Hamartoma quístico retrorrectal (tailgut cyst): reporte de un caso y revisión de la literatura / Retrorectal Cystic Hamartoma (Tailgut cyst): Case Report and Literature Review

Resumen Los hamartomas quísticos retrorrectales (tailgut cyst) son tumores congénitos multiloculados, poco frecuentes y derivados de remanentes embrionarios posanales que a menudo no se diagnostican debido a su rara incidencia, localización anatómica y su presentación clínica inespecífica. Presentamos el caso de un paciente de 21 años con historia de fístula perianal que fue intervenida, pero presentó recidiva y en la resonancia se encontró el hamartoma quístico. El tratamiento definitivo fue la resección completa de la lesión por vía posterior (Kraske-Mason).

Abstract Retrorectal Cystic Hamartoma (tailgut cyst [TGC]) are uncommon, multiloculated congenital tumors derived from embryonic post-anal or tail gut remnants often undiagnosed due to their rare incidence, anatomical location, and non-specific clinical presentation. We presented a 21-year-old patient with a perianal fistula history who underwent surgery. Nonetheless, she showed recurrence, and the cystic hamartoma was found in the resonance imaging. Therefore, the definitive treatment was complete resection of the lesion by posterior approach (Kraske-Mason).

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)

Clinicopathological features of retrorectal tumors in adults: 9 years of experience in a single institution / 대한외과학회지

PURPOSE: Primary tumors of the retrorectal space in adults are very rare. Most of them are benign masses, but malignant masses are reported on occasion. This study aimed to investigate the clinicopathological features of retrorectal tumors. METHODS: The medical records of fifteen patients who underwent surgical resection of a retrorectal tumor from March 2002 to April 2010 in our hospital were reviewed retrospectively. RESULTS: Out of 15 patients, thirteen were females and two males. About 1.7 patients were diagnosed with retrorectal tumor annually in our hospital. The incidence is one per 1,500 surgeries performed under general anesthesia. An anterior approach was performed in eight patients and a posterior approach with excision of the coccyx in five patients. Combined approach was performed in two patients. Four patients (three in abdominal approach and one in combined approach) underwent laparoscopic resection. The mean size of tumors was 6.2 +/- 2.9 cm. Mature teratoma (four) and neurilemmoma (four) were the most common tumors. Except for one case of chondrosarcoma, fourteen tumors were confirmed to be of benign nature in histologic examination. Patients who underwent a transabdominal approach with laparoscopic surgery had no postoperative complication and had a tendency to experience earlier recovery than those with open surgery. CONCLUSION: Surgical resection of a retrorectal tumor is recommended to relieve pressure symptoms and to confirm the diagnosis. A laparoscopic approach may offer excellent visualization of the deep structures in the retrorectal space, reduce surgical trauma, and be helpful for early postoperative recovery.

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.

Perineal Reconstructive Surgery of a Cloaca-Like Perineal Defect: A Case Report

Retrorectal or presacral tumors are rare, with incidences of approximately 0.01%. Generally, these tumors have no specific symptoms and are likely to be found incidentally on CT or MRI scans. Most of them are benign masses, but malignant masses are reported on occasion. The modality of treatment is a resection in cases of a benign mass, but chemotherapy or radiation therapy can be done in cases of malignancy. Like our case, a matured cystic teratoma in the retrorectal area is very rare. In addition, no laparoscopic resection of a retrorecal teratoma was reported until 2008. Thus, we report the case of a 31-yr-old female patient who underwent a total laparoscopic excision for a matured cystic teratoma in the retrorectal space.

Clinicopathological Features of Retrorectal Tumors in an Adult: A Case Report and Review of the Literatures

Retrorectal tumors are particularly rare among the adult population, occurring in 1 of 40,000 hospital admissions. Clinical diagnosis is difficult and is often delayed because of vague symptoms. This study aimed to investigate the clinicopathological features of retrorectal tumors. Between January 1999 and March 2005, 10 patients were diagnosed with retrorectal tumors at the Department of Surgery, Yonsei University Medical Center, and their medical records were reviewed. We analyzed chief complaints, imaging studies, surgical approaches and pathologic examinations. Out of 10 patients, 8 were female and 2 were male. The mean age was 42.8 years. Four patients had no symptoms. Perianal and abdominal pain were the most common presentations. CT and MRI were the most frequently performed imaging studies. Surgery was performed in 9 patients. Postoperative pathologic diagnosis was possible in 9 patients. An epidermal cyst was the most common tumor (4 patients); others included a mature teratoma, an adenocarcinoma from a tail gut cyst, a duplication cyst, a neurogenic tumor, and a smooth muscle cell tumor. Imaging techniques like CT scans, MRI and TRUS are helpful to determine the size and the extent of a tumor and its relationship to the surrounding anatomical structures for the operative approach. A surgical resection is the standard of treatment and demonstrates good results and a good prognosis.

A Retrorectal Neurofibroma Resected by Transsacral Approach

Tumors in the retrorectal area are rare. Surgical approaches to retrorectal tumors include posterior, anterior, or combined approaches. We have encountered a case of a 56- year-old woman with a retrorectal tumor. She had a solid tumor, 3 cm in size, in the posterior midline rectum 7 cm above the anal verge. The tumor was successfully resected by using a posterior (transsacral) approach with a partial sacrectomy and was diagnosed as being a neurofibroma. There were no postoperative complications.