While pancreatic adenocarcinoma is the most common type of pancreatic cancer, there are several rarer tumours that can also develop in the pancreas. The distinction between the different types of tumours is critical as it has important implications for treatment and prognosis. These tumours can be benign or malignant and have specific diagnostic and treatment pathways. This is an overview of some rarer types of pancreatic tumours, their characteristics, and potential treatment options.
Types of Rarer Pancreatic Tumours
Solid Pseudopapillary Neoplasm (SPN)
This rare tumor primarily affects young women and has a relatively favourable prognosis. SPNs are typically slow-growing and can be surgically removed with a good chance of long-term survival.
Acinar Cell Carcinoma (ACC)
ACC starts in the exocrine cells of the pancreas and is quite rare. It can be more aggressive than other types of pancreatic cancer and may require a combination of surgery, chemotherapy, and radiation therapy.
Pancreatoblastoma
This tumour is most commonly found in children and tends to grow slowly. Treatment usually involves surgical removal, and the prognosis is generally good if the tumour is detected early.
Pancreatic Neuroendocrine Tumours (PNETs)
These tumours arise from the endocrine cells of the pancreas and can be either benign or malignant. PNETs can produce hormones that cause various symptoms and may require surgical removal, chemotherapy, or targeted therapies. Symptoms of NETs vary depending on their location and whether they produce hormones. Common symptoms include abdominal pain, diarrhoea, flushing, bloating, wheezing, chronic cough, heartburn, and unexplained weight changes. Diagnosis often involves imaging techniques such as CT scans, MRI, and endoscopic ultrasound, along with biopsies to confirm the nature of the tumour. Treatment options for NETs depend on the type, location, and aggressiveness of the tumour. Surgery is often the primary treatment, especially for localized tumours. Chemotherapy, radiation therapy, and targeted therapies may be used for more advanced or aggressive tumours.
Intraductal Papillary Mucinous Neoplasm (IPMN)
IPMNs are precancerous cysts that can develop into invasive cancer if left untreated. Most IPMNs can be watched with regular imaging. A small subset, however, can show concerning features on imaging or grow rapidly and cause symptoms. These can require surgical removal to prevent progression to cancer.
Serous Cystadenoma
This is a type of benign cystic tumour filled with fluid. Most serous cystadenomas do not become cancerous, but they may need to be monitored for changes.
Mucinous Cystic Neoplasms (MCNs)
MCNs are a type of cystic lesion that occurs in the pancreas. These lesions are more common in women aged 40-60 and are characterized by a mucin-producing epithelial lining surrounded by ovarian-like stroma. While MCNs are benign, they have a high rate of progression to cancer if left untreated. Diagnosis of MCNs typically involves imaging techniques such as CT scans and MRI, and sometimes endoscopic ultrasound with fine needle aspiration (EUS-FNA) to obtain cyst fluid for analysis. Surgical resection is the preferred treatment when feasible- especially for larger lesions-, as it can prevent the progression to invasive cancer.
Diagnosis and Treatment
Diagnosing rarer pancreatic tumours can be challenging due to their uncommon nature and overlapping features with other types of pancreatic lesions. Imaging techniques such as CT scans, MRI, and endoscopic ultrasound are often used to identify these tumours. Biopsies may be necessary to confirm the diagnosis and determine the tumour’s nature.
Treatment options for rarer pancreatic tumours vary depending on the type and stage of the tumour. Surgery is often the primary treatment, especially for benign or localized tumours. Chemotherapy, radiation therapy, and targeted therapies may be used for malignant tumours or those that have spread.
Prognosis and Follow-Up
The prognosis for patients with rarer pancreatic tumours depends on the type of tumour, its stage at diagnosis, and the patient’s overall health. Early detection and appropriate treatment can significantly improve outcomes. Regular follow-up and monitoring are essential to detect any recurrence or progression of the disease.
Conclusion
Rarer tumours of the pancreas, though uncommon, require careful attention and management. Understanding the different types, their characteristics, and treatment options can help patients and healthcare providers make informed decisions about care. If you or someone you know is diagnosed with a rare pancreatic tumour, it’s important to consult with a specialist who can provide personalized treatment and support.