Pancreatic tumours
Key points
Classification of pancreatic tumours
Exocrine Pancreatic Tumours
Benign:
Serous cystadenoma
Mucinous cystadenoma
Mature cystic teratoma
Borderline Malignant:
Solid pseudopapillary tumour of the pancreas (Franz tumour)
Malignant:
Pancreatoblastoma
Mucinous cystadenocarcinoma
Acinar cell carcinoma
Pancreatic ductal adenocarcinoma
Endocrine Pancreatic Tumours
Benign:
Insulinoma
Sporadic gastrinoma
Borderline/Malignant:
Multiple Endocrine Neoplasia (MEN) associated insulinoma and gastrinoma
Solid pseudopapillary tumour
Slow growing
1/3 in head 2/3 tail/body
Predominantly affects women of childbearing age.
Generally has a low risk of malignancy - Metastases in <10%
Imaging: mix of cystic and solid components, tumour being encapsulated with some internal haemorrhages
Pancreatoblastoma
Most common in young children mean 4.5y
50% of cases are in Asian populations
Beckwith-Wiedemann Syndrome (BWS) + Familial Adenomatous Polyposis (FAP) association
Slow growing - large mass
aFP raised in 40-70%
Insulinoma
Inappropriately high serum insulin
Severe hypoglycaemia
More likely to be malignant if associated with MEN syndrome
Difficult to visualise lesion
18 F-DOPA PET/CT if CT/MRI not helpful
Operation:
Resect lesion
Partial or full pancreatectomy if very large or malignant
Gastrinoma
Hypersecretion of gastrin
Zollinger-Ellison syndrome - triad of Severe Peptic Ulceration, Gastric Hypersecretion/GORD, Diarrhoea
60% of gastrinomas occur outside the pancreas within the duodenum and within the 'gastrinoma triangle'
Gastrinoma is the most common islet cell tumour seen in MEN1 (seen in up to 50% of patients)
60-90% are malignant - with lymph node and liver metastases
Investigations:
Serum gastrin levels - will be elevated
Then secretin stimulation test - if gastrin levels pardoxically increase - gastrinoma is likely
Somatostatin receptor scintigraphy
Management:
High dose PPI
Chemotherapy
Resect lesion only if >2cm, as MEN1 associated gastrinoma has a low cure rate
Standard scenario
Child with pancreatic mass
Concerns:
1. Malignancy
2. Local pressure effects and obstructive jaundice, ascending cholangitis
3. Endocrine effects
History:
Duration of mass
Obstructive jaundice symptoms
Hypo/hyperglycaemia
Weight changes
Gastric ulcers
Family history of tumour syndromes e.g. MEN1
Examination:
Mass
Systemic for jaundice
Cachexia
Investigations:
Glucose
aFP + HCG
Gut hormone profile
CT + MRI
Discuss at HPB MDT - transfer to HPB centre
Staging
Decision for biopsy
Differentials:
Benign - serous cystadenoma, mucinous cystadenoma, mature cystic teratoma
Malignant - pancreatoblastoma, solid pseudopapillary tumour (Franz tumour), mucinous cystadenocarcinoma
Benign - Insulinoma + gastrinoma - likely malignant if MEN1
Procedure:
Tail/body - spleen sparing resection
Head - Pancreatoduodenectomy (Whipples)
References
Cofer, Barry R. "Pancreatic Tumors." Pediatric Surgery NaT, American Pediatric Surgical Association, 2020. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829508/all/Pancreatic_Tumors.
Cho MS, Kasi A. Zollinger-Ellison Syndrome. [Updated 2022 Nov 21]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537344/