Abstract
Pseudomyxoma peritonei (pmp) is a rare clinical condition defined as extensive intraperitoneal spread of mucus associated with a variety of mucinous tumours of varying biologic behavior. Although appendix or ovaries have usually been implicated as the primary site, cases have been reported in association with neoplastic lesions of other sites. Pseudomyxoma peritonei originating from urachal remnants is a unique entity, reported only 18 times in the English literature thus far. Considering the rarity of the lesion, we report the case of a 50-year-old man surgically treated for pmp associated with a low-grade mucinous urachal neoplasm. Unique aspects of case are the low histologic aggressiveness of the causative lesion (reported only twice worldwide) and the early stage of the disease, with a relatively small amount of intraperitoneal free mucin. Review of the literature about pmp in general and a collation of previously reported cases of pmp originating from the urachus are presented and discussed.
Highlights
Pseudomyxoma peritonei is a rare clinical condition defined as extensive intraperitoneal spread of mucus associated with variety of mucinous tumours of varying biologic behavior[1,2]
Low incidence forecloses the possibility of large-scale studies, and as a result there is broad diversity in the definitions, pathology, site-of-origin theories, treatment protocols, and prognosis for this disease[5]
At the time of article submission, our patient had been referred to long-term follow-up, but considering the low histologic aggressiveness, the macroscopically complete cytoreduction, and the microscopically negative margins, no adjuvant chemotherapy was administered
Summary
Pseudomyxoma peritonei (pmp) is a rare clinical condition defined as extensive intraperitoneal spread of mucus associated with variety of mucinous tumours of varying biologic behavior[1,2]. Distinctive for pmp is a slow but relentless accumulation of abundant gelatinous fluid within the peritoneal cavity Within this mucinous material, neoplastic cells may be bland and very scanty[1,5]. The appendix has been implicated as the primary site in most cases, incidences have been reported in association with neoplastic lesions of other sites[1,4,5], including ovary[2,8,9], fallopian tube[10], endocervix[11], colorectum[11], retained colorectal tissue dislocated after previous surgical interventions[2,12], small bowel[11], stomach[13], gallbladder[6], lung[14], breast[15], pancreas[16,17,18], mucinous cysts of spleen[19,20], and extraperitoneal tumours[21,22]. Tumour growth usually tends to remain confined to the abdomen for many years, and blood-borne and lymph node metastases are uncommon, the disease has the potential for aggressive clinical behavior, including local tumour recurrence, and is always lethal unless radically treated[1,5,42,43,47,48]
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