Digestive tract

Rare cancer of the digestive tract

Domain 5


The domain of rare digestive tract cancers gathers 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.

Peritoneal malignancies

A second subdomain of the rare digestive tumors domains of EURACAN illustrates the importance of surgical expert teams within reference centers to treat peritoneal cancers.

Pseudomyxoma peritonei

Pseudomyxoma peritonei (PMP) is a clinico-pathological entity characterized by a peritoneal mucin accumulation, originating from a perforated mucinous neoplasm (42). In 90% of cases the primary tumor is located in the appendix, while the remaining cases resulting from ovarian, colorectal or other abdominal organs with no differences in oncological outcomes.

The treatment is based on a cytoreductive surgery (CRS) with the aim of a complete removal of peritoneal lesions involving large peritonectomies and organ resections, often combined with hyperthermic intraperitoneal chemotherapy (HIPEC).

Primary peritoneal serous carcinoma

Primary peritoneal serous carcinoma (PPSC) is a rare condition which arises from the peritoneal epithelium. PPSC has histological and clinical similarity with advanced serous ovarian carcinoma. PPSC can occur many years after ovary removal surgery performed for benign diseases or prophylactic oophorectomy. The tumor appears during adulthood with a median age at diagnosis of 62 years.

The therapeutic strategy is combining CRS often followed by HIPEC in combination with systemic chemotherapy.

Peritoneal desmoplastic small round cell tumors

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive peritoneal tumor 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 massive tumor 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 rare disease which is characterized by the dissemination of multiple benign smooth muscle cell-containing nodules throughout 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

Biliary tract cancer (BTC), is a rare disease in Europe where its incidence is approximately 5 per 100.000 people per year. Its incidence is however variable and more frequent in other regions across the globe.

The majority of BTCs is located perihilar, followed by distal extrahepatic, gallbladder and intrahepatic varying in incidence of primary regionally. Treatment options are: resection in curatieve intent followed by adjuvant capecitabine (where available), systemic chemotherapy for advanced disease consisting of gemcitabine and cisplatin.

Future research focusing on identifying biomarkers to guide treatment choices, explore the role of neoadjuvant therapy, liver transplantation and arterial perfusion in selected cases is ongoing. There are promising early results of targeted and immunotherapy. The molecular subtypes of BTC are now better understood, leading to expected personalized treatment to be developed in the future. Given their particularly poor outcome, access to clinical research within expert center is essential to enable progresses in survival and cure rates.


Anal cancers

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