XXXX : retrospective analysis of management and outcome in a single Portuguese Institution.

@entity1 @entity548 @entity2699 @entity5 @entity158 @entity3 @entity320
@entity320 is an uncommon @entity5 with a poor prognosis. We aim to describe the clinical presentation, treatment and outcome of @entity1 with @entity320 in our center. Retrospective study of @entity1 with  @entity320  treated between 2000 and 2011 at a @entity5 center in Lisbon. Ten @entity1 were identified, eight females and two males, with median age 70.5  years (32-79). Symptoms at presentation were @entity548 (8), anal @entity158 (4) and discomfort (3). @entity5 location was anal (6), rectal (3) and anorectal transition (2). Seven @entity1 had surgery: abdomino-pelvic resection (5) and local resection (2). Among the two @entity1 who underwent local resection, one was an incidental finding in a hemorrhoidectomy specimen. This @entity1 had further adjuvant chemotherapy ( @entity2699 ). Three @entity1 had distant @entity3 at diagnosis, one had chemotherapy and two had best supportive care. Six of the seven  operated @entity1 relapsed in a median time of 5.4  months: distant @entity3 (4), local recurrence (1), both (1). The two local relapse @entity1 had surgical widening of resection margins (1) and radiotherapy (2). One-year survival was 30  %; 3-year survival was 20  %. @entity320 has a poor prognosis due to advanced disease at presentation and aggressive course, with relapse in almost all operated @entity1 . Treatment guidelines have not been established due to the lack of randomized studies. However, recent studies show that sphincter-sparing surgical procedures along with low dose intensity radiotherapy seem to achieve a local control similar to abdomino-pelvic resection. No systemic therapy is considered standard of care for advanced disease, and regimens are extrapolated from cutaneous @entity320 experience.
