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

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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.
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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.

Biliary Tract Cancer
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Biliary tract cancers (BTC) are a group of rare but aggressive cancers that develop in the biliary system, which includes the bile ducts, gallbladder, and other related structures. These cancers are often difficult to diagnose in their early stages, which can lead to poor prognosis if not detected promptly.
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There are several anatomical subtypes of biliary tract cancer:

Gallbladder Cancer: This is the most common type of BTC and occurs in the gallbladder, the organ that stores bile. It is often diagnosed late due to its subtle early symptoms.
Intrahepatic Cholangiocarcinoma: This type of cancer originates in the bile ducts within the liver. It is typically harder to detect because it often has no symptoms until it reaches advanced stages.
Extrahepatic Cholangiocarcinoma: Cancer that affects the bile ducts outside the liver. It may be localized in the hilum (near where the bile ducts enter the liver) or more distal in the bile ducts.
Ampullary Cancer: This rare cancer develops at the ampulla of Vater, where the bile duct and pancreatic duct meet, leading to symptoms related to bile flow or pancreatic function.

The treatment of biliary tract cancers depends on the location and stage of the disease, with surgery being the most effective treatment when feasible. Recently, advances in immunotherapy and targeted treatments have offered new hope, improving survival rates and quality of life for patients with advanced BTC.