Abstract
INTRODUCTION AND IMPORTANCE: Tailgut cysts (TGC) are rare retrorectal lesions originating from embryological remnants of the hindgut. Malignant transition is rare; treating it with an unplanned diagnostic algorithm can complicate the clinical course. CASE PRESENTATION: We report a case of retrorectal mucinous adenocarcinoma arising from a TGC in a 63-year-old female. She had experienced constipation, recurrent urinary tract infections, and difficulty voiding for three months. A total hysterectomy was performed elsewhere for a presumed leiomyoma. A transrectal biopsy was negative, but partial cystectomy was attempted. Histology showed a benign lesion. Postoperatively, she developed pelvic sepsis and urinary/fecal incontinence and was referred to our clinic. MRI revealed a 16 × 12 cm peri-coccygeal cyst compressing the rectum and bladder. The cyst, rectum, and coccyx were resected en bloc. Pathology showed mucinous adenocarcinoma arising from TGC with positive radial margins. Adjuvant chemoradiotherapy was recommended. CLINICAL DISCUSSION: This case highlights diagnostic and management challenges in retrorectal lesions. Inadequate interventions such as transrectal biopsy or partial resection may result in sepsis and tumor spillage. A multidisciplinary and experienced team is essential for optimal outcomes. CONCLUSION: Management of retrorectal lesions should be tailored by a multidisciplinary team. The index surgical approach determines the ultimate outcome. Transrectal biopsies and partial resections should be avoided due to the high risk of pelvic sepsis and tumor dissemination.