[Retrorectal cystic hamartomas. Report of one case]

Retrorectal cystic hamartomas (RCH) are rare congenital lesions of the presacral space, of which 68 cases are reported under different terms. Clinicopathologic features are usually constant and similar to the present case. A 23-year-old woman complained of abdominal and perineal pains for several months. Physical examination revealed a nodular mass in the posterior part of the rectum. A pelvic MRI showed a 5.5 cm cystic retrorectal mass compressing the rectum. The patient underwent surgical resection. Pathologic examination found an ill-defined nodular mass, composed by numerous cysts surrounded by fibroadipose tissue. Cysts were lined by different epithelia: keratinized and non keratinized squamous, transitional, ciliated and mucus-producing columnar epithelia. Few mucinous glands were noted, connected to some cysts. These epithelial structures were surrounded by connective tissue in which well-differentiated bundles of smooth muscle fibers were present without well-formed muscularis. The RCH differential diagnosis includes principally congenital cysts: epidermal cysts, cystic teratomas, dermoid cysts, anal gland cysts and rectal duplications. An embryologic origin of RCH from remnants of the postanal gut is currently accepted. Loco-regional inflammatory process frequently complicates this lesion and can cause perirectal fistulae. RCH also possesses a malignancy potential, with development of adenocarcinomas. To avoid these complications, complete excision is recommended.