From the Guidelines
Pancreatic mucinous cyst adenocarcinoma is a rare but important subset of pancreatic cancers, predominantly affecting women with a female-to-male ratio of approximately 9:1, and typically occurring in middle-aged individuals between 40-50 years of age 1.
Epidemiology
The epidemiology of pancreatic mucinous cyst adenocarcinoma is characterized by:
- A strong female predominance, with a female-to-male ratio of approximately 9:1 1
- A typical age range of 40-50 years at primary diagnosis 1
- A location in the pancreatic body or tail 1
- A risk of malignant transformation that increases with age and cyst size 1
Risk Factors
Risk factors for pancreatic mucinous cyst adenocarcinoma include:
- Female sex 1
- Age over 40 years 1
- Cyst size larger than 4 cm 1
- Presence of mural nodules, thickened or enhanced cyst walls, and rapid growth of the cyst 1
Diagnosis and Treatment
Diagnosis of pancreatic mucinous cyst adenocarcinoma is typically made by imaging studies such as CT or MRI, and conventional ultrasound or EUS 1. Early detection and surgical resection of MCNs before malignant transformation occurs is associated with excellent long-term survival rates approaching 100% 1. However, the prognosis for invasive mucinous cystadenocarcinoma is significantly worse, though still better than conventional pancreatic ductal adenocarcinoma 1.
Outcome
The overall incidence of invasive carcinoma arising in MCNs ranges from 12-30% in surgical series 1. Morbidity, mortality, and quality of life outcomes are significantly improved with early detection and surgical resection of MCNs 1.
From the Research
Epidemiology of Pancreatic Mucinous Cyst Adenocarcinoma
- Pancreatic mucinous cystic neoplasms (MCNs) occur almost exclusively in women, with a female-to-male ratio of 20:1 2
- MCNs are mainly located in the pancreatic body or tail (93-95%) and are usually found incidentally at the age of 40-60 years 2
- The majority of patients with MCNs are women (71.4%), with a median age of 65 years 3
- Men with MCN-associated adenocarcinoma/high-grade dysplasia have a higher frequency of lymph node-positive disease compared to women (57% vs. 22%) 4
Demographics and Clinical Characteristics
- The median age of patients with mucinous cystadenocarcinoma (MCC) is higher than those with mucinous cystadenoma (MCA) 5
- Tumor size, age, and serum levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, and CA12-5 may contribute to the management of patients with MCN 5
- Patients with non-invasive (in situ) disease have prolonged survival compared to those with invasive disease (median OS not reached vs. 50.2 months) 3
Survival and Prognosis
- Five-year survival after surgical resection of a malignant MCN is approximately 60% 2
- Patients who undergo pancreatectomy have better survival compared to those who do not undergo surgery (81.4 vs. 6.6 months) 3
- Nodal positive disease is the most important factor of decreased survival for invasive adenocarcinoma (HR: 2.2) 3
- Patients with adenocarcinoma arising from a mucinous cystic neoplasm of the pancreas have excellent survival when they undergo pancreatectomy, especially if the disease is still in situ 3