Fifty-three examples of developmental "tailgut cysts" in the retrorectal space occurred predominantly in women and caused symptoms of mass effect or pain in 51%. The lesions were usually multicystic and lined by a variety of epithelial types, including ciliated columnar, mucin-secreting columnar, transitional, and squamous epithelium. Inflammation was present in 50%. In one patient a poorly differentiated adenocarcinoma was present. These cysts are most likely derived from remnants of the embryonic tailgut and differ from teratomas. Complete excision of the multilocular and multicystic process prevents recurrent draining sinuses and eliminates the possibility of malignant change.

Tailgut cysts : Report of 53 cases

Background.—Retrorectal cystic hamartomas, or tailgut cysts, are rare congenital lesions that typically present as presacral masses. These lesions are frequently clinically unrecognized and misdiagnosed. Malignant change is extremely rare. Only 10 additional cases with associated malignancy were recovered from the literature. We describe the clinicopathologic features of 5 cases, including 2 cases with malignant transformation. Results.—All patients were women (age range, 36–69 years). The most common symptoms were pain with defecation and rectal bleeding. One patient was asymptomatic. All lesions presented as multicystic presacral masses and all were surgically resected. The lesions varied in size from approximately 2 to 12 cm (average, 9.5 cm) and overall had similar histology composed of a variety of epithelial linings (stratified squamous, transitional, and simple or ciliated pseudostratified columnar). Skin adnexa, neural elements, and heterologous mesenchymal tissue, discriminators between ...

Retrorectal cystic hamartoma: report of 5 cases with malignancy arising in 2.

Gastrointestinal tract duplications are uncommon congenital abnormalities arising anywhere along the alimentary tract. The most common modalities used to image duplications are US and contrast medium examinations. CT and MRI are less often used, but can be helpful in difficult cases that require a multiplanar approach. In this article, we discuss and illustrate a wide spectrum of gastrointestinal tract duplications from the oesophagus to the rectum, and illustrate the associated abnormalities of gastrointestinal tract duplications.

Imaging features of gastrointestinal tract duplications in infants and children: from oesophagus to rectum

INTRODUCTION: Recurrent perianal sepsis is a difficult problem to manage in colorectal surgical practice. One cause is rectal duplication cyst, a rare congenital lesion that is easily overlooked. Many cases have associated congenital defects, especially musculoskeletal anomalies, and may provide a clue to the underlying condition. Early diagnosis is important because these cysts do not resolve spontaneously and may undergo malignant change. METHODS: We present two cases of middle-aged females who presented with perianal sepsis secondary to rectal duplication cyst. The first case had numerous surgical procedures for a perianal fistula during a ten-year period. She had associated sacral anomalies consistent with Currarino syndrome. The second case presented with a perineal mass after a bout of perianal inflammation. Both cases had the entire cyst surgically excised. RESULTS: There were no complications postoperatively and no recurrence at follow-up. Histopathology revealed no malignancy in the cyst. CONCLUSIONS: Rectal duplication cyst is a rare cause of recurrent perianal sepsis that should be considered in difficult cases, especially in those with associated musculoskeletal anomalies. Complete surgical excision is the preferred treatment to prevent recurrence and the risk of malignant degeneration. [Key words: Cyst; Duplication; Retrorectal; Rectum; Presacral]

Rectal duplication cyst presenting as perianal sepsis: report of two cases and review of the literature.

Tailgut cysts (retrorectal cystic hamartomas) are rare lesions occurring in the retrorectal/presacral space of young women, considered to be of developmental origin. In this study, we report the clinicopathological characteristics of an adenocarcinoma developing in an unusual tailgut cyst. A 47-year-old female presented with a 3-month history of an enlarging presacral mass, extending into the gluteal area. The patient underwent surgical resection. Microscopic examination revealed a mucinous adenocarcinoma with intestinal features arising in a tailgut cyst. In addition to the usual components previously described in this lesion (cuboidal, columnar, ciliated or squamous epithelial lining with scattered smooth muscle fibers in the wall), our case was unique in that it was associated with a meningothelial proliferation and benign thyroid tissue with oncocytic changes. These additional features bring into question the proposed hindgut origin of tailgut cysts.

Adenocarcinoma arising in a tailgut cyst with prominent meningothelial proliferation and thyroid tissue: case report and review of the literature

A case of a rare congenital malformation, duplication of the rectum, has been presented. The literature has been reviewed, various theories of etiology considered and the pathology, clinical features and treatment discussed.

James K.B. Purves

Papers

Duplications of the rectum