Laparoscopic Resection Combined with a Transsacral Approach for a Recurrent Tailgut Cyst with a Refractory Fistula.

Tailgut cyst is a rare cystic disease of the anterior sacral surface and the remains of an embryonic tail gut. Tailgut cysts have a potential for malignancy, and complete resection with an adequate surgical margin is necessary. Even if incomplete resection does not result in recurrence of malignant disease, there is a risk of local infection leading to refractory fistulas. The optimal treatment for such refractory recurrent lesions has not been reported. We describe a case in which the combination of laparoscopic and transsacral approaches was effective for resecting a recurrent refractory fistula after incomplete resection of a tail gut cyst.

Minimally invasive approach for retrorectal tumors above and below S3: a multicentric tertiary center retrospective study (MiaRT study).

BACKGROUND: Retrorectal tumors are uncommon lesions developed in the retrorectal space. Data on their minimally invasive resection are scarce and the optimal surgical approach for tumors below S3 remains debated. METHODS: We performed a retrospective review of consecutive patients who underwent minimally invasive resection of retrorectal tumors between 2005 and 2022 at two tertiary university hospital centers, by comparing the results obtained for lesions located above or below S3. RESULTS: Of over 41 patients identified with retrorectal tumors, surgical approach was minimally invasive for 23 patients, with laparoscopy alone in 19, with transanal excision in 2, and with combined approach in 2. Retrorectal tumor was above S3 in 11 patients (> S3 group) and below S3 in 12 patients (< S3 group). Patient characteristics and median tumor size were not significantly different between the two groups (60 vs 67 mm; p = 0.975). Overall median operative time was 131.5 min and conversion rate was 13% without significant difference between the two groups (126 vs 197 min and 18% vs 8%, respectively; p > 0.05). Final pathology was tailgut cyst (48%), schwannoma (22%), neural origin tumor (17%), gastrointestinal stromal tumor (4%), and other (19%). The 90-day complication rates were 27% and 58% in the > S3 and < S3 groups, respectively, without severe morbidity or mortality. After a median follow-up of 3.3 years, no recurrence was observed in both groups. Three patients presented chronic pain, three anal dysfunction, and three urinary dysfunction. All were successfully managed without reintervention. CONCLUSIONS: Minimally invasive surgery for retrorectal tumors can be performed safely and effectively with low morbidity and no mortality. Laparoscopic and transanal techniques alone or in combination may be recommended as the treatment of choice of benign retrorectal tumors, even for lesions below S3, in centers experienced with minimally invasive surgery.

Cystic lesions of the retrorectal space.

Cysts of the retrorectal space comprise a heterogeneous group of rare lesions. Most develop from embryological remnants and include tailgut cysts, dermoid cysts, rectal duplication cysts, anal canal duplication cysts, sacrococcygeal teratomas and anterior meningocoele. Tailgut cyst is the most common cyst of developmental origin, usually presenting as a multilocular cystic mass with mucoid content and lined by multiple epithelial types. Compared with tailgut cysts, rectal duplication cysts display all layers of the large bowel wall including a well-defined muscularis propria. Retrorectal cysts of non-developmental origin are far less common and represent lesions that either infrequently involve the retrorectal space or undergo extensive cystic change. This review provides an overview of the various histological types of cystic lesions of the retrorectal space, divided into cysts of developmental origin and those of non-developmental origin. A practical pathological and multidisciplinary approach to diagnosing these lesions is presented.

Risk of malignancy and outcomes of surgically resected presacral tailgut cysts: A current review of the Mayo Clinic experience.

AIM: The aim of this study was to describe the surgical management, outcomes and risk of malignancy of presacral tailgut cysts. METHOD: A retrospective analysis of all patients who underwent resection of tailgut cyst at Mayo Clinic in Arizona, Florida and Minnesota between 2008 and 2020 was performed. Demographics, presentation, evaluation, surgical approach, postoperative complications, pathology and recurrence rates were reviewed. RESULTS: Seventy-three patients were identified (81% female) with a mean age of 45 years. Thirty-nine patients (53%) were symptomatic, most commonly with pelvic pain (26 patients). Digital rectal examination identified a palpable mass in 68%. Mean tumour size was 6 cm. Resection was primarily performed through a posterior approach (77%, n = 56), followed by a transabdominal approach (18%, n = 13) and a combined approach (5%, n = 4). Six patients underwent a minimally invasive resection (laparoscopic/robotic). Coccygectomy or distal sacrectomy was performed in 41 patients (56%). Complete resection was achieved in 94% of patients. Thirty-day morbidity occurred in 18% and was most commonly wound related; there was no mortality. Malignancy was identified in six patients (8%). For the 30 patients with follow-up greater than 1 year, the median follow-up was 39 months (range 1.0-11.1 years). Local recurrence was identified in three patients and distant metastatic disease in one patient. CONCLUSION: The rate of malignancy in presacral tailgut cysts based on this current review was 8%. Overall recurrence was 5% at a median of 24 months.

Laparoscopic Synchronous Resection for Descending Colon Cancer and Tailgut Cyst.

A 67-year-old woman underwent polypectomy for a tumor at the descending colon. Pathologically, the tumor was diagnosed as adenocarcinoma with an invasion of 2000 µm. Computed tomography showed a swollen paracolic lymph node and a mass lesion in the presacral space. Magnetic resonance imaging revealed a multio-cular cystic lesion. On diagnosis of descending colon cancer and tailgut cyst, she underwent synchronous lapa-roscopic resection. Histopathologically, the colon cancer was diagnosed as pT1bN1M0, pStage IIIa. The pre-sacral cystic lesion was diagnosed as a nonmalignant tailgut cyst with negative surgical margin. The patient is currently doing well without recurrence at 28 months.

The Controversy of Surgical Intervention for Anal Canal Duplication in Children.

OBJECTIVE: Since the first definition of anal canal little has been discovered about the etiology of this rare condition. We present four asymptomatic cases of anal canal duplication with diverse clinical and surgical findings. METHODS: A retrospective chart review was performed on four infants presenting with asymptomatic anal canal duplication, born between 2014 and 2016. Clinical characteristics and pathologic findings of patients either by radiological imaging or pathology were evaluated. The primary outcome measure was the complications. RESULTS: All patients were followed-up with physical examination and ultrasound for a mean of 3.5±1.0 years, lastly seen at the beginning of 2018. The female to male ratio was 3:1. Duplicate anal canal length varied between 12-20mm, and two of the four patients had a presacral cystic mass confirmed as a tail gut cyst following surgery. At follow-up, none of the patients had developed symptoms related to anal canal duplication, regardless of whether they had surgical intervention. CONCLUSION: Though surgical management is the preferred treatment for anal canal duplication, it seems that patients who do not undergo surgery might remain free of symptoms, suggesting that surgical intervention may be unnecessary.

Malignant transformation of tailgut cysts is significantly higher than previously reported: systematic review of cases in the literature.

AIM: The best treatment for tailgut cysts has not been firmly established. We report a systematic review of the cases in the available literature in order to provide an evidence base for treatment. METHOD: A systematic search of articles wholly or partly in English was made of PubMed, Embase and Google Scholar; additional studies were discovered by searching reference lists and contacting authors directly. Search terms 'tailgut cyst', 'tail gut cyst', 'retrorectal hamartoma' and 'retrorectal tumour' were used for case reports or case series; no publication date restrictions were imposed. Only studies with histological confirmation of diagnosis and reporting the age and gender of patients were included. Papers were excluded by consensus between the first two authors. RESULTS: A total of 196 individual cases were analysed in detail including 51 cases of neoplasia. The overall rate of neoplastic transformation was 26.6%. Although the male:female cyst incidence ratio was 1:4, men over 18 had a significantly greater relative risk of neoplasm at 1.94 (P = 0.0055). Radiological evidence of nodular thickening of the cyst wall significantly increased the relative risk of the presence of cancer (P = 0.0023). CONCLUSIONS: Current orthodoxy that these are not dangerous embryological remnants is unfounded and may be false. The available data suggest the risk of malignant transformation is high and will apply to any residual tissue after excision. The same rationale behind total mesorectal excision in rectal cancer applies to tailgut cysts. Consequently they should be resected with similar oncological margins.

Tailgut cyst: report of three cases and review of the literature.

INTRODUCTION: Tailgut cysts or retrorectal cystic hamartomas are congenital developmental lesions which are often misdiagnosed due to their rare incidence, anatomical position and variable clinical presentation. CASE REPORT: We report three clinical cases: one of a 67-year old woman with high fever and anal bulging; the second case was a 50-year old woman with diffuse abdominal pain and the third case was a 52-year old woman with high fever and no abdominal or rectal pain. The rectal examination and MRI indicated the presence of a tailgut cyst. In all the cases a complete resection through a posterior perineal route was performed. Histopathological examination confirmed the diagnosis of a tailgut cyst, with a malignant component identified in the third case. DISCUSSION: The discussion presents a brief review of the relevant information described in the literature to highlight the cornerstones for appropriate diagnosis and treatment of a tailgut cyst. Tailgut cysts are to be considered in the differential diagnosis of retrorectal or presacral masses as malignant transformation can occur.

Does a retrorectal tumour remain a challenge for surgeons?

Background: Retrorectal tumours are lesions with a wide range of histological differentiation that are often diagnostic and clinical challenges due to their rare occurrence. Many cases of this pathology are treated in regional hospitals, which results in serious complications because physicians fail to recognize this pathology. We present our experience in treating these tumours. Methods: A retrospective analysis of a prospectively maintained database was performed using the Vilnius University Hospital Santaros Clinics patient database. Thirty-five cases were identified. Results: Occurrence of retrorectal tumours was higher in women than in men and accounted for 82.86% and 17.14%, respectively. Computer tomography and magnetic resonance imaging were the main methods used to confirm diagnosis and plan surgical treatment. We have used a laparotomy, perineal or combined approach for tumour extirpation. The laparotomy approach was the most used, followed by perineal extirpation. The most common histological type was cystic hamartoma, accounting for 20% of cases. In 80% of cases, the histological findings greatly varied. Hospital stays varied from 3 to 21 days with a mean of 11.6 ± 5.83 days. The postoperative complication rate was 17.14% and was present in six cases. Overall survival was 85.17%, with an average follow-up period of 71.83 months. There were no recurrent tumours diagnosed during follow-up. Conclusion: Retrorectal tumours are a very rare pathology with high histological heterogeneity and problematic diagnostics. Patients should be referred to a tertiary centre that has experience and diagnostic capabilities for the best diagnostic and treatment options.

Neuroencorine Tumor Arising within a Tailgut Cyst in an Adolescent Boy.

INTRODUCTION: Neuroendocrine tumors (NET) within tailgut cysts (TC) are usually seen in middle-aged females. A 14 year-old boy who underwent surgical excision of NET within TC is presented to discuss the clinical features, diagnosis and treatment options. CASE REPORT: A 14-year-old boy with rectal bleeding and constipation had a 5 × 4 cm mass detected upon rectal examination. The cystic mass was totally excised. The histopathological evaluation revealed a low-grade NET within mucularis layer of the cyst showing a positive immunhistochemical staining for cytokeratin and synaptophysin. CONCLUSION: Although, the malignant transition of TC is commonly observed in middle age females, males and children can also be affected. This is first report of a pediatric TC with malignant transformation. Total surgical excision of cysts is mandatory for local control of the disease and chemotherapy should be reserved for the patients who have positive surgical margins.

Management of tailgut cysts in a tertiary referral centre: a 10-year experience.

AIM: Tailgut cysts are rare congenital lesions typically presenting as presacral masses. A variable clinical presentation often leads to misdiagnosis and unsuccessful operations. METHOD: A retrospective analysis was performed of tailgut cysts presenting to one surgeon at St Mark's hospital between 2003 and 2013. The patient demographic data and clinicopathological and radiological features, together with perioperative details and recurrence, were reviewed. RESULTS: A total of 17 patients (15 women) with a median age of 35 (21-64) years were included in the study. The mean duration of symptoms before referral was 40 months, with sepsis predominating in 12 cases. Fifteen of the patients had previously undergone surgery (mean 2.9 procedures). A posterior surgical approach was adopted in all patients with a coccygectomy performed in 13. A loop colostomy was formed in three patients. Two of them went on to have a secondary pull-through operation after an initial failed local repair of rectal injury. One case was reported to show malignant degeneration on histological examination. There was one recurrence during a median follow-up period of 13 (3-36) months. CONCLUSION: Tailgut cysts are an uncommon yet important cause of chronic perianal sepsis. Suspicion should be raised in a patient, usually female, presenting with a history of unsuccessful procedures. Diagnosis can be made by clinical assessment and MRI. Complete excision usually resolves the problem.

Imaging of tailgut cysts.

Tailgut cysts are congenital lesions that arise from the primitive hindgut in the true embryonic tail but fail to regress during gestation. These lesions are rare and more frequently encountered later in life and more commonly in women, and are the most common primary retrorectal tumor. Tailgut cysts may be asymptomatic or cause rectal bleeding, pain, or symptoms related to mass effect on the rectum or bladder. Pathologically, tailgut cysts are typically multilocular, lined with a variety of epithelial cell types, and are most frequently benign. Imaging is the linchpin of diagnosis due risks associated with biopsy. The purpose of this pictorial review is to present the spectrum of imaging findings associated with tailgut cysts on CT and MRI with focus on the use of advanced MRI and diffusion-weighted imaging. We present case examples of tailgut cysts, their CT and MR imaging findings, and diagnostic and management considerations.

A carcinoid tumour arising within a tailgut cyst: a diagnostic challenge.

A 49-year-old man was referred with constipation that had lasted for a few months. On colonoscopy, a subepithelial tumour more than 4 cm in size was seen in the rectum. He underwent endoscopic ultrasound and pelvic magnetic resonance imaging. He was preoperatively diagnosed with a rectal duplication cyst based on imaging studies. However, the final histopathologic diagnosis after transanal excision of the rectal mass was rectal carcinoid tumour with tailgut cyst. Tailgut cysts are very rare congenital lesions in the presacral area and are most often discovered incidentally in middle-aged women. It is difficult to distinguish the imaging appearance of tailgut cysts from that of many other retrorectal cysts. Malignant transformation of tailgut cysts has been estimated to occur in 2 to 13% of cases. We report the diagnostic difficulties encountered in a case of carcinoid tumour arising from a tailgut cyst in a male patient.

A neuroendocrine tumor arising in a tailgut cyst: Case report and literature review.

INTRODUCTION AND IMPORTANCE: Cystic lesions in the retrorectal space include developmental abnormality, inflammatory process, and tumor-relevant cysts. Among them, the tailgut cyst is the most common lesion which is featured by the complex epithelium lining the wall. It is generally accepted that tailgut cysts are embryonic residues and are mostly benign, but there are also reports about malignant transformation and even metastasis. CASE PRESENTATION: A 44-year-old female complained a sacrococcygeal discomfort more than one year. The imaging diagnosis was an infectious cyst. After surgery, a solid region was defined in a cyst. Morphologically, the region was composed of bland epithelia forming glandular or ribbon-like structure, with round nuclei and fine chromatin. Immunohistochemically, the cells were positive for CK7, CD56 and synaptophysin. The Ki-67-positive cells were about 1 %. The final diagnosis is a low-grade neuroendocrine tumor arising in a tailgut cyst. The patient was living without recurrence by the follow-up of 20 months after surgery. CLINICAL DISCUSSION: By reviewing the previously reported NET arising from tailgut cysts, we summarized 29 cases of neuroendocrine neoplasms that reported detailed information, and the majority are women. We found that the higher-grade tumor presented a higher tendency of distant metastasis or recurrence after surgery. Complete resection and full evaluation by pathologists are necessary to get a correct diagnosis and avoid disease progression. CONCLUSION: We reported the rare case of NET G1 arising from a tailgut cyst and reviewed relevant reports, in order to broaden differential diagnoses when an isolated mass is identified in the retrorectal space.

Analysis of the clinicopathological features of tailgut cyst with emphasis on the development of neoplastic lesions.

Tailgut cyst is a rare congenital cyst occurring in the retrorectal space and development of neoplastic lesions in tailgut cyst has been reported. Due to the rarity of the tumor, the histogenesis of neoplastic lesions in tailgut cyst has remained elusive. In the present study, the clinicopathological features of tailgut cyst were analyzed with a particular focus on the development of neoplastic lesions. The clinicopathological features of four patients with tailgut cyst (one female and three males) were retrospectively reviewed. No symptoms were present in two patients. Perineal discomfort, and constipation and urinary retention, were described in the other two patients, respectively. Magnetic resonance imaging showed that the cystic lesions were hypointense on T1- and hyperintense on T2-weigted images in all patients. Histopathological analysis revealed that all lesions were multilocular, and cystic walls were covered by squamous and ciliated epithelia without nuclear atypia. The development of neoplastic lesions was noted in two patients. Dysplastic change composed of piling-up proliferation of glandular cells with mild to moderate nuclear atypia was present in one patient, and invasive adenocarcinoma with a dysplasia component was observed in another patient. Dysplasia of the glandular cells, as seen in two patients in the present series, may be a precursor lesion of invasive adenocarcinoma; therefore, adenocarcinoma arsing in tailgut cyst may show a dysplasia-carcinoma sequence. While the reported incidence of neoplastic lesions in tailgut cysts is ~9% or less, their frequency remains to be accurately determined. Therefore, complete surgical resection is important for the management of patients with tailgut cyst. Additional clinicopathological and molecular studies with large cohorts may be required to clarify the histogenesis of neoplastic lesion in tailgut cyst.

Surgical resection of retrorectal tumours in adults: long-term results in 47 patients.

AIM: Retrorectal tumours (RT) are uncommon, and diagnosis and management remain difficult. The aim of this study was to evaluate the results of the surgical management of RT in our institution. METHOD: Medical notes of all patients operated on for RT were reviewed. Clinical, radiological, surgical, histological data as well as morbidity and long-term results were noted. RESULTS: Forty-seven patients [34 women (72%), mean age 45.8 (range 17-85) years] underwent surgery for RT between 1997 and 2011. The commonest symptoms were pain (n = 31) and suppuration (n = 10). Thirty-nine (83%) patients underwent preoperative magnetic resonance imaging (MRI). Malignant lesions exhibited typical characteristics on MRI including heterogeneity (n = 5, 83%), solid appearance (n = 4, 67%), a low-T1 signal and high-T2 intensity (n = 5, 83%), enhancement after gadolinium injection (n = 5, 83%), irregular margin (n = 4, 67%) and extension above S3 (i = 5, 83%). A Kraske approach was used in 42 (89%) patients with resection of the coccyx in 25 (60%) and an abdominal or combined approach for the remaining five. Four patients developed complications (two haematoma, two abscess), but only one (haematoma) required reoperation. Histological examination showed 38 (80.9%) benign lesions. After a median follow-up of 71 (2-168) months, 5-year disease-free survival was 75% for malignant lesions and 93.1% for benign lesions (P = 0.023). Four (4/42; 9.5%) patients had moderate perineal pain after a Kraske approach, while no anal dysfunction was seen. CONCLUSION: Magnetic resonance imaging was the most helpful investigation for retrorectal tumours. The posterior trans-sacrococcygeal approach is the procedure of choice for complete resection for most, especially for benign and cystic lesions without extension above S2.

Malignant transformation of tailgut cyst to squamous cell carcinoma, a rare case with poor outcome.

Tailgut cyst, a type of retro-rectal cyst, is a rare condition requiring evaluation for malignant transformation. We report a case of squamous cell carcinoma arising in the retro-rectal cyst, in a 51-year-old female who underwent incomplete resection of the cyst and chemo-radiotherapy, subsequently became locally recurred and metastatic.

Liver metastases of a neuroendocrine tumor arising from a tailgut cyst treated with interventional locoregional therapies: a case report and review of the literature on recurrent cases.

A tailgut cyst is a rare, developmental cyst occurring in the presacral space. Although primarily benign, malignant transformation is a possible complication. Herein, we report a case of liver metastases after resection of a neuroendocrine tumor (NET) arising from a tailgut cyst. A 53-year-old woman underwent surgery for a presacral cystic lesion with nodules in the cyst wall. The tumor was diagnosed as a Grade 2 NET arising from a tailgut cyst. Thirty-eight months after surgery, multiple liver metastases were identified. The liver metastases were controlled with transcatheter arterial embolization and ablation therapy. The patient has survived for 51 months after the recurrence. Several NETs derived from tailgut cysts have been previously reported. According to our literature review, the proportion of Grade 2 tumors in NETs derived from tailgut cysts was 38.5%, and four of the 5 cases of Grade 2 NETs (80%) relapsed, while all eight cases of Grade 1 NETs did not relapse. Grade 2 NET may be a high-risk group for recurrence in NETs arising from tailgut cysts. The percentage of Grade 2 NETs in tailgut cysts was higher than that of rectal NETs, but lower than that of midgut NETs. To the best of our knowledge, this is the first case of liver metastases of a neuroendocrine tumor arising from a tailgut cyst that was treated with interventional locoregional therapies, and the first report to describe about the degree of malignancy of neuroendocrine tumors originating from tailgut cysts in terms of the percentage of Grade 2 NETs.

Promoting Laparoscopic Anterior Approach for a Very Low Presacral Primary Neuroendocrine Tumor Arising in a Tailgut Cyst.

BACKGROUND: Tailgut cysts are rare congenital lesions that develop in the presacral space. As they can potentially conceal primary neuroendocrine tumors, surgical excision is suggested as the treatment of choice. However, specific management guidelines have yet to be developed. A posterior approach is usually preferred for cysts extending to the third sacral vertebral body. Conversely, a transabdominal approach is preferred for lesions extending upward to achieve an optimal view of the surgical field and avoid injuries. CASE REPORT: Here, we report a case of a 48-year-old man suffering from perianal pain and constipation. Digital rectal examination and magnetic resonance imaging revealed a presacral mass below the third sacral vertebral body. A laparoscopic transabdominal presacral tumor excision was performed. The final histological diagnosis was a rare primary neuroendocrine tumor arising from a tailgut cyst. The postoperative course was uneventful, and no signs of recurrence were observed at the six-month follow-up. CONCLUSIONS: This study may help establish more well-grounded recommendations for the surgical management of rectal tumors, demonstrating that the laparoscopic transabdominal technique is safe and feasible, even for lesions below the third sacral vertebral body. This approach provided an adequate view of the presacral space, facilitating the preservation of cyst integrity, which is essential in cases of malignant pathologies.

Mucinous adenocarcinoma of a tailgut cyst.

A tailgut cyst is a rare congenital lesion that develops from a residual posterior remnant of the intestine and presents as a mass in the presacral space. They are generally asymptomatic or have atypical symptoms, are usually benign but may rarely become malignant. We report a case of a 37-year-old female who initially presented to the Surgical Department of Korle Bu Teaching Hospital, Accra, Ghana with a malignant tailgut cyst after having repeated surgical procedures for recurrent perianal infective pathologies but still had persistence of symptoms. The lesion was initially excised and found to be a dermoid cyst histologically. The mass recurred after a year and had a re-excision; the lesion was diagnosed histologically as mucinous adenocarcinoma. This report emphasizes the different forms of presentation of a patient with a tailgut cyst and the possibility of malignant transformation, as well as the presentation of this tailgut cyst which can be diagnosed using radiological investigations and histological findings. Funding: None.