BACKGROUND: Perianal mucinous adenocarcinoma is a rare variant of anal canal epithelioid tumors. Our objective in this report is to examine the clinical features, pathology, treatment, and outcome for patients with perianal mucinous adenocarcinoma. METHODS: A retrospective review identified four patients with histologically proven perianal mucinous adenocarcinoma. The medical records of these patients were reviewed for presentation, therapy, and outcome. RESULTS: Pain and bleeding were present in all cases. In three of four patients, chronic perirectal disease was present, including two abscesses and one fistula. All patients had extensive local disease at presentation. One patient presented with bilateral inguinal nodal metastases. Two patients received neoadjuvant chemotherapy and radiation, with a third patient receiving radiation alone. Two of these three patients underwent abdominoperineal resection. Three patients subsequently died (all of progression and/or recurrence) 2-48 months after diagnosis. The fourth patient (who was treated with chemotherapy and radiation followed by abdominoperineal resection) is alive and disease free at 12 months. CONCLUSIONS: Perianal mucinous adenocarcinoma is a rare disease with a poor prognosis, mostly due to its advanced nature at the time of diagnosis. Chemoradiation followed by surgery may improve outcome in selected individuals.
BACKGROUND: Perianal mucinous adenocarcinoma is a rare variant of anal canal epithelioid tumors. Our objective in this report is to examine the clinical features, pathology, treatment, and outcome for patients with perianal mucinous adenocarcinoma. METHODS: A retrospective review identified four patients with histologically proven perianal mucinous adenocarcinoma. The medical records of these patients were reviewed for presentation, therapy, and outcome. RESULTS:Pain and bleeding were present in all cases. In three of four patients, chronic perirectal disease was present, including two abscesses and one fistula. All patients had extensive local disease at presentation. One patient presented with bilateral inguinal nodal metastases. Two patients received neoadjuvant chemotherapy and radiation, with a third patient receiving radiation alone. Two of these three patients underwent abdominoperineal resection. Three patients subsequently died (all of progression and/or recurrence) 2-48 months after diagnosis. The fourth patient (who was treated with chemotherapy and radiation followed by abdominoperineal resection) is alive and disease free at 12 months. CONCLUSIONS: Perianal mucinous adenocarcinoma is a rare disease with a poor prognosis, mostly due to its advanced nature at the time of diagnosis. Chemoradiation followed by surgery may improve outcome in selected individuals.
Authors: Natalia Mukai; Lílian Vital Pinheiro; Maria de Lourdes Setsuko Ayrizono; Guilherme Cardinali Barreiro; Paulo Kharmandayan; Mariana Hanayo Akinaga; Adriano Mesquita Bento; Carlos Augusto Real Martinez; Rita Barbosa de Carvalho; Marc Ward; Cláudio Saddy Rodrigues Coy; Raquel Franco Leal Journal: Int J Surg Case Rep Date: 2016-07-07