PSEUDOMYXOMA PERITONEI: A RARE ENTITY
Bahar Canbay TorunDear Editor,
I write to bring an attention to a rare disease, pseudomyxoma peritonei (PMP), which was first described by Werth in 1884, yet it remains an orphan disease with limited awareness (1). The incidence of PMP is one to two patients/per million in a year that might be due to misdiagnosis (2).
PMP typically arises from a ruptured mucinous tumour, usually originating from the appendix, stomach, gall bladder, small and large intestine, fallopian tubes, ovaries, pancreas and lung, and mucin accumulates inside the peritoneal cavity and is called ‘jelly belly’ (1). PMP is histopathologically classified into four subtypes, based on present of tumour cells, the amount of mucin and the aggressiveness level, which are low grade, high grade, high grade with signet ring cells and acellular mucin (2).
