Mature cystic teratomas of the pancreas are extremely rare tumours encountered in day-to-day clinical practice. Only few cases have been reported to date involving all age groups. The management, diagnosis and evaluation of this tumor are questionable, with definitive diagnosis taking place intra-operatively. We hereby report the case in a 30 year-old-male who presented with newly diagnosed diabetes mellitus and during the follow up he was noted to have elevated liver enzymes clinically, he was asymptomatic. The computerized tomography revealed a retropancreatic mass and pushing the mesenteric veins anteriorly. The mass was hypodense in nature and there was presence of calcification. Although the patient was asymptomatic, the decision for resecting the mass was made in view of the size and possibility of malignancy. In conclusion, considering the size and approximity of the mass to the pancreas, Whipple procedure's is the most appropriate approach although the histological diagnosis has not been established preoperatively.
Solid-pseudopapillary tumour (SPT) is a rare exocrine tumour of the pancreas and is considered to have low malignant potential. Few morphological criteria are used to predict malignant behaviour such as equivocal perineural invasion, angioinvasion and invasion to surrounding tissue, and should be designated as solid-pseudopapillary carcinoma (SPC). We report a case of SPC. Clinical and radiological findings are typical for SPT with no metastatic disease. There is no tumour recurrence after 4 months postresection. Clinical history and radiological findings were retrieved from the patient's record sheet and Viarad system. H&E staining and few immunoproxidase staining were reviewed by several pathologists. The histological findings are typical for SPT, with additional perineural invasion. There is no angioinvasion or capsular invasion identified. This is our first experience in diagnosing and managing SPC. We look forward to seeing the patient's disease status during her next routine follow-up. We expect good disease-free survival and very low risk of tumour recurrence, in view of only one risk factor (perineural invasion) and uninvolved surgical margins by the tumour.
Patients with pancreatic adenocarcinoma are known to have a high mortality rate. The 5-year survival rate still remains low even now compared to that of the 1960's despite new advances in management including surgery, chemotherapy, pathological classification and molecular diagnostic technologies. Precursors to invasive pancreatic adenocarcinoma have been identified in the last ten years that include mucinous cystic neoplasm, intraductal papillary mucinous neoplasm and pancreatic intraepithelial neoplasia. p53 protein accumulation in the nuclei is a common molecular event in most human neoplasms. Our objective is to investigate p53 expression in pancreatic adenocarcinoma and precursor lesions and their significance. The selected study material encompassed 31 invasive ductal adenocarcinoma, 15 mucinous cystic neoplasm and papillary mucinous neoplasm, and 27 cases of pancreatic intraepithelial neoplasia including grade 1, 2 and 3. Immunoscore was given for each case based on intensity of staining and percentage of cells positive and compared between precursor lesions and invasive adenocarcinoma. A score of 50 and above was considered significant. The results showed that p53 expression increased progressively and significantly with the grade of pancreatic intraepithelial neoplasia and adenocarcinoma (p-value < 0.001). These findings support the concept of multistep carcinogenesis in pancreatic adenocarcinoma and suggest that p53 inactivation occurs in the progression of precursors to pancreatic adenocarcinoma.
Vasoactive intestinal peptide-producing tumour (VIPoma) or Verner-Morrison syndrome is a very rare neuroendocrine tumour. It occurs in less than ten percent of all pancreatic islet cell tumours, and about 70 percent to 80 percent of these tumours originate from the pancreas. Diagnosis is characteristically delayed. The first-line treatment is surgical. It may be curative in forty percent of patients with benign and non-metastatic disease. Palliative surgery is indicated in extensive disease, followed by conventional somatostatin analogue (octreotide) therapy. Somatostatin analogues improve hormone-mediated symptoms, reduce tumour bulk and prevent local and systemic effects. We present a female patient with VIPoma syndrome, which had metastasised to the liver at diagnosis. The patient underwent palliative Whipple procedure and subsequent cytoreductive radiofrequency ablations to her liver metastases. Unfortunately, after symptomatic improvement for three years, her disease progressed. Currently, she is on daily octreotide, achieving partial control of her symptoms.
Pancreatic carcinoid tumours are rare, particularly within the paediatric population. The clinical presentation is largely dependent on the functionality of the tumour. Although the tumour is generally slow-growing, surgical resection is still the mainstay of curative treatment. Morbidity is, however, significantly contributed by secretion of excess hormones; in view of this, biotherapy is an important treatment strategy. Octreotide, a somatostatin analogue, has been shown to be successful in both symptomatic control and stability of tumour progression. We report a 12-year-old girl, who presented with hypertensive crisis, and showed good response to a combination of chemotherapy and octreotide.
A 15-year-old girl, who was previously well, complained of a mass in the abdomen after a minor motor vehicle accident. Physical and radiological investigations revealed a mass in the body of pancreas containing proteinaceous material and multiple nodules in both lobes of liver. Serological investigations for malignancy were normal. Histopathological examination of the resected specimen showed pancreatoblastoma. Pancreatoblastoma is an unusual malignant tumour seen in infants and children although rare cases have also been reported in adults. They are clinicopathologically distinct from adult pancreatic ductal carcinoma. The histogenesis, clinical features and treatment options are discussed along with presentation of the case.
Recent studies have demonstrated a reduction in the morbidity and mortality of pancreatic resection and improvement in the actuarial 5-year survival for patients with resected ductal adenocarcinoma. We reviewed the clinico-pathological characteristics of patients who underwent resection with curative intent for ductal adenocarcinoma of the pancreas between 1980 and 1993.
Congo red screening of tumour material examined at the Department of Pathology, University of Malaya revealed intratumour deposits of amyloid in 12% of nasopharyngeal carcinomas, 66% of basal cell carcinomas, 100% of medullary carcinomas of the thyroid, 56% of islet cell tumours of the pancreas, 1 out of 16 carcinoids and 1 out of 100 thyroid adenomas. All the deposits were permanganate resistant and did not contain AA protein, indicating that what was encountered was not secondary amyloid. The deposits showed variable staining for immunoglobulin light chains and amyloid P component with a standard peroxidase antiperoxidase method. The possibility that intratumour amyloid has a neoplastic origin is discussed.