pseudomyxoma peritonei ( pmp ) is an extremely rare disease , with an annual incidence of approximately one case per million . although such tumours are typically slow - growing , they vary highly in their invasive and metastatic capacity . due to the heterogenous nature of pmp , proposed dividing pmp into 3 categories : disseminated peritoneal adenomucinosis , peritoneal mucinous carcinomatosis ( pmca )  the most aggressive subtype  and an intermediate group with features of both subtypes . in contrast , other authors such as misdraji   have suggested classifying the pathology into low- and high - grade disease . the optimal treatment of pmp is unclear ; however , the most common approach is surgical debulking with intraperitoneal chemotherapy during or immediately after surgery . in certain specialized centres , cytoreductive surgery with hyperthermic intraperitoneal chemotherapy is the treatment of choice . as the disease progresses , mucinous ascites accumulate in the peritoneum , eventually leading to gastrointestinal obstruction . because this bowel obstruction is frequently inoperable , the main aim of treatment is to relieve the associated symptoms ( primarily pain , nausea , and vomiting ) . somatostatin analogues , such as lanreotide and octreotide , are also commonly used due to their antisecretory ac - tivity . here we describe a patient with appendiceal pmca and peritoneal dissemination who responded well to symptomatic treatment with lanreotide , with radiological evidence demonstrating a notable reduction in peritoneal ascites . in july 2011 , a 54-year - old female patient was referred to the gynaecology department for the evaluation of a suprapubic mass . complementary tests performed during the diagnosis revealed the presence of several tumour markers [ carbohydrate antigen 199 ( ca 199 ) : 45 u / ml ; carcinoembryonic antigen ( cea ) : 2 ng / ml ; carbohydrate antigen 125 ( ca-125 ) : 56 u / ml ] . an abdominal ultrasound revealed a mass of 10 cm located on the anterior wall of the uterus , with scant free liquid in the peritoneal cavity . the patient underwent an abdominal laparotomy in august 2011 , revealing a 12-cm left adnexal mass with a stony consistency , identified as a signet ring cell carcinoma on intraoperative biopsy . a 4-cm tumour located on the right ovary a hysterectomy and double adenexectomy were performed , and the exploration of the abdominal cavity revealed a hardened appendix , with miliary seeding of the peritoneum and the diaphragmatic cupola . given these findings , an appendicectomy and omentectomy were performed and multiple biopsies of the peritoneum were taken . r2 surgery was then performed , although the cytoreductive surgery was deemed incomplete due to the persistence of the macroscopic disease ( > 2.5 cm ) . a pathological examination of the surgical specimen revealed a signet ring cell adenocarcinoma in the appendix , with metastases to the ovaries , fallopian tubes , omentum , and the left and right paracolic gutters . the patient was not considered a candidate for hyperthermic intraperitoneal chemotherapy and was therefore referred to the medical oncology department in september 2011 for further evaluation and treatment . first - line palliative chemotherapy was initiated according to the following scheme : capecitabine 2,000 mg / m / day for 14 days + oxaliplatin 130 mg / m ( 6 ) for 4 cycles until january 2012 , at which time second - line chemotherapy was initiated ; day 1 , 5-fu 400 mg / m + lv 400 mg / m + irinotecan 180 mg / m + 5-fu 2,400 mg / m in continuous perfusion for 46 h due to abdominal progression ( tense ascites ) and elevated serum tumour markers ( ca 199 : 104 u / ml ) . the patient received a total of 12 cycles of chemotherapy until june 2012 when treatment was halted due to unacceptable toxicity ( diarrhoea and neutropaenia , both grade 3 ) . after the patient recovered from the chemotherapy - induced toxicity , bimonthly follow - ups in the outpatient clinic were initiated . her general health status remained good , without any notable digestive symptoms and with an excellent functional status . however , in january 2013 , the patient developed bowel obstruction secondary to tumour progression and was admitted to the hospital . an abdominal ct scan showed a peritoneal invasion , with a hypodense region surrounding both the liver and the spleen , and marked duodenal dilatation up to the ligament of treitz . symptomatic treatment , including parenteral nutrition , corticosteroid therapy , antiemetics , and nasogastric intubation , was prescribed , with a good clinical response . the general surgery department ( peritoneal carcinomatosis unit ) evaluated the patient for possible palliative surgery , but the case was considered unsuitable for surgery . symptom control during hospitalization was good and the nasogastric tube was removed due to a lack of vomiting episodes . the patient maintained adequate kidney , hematologic , and liver function , and she was discharged from the hospital to home care , which included continued parenteral nutrition , supportive care , and monthly follow - ups at the medical oncology outpatient department . from march to november 2013 , the patient presented a sufficient performance status [ eastern cooperative oncology group ( ecog ) grade 1 ] with no abdominal pain or bilious vomiting . no radiological changes were observed on any images taken during outpatient follow - up . the only change of note was an increase in serum levels of ca 199 ( 330 u / ml ) . in january 2014 however , no worsening of other symptoms was observed , and kidney , liver , and hematologic functions remained adequate . considering the neuroendocrine differentiation of the primary tumour in the context of the patient 's increased vomiting frequency , we decided to initiate treatment with lanreotide autogel ( somatuline autogel ; ipsen ) , a long - lasting extended - release gel formulation of lanreotide , at a dose of 120 mg every 28 days . a phase iii trial published in 2012 suggested that this drug might be effective in the symptomatic treatment of inoperable bowel obstruction due to peritoneal carcinomatosis . our decision was further bolstered by a review published in that same year suggesting that somatostatin analogues may provide both direct and indirect antitumour effects in neuroendocrine tumours . an abdominal ct scan performed after treatment with lanreotide autogel showed a clear tumour response , with a notable decrease in ascites ( fig . 1 ) . in addition , vomiting frequency was reduced , and serum ca 199 decreased from 330 to 230 u / ml . for several months , the patient continued treatment with lanreotide autogel , home parenteral nutrition ( without oral ingestion of food ) , and monthly check - ups . however , in november 2014 , the patient was hospitalized due to acute respiratory failure in the context of a lower airway infection . a few days after admission , the patient died ( on november 11 , 2014 ) due to multiorgan failure ( despite the use of antibiotics , oxygen therapy , and non - invasive ventilatory support ) . in the case report presented here , the use of lanreotide autogel reduced disease - related symptoms , and , interestingly , also reduced the number of peritoneal ascites . these are important findings for patients with pmp given the highly negative impact of pmp - associated symptoms on quality of life and the poor long - term survival outcomes , especially in aggressive histological subtypes such as pcma . the benefits of short - acting somatostatin analogues ( e.g. , octreotide ) for the symptomatic treatment in inoperable bowel obstruction are well documented [ 6 , 7 , 8 ] . somatostatin analogues inhibit the release of various gastrointestinal hormones , thus reducing gastrointestinal secretions and slowing intestinal motility , and also increasing water and electrolyte absorption . lanreotide microparticles , a synthetic somatostatin analogue , has also been proven efficacious for this indication . recently , the same formulation of lanreotide ( lanreotide autogel ) that we used to treat the symptoms of bowel obstruction in our patient was reported to be efficacious in another case . the authors of that case report highlighted an important advantage of long - acting lanreotide over octreotide : less frequent administration ( three times / day for short - acting somatostatin analogues vs. once monthly administration for lanreotide autogel ) . in addition to the beneficial effects of somatostatin analogues on symptom control in secretory neuroendocrine tumours , several recent publications suggest that these drugs may also have direct and indirect antitumour effects . a systematic review published in 2012 evaluated publications that supported an antitumour role for the somatostatin analogues octreotide and lanreotide . according to that review , the published data support an antitumour effect for these drugs . more recently , results were published from the clarinet trial , a randomized , double - blind , placebo - controlled multinational study of lanreotide autogel in patients with advanced , well- or moderately differentiated , grade 1 or 2 somatostatin receptor - positive neuroendocrine tumours . patients randomized to receive somatuline autogel had a significantly longer progression - free survival , a finding that provides evidence for the antiproliferative effects of lanreotide . in this case report , the use of lanreotide autogel reduced disease - related symptoms and peritoneal ascites in a patient presenting with pmp . randomized studies are needed to further investigate the antiproliferative effects of lanreotide observed in this patient , who had a neoplasm with neuroendocrine differentiation and a negative octreoscan . the patient 's data have been completely anonymized and , therefore , no informed consent was considered necessary .