Pseudomyxoma peritonei secondary to epithelial appendicular neoplasms. Experience in a non-specialised centre.
BACKGROUND
Pseudomyxoma peritonei is an infrequent entity, defined by collections of gelatinous material in the abdomen and pelvis and mucinous implants on peritoneum, secondary to the rupture of a mucinous lesion, usually of ovarian or appendiceal origin.


METHODS
We present our experience of 11 cases (6 males and 5 females) diagnosed with pseudomyxoma peritonei secondary to epithelial appendicular neoplasms over 27 years. The mean age of the patients was 68 years. Clinical manifestations were abdominal distension (55%), right lower quadrant pain (45%) suggesting acute appendicitis and constitutional syndrome (36%). An abdominal mass was detected at physical examination in 4 patients. CT scan revealed a tumour in right iliac fossa in 4 patients, peritoneal enlargement in 1 and a liquid collection in 1. Preoperative diagnosis was acute abdomen in 5 patients, peritoneal carcinomatosis in 3 and undetermined abdominal mass in 3.


RESULTS
Surgical findings suggested pseudomyxoma peritonei in 8 patients and peritoneal carcinomatosis in 3. Appendicectomy was performed in 9 patients, and in 3 of them bilateral anexectomy was also performed. One patient underwent ileocaecal resection and another a right hemicolectomy. In all the cases, mucinous material was eliminated as much as possible. Pathology revealed mucinous cystoadenoma in 6 cases, mucinous cystoadenocarcinoma in 3 and epithelial hyperplasia in 2 patients. Median survival was 54 months, with a 5- year survival rate of 40%. The last case we treated was sent to a reference centre for the treatment of pseudomyxoma peritonei.


CONCLUSIONS
There is no consensus on the best treatment for pseudomyxoma peritonei. We recommend avoiding incomplete surgical resections in non-reference centres and submitting patients to a reference centre to undergo adequate treatment.
Department of Surgery, University Hospital Ramón y Cajal, Madrid, Spain. jruiztovar@gmail.com
J
J
Ruiz-Tovar
V
V
Morales Castiñeiras
D
D
García Teruel
A
A
Sanjuanbenito Dehesa
E
E
Lobo Martínez
E
E
Martínez Molina
D000328:Adult; D000368:Aged; D001062:Appendectomy; D001063:Appendiceal Neoplasms / Q000473:pathology* / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D018282:Cystadenocarcinoma, Mucinous / Q000473:pathology* / Q000601:surgery; D018291:Cystadenoma, Mucinous / Q000473:pathology* / Q000601:surgery; D004847:Epithelial Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010534:Peritoneal Neoplasms / Q000473:pathology*; D011553:Pseudomyxoma Peritonei / Q000473:pathology*; D015996:Survival Rate