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  1. Alshareefy Y, Cummins S, Mazzoleni A, Sharma V, Guggilapu S, Leong AWY, et al.
    Medicine (Baltimore), 2023 Nov 17;102(46):e36094.
    PMID: 37986400 DOI: 10.1097/MD.0000000000036094
    Pancreatic neuroendocrine tumors (PanNETs) are a rare subtype of pancreatic cancer and can be divided into functional (30-40%) and nonfunctional subtypes. The different subtypes of functional PanNETs (F-PanNETs) have a variety of classical presentations that raise suspicion for an underlying PanNET. It is estimated that 90% of PanNETs are sporadic, and the PI3K-Akt-mTOR and ATRX/DAXX signaling pathways have been recognized as key genetic pathways implicated in the pathogenesis. The other 10% of PanNETs may occur in the context of familial cancer syndromes such as MEN1. Chromogranin A is the most useful biomarker currently; however, several studies have shown limitations with its use, especially its prognostic value. Synaptophysin is a novel biomarker which has shown promising preliminary results however its use clinically has yet to be established. Blood tests assessing hormone levels, cross-sectional imaging, and endoscopic ultrasound remain at the core of establishing a diagnosis of F-PanNET. The treatment options for F-PanNETs include surgical methods such as enucleation, systemic therapies like chemotherapy and novel targeted therapies such as everolimus. The prognosis for F-PanNETs is more favorable than for nonfunctional PanNETs, however metastatic disease is associated with poor survival outcomes. Researchers should also focus their efforts on identifying novel pathways implicated in the pathogenesis of F-PanNETs in order to develop new targeted therapies that may reduce the need for surgical intervention and on the establishment of novel biomarkers that may reduce the need for invasive testing and allow for earlier detection of F-PanNETs.
    Matched MeSH terms: Adenoma, Islet Cell*
  2. Fock KM, Nambiar R
    Med J Malaysia, 1984 Jun;39(2):170-2.
    PMID: 6096683
    A 22-year-old Malay man with recurrent hypoglycaemic fainting spells was found to have hyperinsulinism. Although the CT scan of the abdomen and arteriogram failed to demonstrate any tumour in the pancreas, three tumours were found in the body of the pancreas at laporatomy, An appraisal of the techinques currently available for diagnosis and localisation of insulinoma is presented.
    Matched MeSH terms: Adenoma, Islet Cell/diagnosis*
  3. Mohamed Shah FZ, Mohamad AF, Zainordin NA, Eddy Warman NA, Wan Muhamad Hatta SF, Abdul Ghani R
    Ann Med Surg (Lond), 2021 Apr;64:102240.
    PMID: 33868680 DOI: 10.1016/j.amsu.2021.102240
    Introduction: Insulinoma is a functioning pancreatic neuroendocrine tumor primarily leading due to hypoglycemia due to hypersecretion of insulin. This case illustrates the real challenges faced in the detection of an occult insulinoma, which resulted in a protracted course of the disease.

    Case presentation: A 33-year-old female presented with recurrent hypoglycemia. Endogenous hyperinsulinemia was confirmed by a prolonged fast, however serial imaging was negative. Incidental finding of an ovarian mass gave rise to the suspicion of an insulin-producing ovarian tumor. Subsequent multimodality pancreatic imaging remained negative, requiring more invasive investigations. The tumor was localized by specialized arteriography using calcium stimulation to support the diagnosis of an insulinoma. However, repeated negative imaging led to further delays in definitive management, with worsening hypoglycemia. The surgery was finally performed three years after the initial presentation with successful removal of the tumor using intra-operative ultrasound.

    Clinical discussion: It is important to emphasize that preoperative radiological imaging is useful to localize pancreatic lesions. However, most insulinomas could only be detected intraoperatively. The absence of suggestive radiological evidence should not deter surgeons from proceeding with definitive surgical intervention.

    Conclusion: The case highlights the importance of a multidisciplinary approach in the management of a complicated case.

    Matched MeSH terms: Adenoma, Islet Cell
  4. Looi LM
    Ann Acad Med Singap, 1986 Jan;15(1):52-6.
    PMID: 3010797
    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.
    Matched MeSH terms: Adenoma, Islet Cell/pathology
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