Perianal Mucinous Adenocarcinoma: A Case Report and a Systematic Review of the Literature.
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BACKGROUND: Mucinous adenocarcinoma of anus is an uncommon neoplasm of the gastrointestinal tract. In most of the cases, early diagnosis of this disease is difficult since its symptoms frequently mimic benign inflammatory conditions. METHODS: A systematic PubMed and Scopus search was conducted, a propos of a case report. RESULTS: One hundred fifty patients from 93 case reports were included. The mean age of the patients was 60.5 years (range: 18-81). The majority of them were males (124 out of 150, 82.7%), while the main known risk factor was the history of chronic fistula (109 out of 150, 72.7%). Recurrent perianal sepsis and perianal pain were the principal symptoms at the time of presentation. No symptoms have been reported only in three patients (3 out of 150, 2%). Regarding the prior surgical history of the patients, multiple abscess drainage and perianal fistula's related interventions were present in 62 (41.3%) and 19 (12.7%) patients, respectively. Neoadjuvant chemoradiotherapy was administered in 53 out of 150 patients (35.3%), while the majority of them have been treated with combined treatment of chemotherapy and radiotherapy. APR and its variations were the most applied surgical treatment (68%). Adjuvant chemoradiotherapy was administered almost up to one-third of the included patients (34%). Recurrence of the disease was reported in 41 out of 150 patients (27.3%). Death was reported in 44 out of 150 patients (29.3%). CONCLUSION: Review of the available published literature suggests that perianal mucinous adenocarcinoma is an extremely rare neoplasia. Τhere is no consensus as far as the diagnosis and the treatment strategies, due to the rarity of this neoplasm. High degree of clinical suspicion as well as histopathological confirmation is the principal requisites for the diagnosis of mucinous adenocarcinoma, especially in the ground of chronic ulcero-proliferative perianal lesions.