A 37-year-old woman with an uncomplicated pregnancy was admitted to our hospital for delivery of her baby. During the birthing process, it was found that her birth canal was obstructed by a large presacral mass. Delivery of the infant was by cesarian section and the mass was subsequently evaluated. Computed tomography and magnetic resonance imaging scans (Figure 1) showed a 5.6 × 5.3-cm, well-circumscribed, hypodense, cystic lesion in the presacral region to the left of midline. The patient underwent an exploratory laparotomy and excision of the mass. During surgery, the mass was adherent to, but easily separated from, the rectum. The patient made an uneventful postoperative recovery and was discharged home after 3 days. The surgical specimen consisted of 2 fragments of fibrofatty tissue, the larger of which measured 6 × 5 × 2.5 cm and represented a ruptured cystic mass. The lining of the cyst was focally hemorrhagic with bright yellow areas. Microscopic examination revealed the wall to be composed of smooth muscle lined by a variety of epithelia, including stratified squamous, stratified and pseudostratified ciliated columnar, and transitional (Figures 2 through 4). Histologic sections of the bright yellow areas of the cyst wall revealed denuded epithelium with extensive xanthogranulomatous reaction.What is your diagnosis?We present a case of a 37-year-old woman with a presacral mass that was causing obstruction of the birth canal during delivery of her baby. Computed tomographic scans showed a circumscribed, hypodense, cystic mass. A laparoscopic excision of the mass was performed. The cyst wall was composed of smooth muscle lined by a variety of epithelia, consistent with a tailgut cyst.Tailgut cysts are rare developmental cysts in the presacral space and have been described under different names such as cyst of postanal intestine, retrorectal cystic hamartoma, mucus-secreting cyst, enterogenous cyst, simple cyst, myoepithelial hamartoma of the rectum, and retrorectal cysts.12 They are believed to arise from the remnants of the embryonic hindgut. The embryo at the 3.5- to 8.0-mm stage (28–35 days) of development possesses a true tail and the primitive hindgut extends into this tail caudal to the site of subsequent formation of anus. This is called the tailgut or postanal gut. It normally atrophies by the eighth week. Persistence of this tailgut is believed to be the origin of the tailgut cyst. A second theory is that it develops from remnants of the neurenteric canal.2Fewer than 100 cases have been reported to date; the largest series of 53 cases was reported by Hjermstad and Helwig from the Armed Forces Institute of Pathology.2 Tailgut cysts are more common in women (female-to-male ratio, 3:1). Nearly half the patients are asymptomatic and the other half present with symptoms of mass effect or pain. The cysts may be unilocular or multilocular and range in size from 2 to 12 cm in greatest dimension. They are soft, well-circumscribed masses, limited to the presacral space, with no communication with the rectum. Cysts may be filled with clear to straw-colored thin, watery fluid or thick, mucoid fluid. A variety of epithelial linings may be seen including stratified squamous, cuboidal, transitional, stratified columnar, mucinous, or ciliated columnar. Interrupted bundles and wisps of smooth muscle are present, separated from the epithelium by a thin layer of fibrous tissue; however, there is no distinct nerve plexus.12 Inflammation, including xanthogranulomatous type, is present about half the time. Complications include infection, inflammation, recurrent perianal fistulae, and malignant transformation; adenocarcinoma and carcinoid are the 2 commonest malignancies reported.1 Complete surgical excision is the treatment of choice, which eliminates the problem of recurrent draining sinuses as well as the possibility of malignant change.The presacral (retrorectal) space is a potential space bounded anteriorly by the rectum, posteriorly by the sacrum, superiorly by the peritoneal reflection, and inferiorly by the levator ani and coccygeus muscle. Congenital lesions are the most common causes of tumors and cysts in this location; teratomas in children and chordomas in adults are the most frequent.3 The differential diagnosis of tailgut cysts includes rectal duplication cysts, cystic teratoma, and dermoid and epidermoid cysts. Rectal duplication cysts are rare with fewer than 70 cases reported in the literature.4 Duplication cysts are more common in the ileum. They are also known as enteric cyst, enterogenous cyst, or enterocystoma. They are thought to result from incomplete separation of notochord, defects of recanalization, caudal twinning, or persistence of epithelial buds. Three main characteristics that separate rectal duplication cyst from tailgut cyst are firm attachment to the gastrointestinal tract, well-developed smooth muscle layer with myenteric plexus, and a mucosal lining reflective of the gastrointestinal tract with muscularis mucosae, villi, and crypts. Presacral teratomas are rare and contain derivatives from all 3 germ layers of ectoderm (squamous epithelium, and skin appendages), endoderm (respiratory or gastrointestinal epithelium, and pancreas), and mesoderm (connective tissue, smooth muscle, cartilage, and bone). The incidence of malignancy in presacral teratomas is said to be 10% to 50% with a sharp increase if diagnosed after 2 months of age. Dermoid and epidermoid cysts are unilocular and lined by stratified squamous epithelium. Dermal appendages are present in dermoid cyst and absent in epidermoid cyst.
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