METHODS: We retrospectively examined the charts of 15 patients who were treated for retrorectal cysts from January 2001 to November 2009. RESULTS: All 15 patients were female. The average age was 41 years (range, 21 to 60 years). Fourteen patients (93.3%) were symptomatic, and the most common symptom was anal pain or discomfort. Nine patients (60%) had more than one previous operation (range, 1 to 9 times) for a supralevator abscess, an anal fistula, etc. In 12 patients (80%), the diagnosis could be made by using the medical history and physical examination. Thirteen cysts (80%) were excised completely through the posterior approach. The average diameter of the cysts was 4.8 cm (range, 2 to 10 cm). Pathologic diagnoses were 8 tailgut cysts (53.3%), 5 epidermoid cysts (33.3%) and 2 dermoid cysts (13.3%). The average follow-up period was 18.3 months (range, 1 to 64 months). CONCLUSION: In our experience, high suspicion and physical examination are the most important diagnostic methods. If a female patient has a history of multiple perianal operations, a retrorectal bulging soft mass, a posterior anal dimple, and no conventional creamy foul odorous pus in drainage, the possibility of a retrorectal developmental cyst must be considered.

Tailgut Cyst in a Neonate

Tailgut cysts are rare congenital lesions thought to arise from vestiges of the tailgut/postanal gut, which is a primitive gut temporarily present at the most caudal portion of the embryo. The lesions are usually multilocular cysts lined by various types of epithelium in the retrorectal space. We experienced a tailgut cyst in an 18-day-old girl initially presented as an epidermoid cyst like skin lesion in the coccygeal region. MRI showed a well-defined multiloculated cystic mass between the rectum and coccyx, measuring 12x23 mm and markedly hyperintense on T2 weighted images and hypointense on T1-weighted images. The cyst was surgically removed via a posterior sagittal approach and microscopically lined by ciliated columnar, transitional and squamous cells. Tailgut cysts are usually found in adult females, and extremely rarely in neonates. We report a tailgut cyst in a neonate with a review of the literature.

The Diagnosis of Tailgut Cyst in the Setting of Elevated Serum CEA Levels

Tailgut cysts (TGCs) are rare congenital lesions arising from remnants of the postanal gut, which normally involutes by the 8th wk of embryonic development (the 3-8 mm stage). They are usually found in the retrorectal or presacral space. They often present in middle aged women with perirectal symptoms. CEA producing adenocarcinoma from a TGC is extremely rare, but such cases have recently been reported in the literature. The diagnosis has to differentiate between carcinomas of the colorectum, hamartomas, lymphomas, teratomas, chordomas, abscess formation, dermoid cysts, epidermoid cysts and enteral cysts. An elevated level of CEA may point to malignancy, but other causes must be excluded. Complete surgical resection is the therapy of choice. We report here on a case of a tailgut cyst that occurred in a 72-year-old male suffering from pneumoconiosis, and this patients showed unusual findings such as an unusual location and an elevated serum CEA level.

Carcinoid Tumor Arising within a Tailgut Cyst: A Case Report

Tailgut cysts are rare congenital lesions. They are believed to develop from remnants of the embryonic hindgut. Malignancy in tailgut cyst is extremely unusual. We experienced a case of a carcinoid tumor arising within a tailgut cyst at the presacral space. A 40-year-old woman was admitted for acute anal pain. Digital rectal examination revealed a 2-cm-sized submucosal tumor in the posterior midline rectum 4 cm above the anal verge. On sigmoidoscopic examination, the overlying rectal mucosa seemed to be intact. We performed a transanal incisional biopsy. The pathological diagnosis of the tumor biopsy revealed a malignant neuroendocrine tumor. The patient underwent an abdominoperineal resection. The tumor proved to be mutilocular cysts with a solid component. The cysts were diagnosed as tailgut cysts that were lined by a variety of epithelial types, including inner columnar cells, outer cuboidal cells, and transitional cells. The solid component in the cysts was confirmed as a carcinoid tumor on microscopic examination. Six months after the operation, she was found to have liver and brain metastases.

Adenocarcinoma Arising from Tailgut Cyst

Retrorectal cystic harmatoma; Tailgut cyst, is a rare congenital disease typically presented as presacral mass, and malignant change of this disease is extremely rare. Frequently, This disease is misdiagnosed or confused at initial time. So, we have a case of tailgut cyst and report the clinical symptom and the result. A 40-year-old woman has admitted at Severance hospital for the anal pain. About 6cm sized irregular cystic and solid mass in the retrorectal area involving coccygeal bone and right gluteal muscle tendon were detected in magnetic resonance image. Under the impression of malignant tailgut cyst, the patient underwent Hartmann's operation (abdomino-sacral approach) under the sacral resection (S4, 5). The pathology was adenocarcinoma (poorly differentiated) arising from a tailgut cyst involving rectal adventitia, gluteus muscle and sacral bone. For the following 4 months after the operation, The patient is doing well with no evidence of recurrence.

A Case of a Tailgut Cyst

The tailgut is a blind extension of the hindgut into the tail fold just distal to the cloacal membrane. Remnants of this structure may form a tailgut cyst. This is prone to infection and chronic fistula formation and has a long-term risk of malignancy. Non-recognition and incomplete treatment leads to morbidity. A twenty one year-old female patient visited our hospital with repeated perianal pain and discharge. Preoperative abdominopelvic MRI and endorectal ultrasonography revealed a retrorectal cyst suggestive of a tailgut cyst. She underwent a complete excision of the retrorectal mass through a presacral approach. She recovered uneventfully. This report includes the case and a brief review of tailgut cysts.

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

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.

Two Cases of Tailgut Cyst

Tailgut cysts in retrorectal or presacral space are rare and the derivatives of the embryonic post-anal gut. It is thought to arise from vestiges of embryonic hindgut. The lesions were usually multicystic and lined by a variety of epithelial types, including ciliated columnar, mucin-secreting columnar, transitional, and squamous epithelium. Tailgut cyst has been found in men and women of various ages but is more common in women and is usually associated with middle age. They may be the source of the chronic perirectal symptoms and rarely undergo malignant change, so early diagnosis and accurate evaluation is important. Complete surgical resection should be considered because of a long term risk of malignant change. We report two cases of tailgut cyst.

Tailgut Cyst A case report

Tailgut cyst is a rare congenital lesion in retrorectal space. The clinical significance of tailgut cyst presents its morbidity that occurs in the unrecognized and incompletely treated lesion. A forty four year-old female patient visited with lower abdominal pain during defecation. Preoperative abdominopelvic MRI and endorectal ultrasonography revealed a retrorectal mass suggestive of leiomyoma, dermoid cyst, teratoma, or duplication cyst of rectum. She underwent complete resection of retrorectal mass by transsphincteric approach. The mass was multilocular cyst lined by multiple types of epithelium. It was histologically confirmed as a tailgut cyst. She recovered uneventfully. This report includes the case and a brief review of tailgut cyst.

Tailgut Cyst with Glomus Coccygeum: Report of a case

Tailgut cyst of retrorectal space is uncommon and its classification and prognosis are unclear. The lesion usually consists of a multiloculated cyst lined by squamous, transitional, and glandular epithelium. Disorganized fascicles of smooth muscle may be seen in the wall. Glomus coccygeum, normally located at the tip of coccyx, is incidentally discovered in the presacral mass including tailgut cyst. We report a case of tailgut cyst in a 35 year old female who had internal hemorrhoid and intermittent anal pain for 3 years. Pelvic computerized tomography revealed a 3x2 cm sized mass in the presacral area. The mass was multicystic and lined by squamous, transitional, and peudostratified ciliated columnar epithelium with inflammatory cells and scattered smooth muscle in the wall. Dermal adnexal structures were not present, which excluded dermoid cyst. A glomus body was identified in the surrounding soft tissue.