Rare digestive tract cancers gather a heterogeneous group of malignancies for which management issues and clinical presentations vary considerably. EURACAN is currently engaged in primary peritoneal malignancies, biliary tract cancer and anal cancers.
Rare cancer of the digestive tract
Peritoneal malignancies
The importance of surgical expert teams within reference centres to treatperitoneal cancers needs to be stressed.
Primary peritoneal serous carcinoma
Primaryperitoneal serous carcinoma (PPSC) is a rare condition which arises from theperitoneal epithelium. PPSC has histological and clinical similarity withadvanced serous ovarian carcinoma. PPSC can occur many years after ovaryremoval surgery performed for benign diseases or prophylactic oophorectomy. Thetumour appears during adulthood with a median age at diagnosis of 62 years.Thetherapeutic strategy is combining CRS often followed by HIPEC in combinationwith systemic chemotherapy.
Peritoneal desmoplastic small round cell tumours
Desmoplastic small round cell tumour (DSRCT) is a rare and aggressive peritoneal tumour affecting children, adolescents and young adults with an estimated incidence between 0.2 and 0.5 new cases per million per year (48). DSRCT usually presents with a massive tumour burden spread throughout the abdomen and beyond. Recent studies showed that a multimodality approach based on complete CRS in association with HIPEC and postoperative whole abdominal radiation is an effective local control therapy.
Diffuse peritoneal leiomyomatosis
Diffuse peritoneal leiomyomatosis (DPL) is a rare disease which is characterised by the dissemination of multiple benign smooth muscle cell-containing nodules through out the peritoneum, the omentum and the pelvic structures. DPL manifests during adulthood and is predominantly found in women. Malignant transformation is rare and only seen in a few cases. Depending on the extent of the disease, first-line treatment for DPL is surgical excision or CRS.
