Tailgut Cyst Adenocarcinoma in a Young Female

Abstract

ABSTRACT Tailgut cysts (TGCs) are rare congenital anomalies which arises when TGC fail to regress on the 56th day of normal embryological development. TGC present as a space-occupying lesion in the presacral space. The diagnosis is challenging because of its rarity. Malignant transformation of TGC can occur rarely. In literature <30 cases of adenocarcinoma are reported as of now. Here, we present the case of TGC adenocarcinoma with a fistulous tract formation with vagina. A 23-year-old female presented with complaints of abdominal pain associated with fever and vomiting initially. Contrast-enhanced computed tomography of the abdomen shows thick-walled hypodense lesion in midline displacing rectum. Marsupialization of cyst done at the outside hospital. The patient presented to us with complaints of per vaginal discharge and lower abdominal pain 6 months postsurgery. On evaluation with magnetic resonance imaging (MRI) abdomen, it shows complex cystic lesion in the presacral space. Excision of mass done. Histopathological evaluation shows moderately differentiated adenocarcinoma arising from hindgut cyst. The patient presented with recurrent disease near cervix 3 months postsurgery with complaints of abdominal pain. The patient was planned for chemotherapy but was lost to follow up. TGC present as a space-occupying lesion in the presacral space. There are a wide variety of benign and malignant conditions developed in this space. The clinical features include urinary obstruction, obstipation, sciatica, low back ache, and rectal bleed. Malignant transformation of TGC is very rare. The most common type of malignancies is adenocarcinoma and carcinoid tumor. There are <30 cases of TGC adenocarcinoma reported in the literature. MRI is the gold standard in the diagnosis. Biopsy is controversial due to the fear of tumor seeding. Surgery is the definitive management in TGC. In benign lesions, only surgery has lifelong expectancy. Adjuvant radiotherapy ± chemotherapy has been tried with good outcome in some malignant cases. TGCs are rare congenital anomalies which usually get missed on the diagnosis. Benign lesions have good outcomes only with surgical resection. There are risks of malignant transformation; hence, early surgical resection should be done. Malignant lesions have poor outcomes. Adjuvant radiotherapy ± chemotherapy has been tried with good outcomes.