Secrete and Exude-Pancreatic Neuroendocrine Tumour

Abstract

Pancreatic neuroendocrine tumour is derived from neuroendocrine cells of pancreas or hormone secreting cells within vicinity of pancreas and is categorized into indolent well differentiated neuroendocrine tumour (WDNET) and the aggressive, rapidly progressive poorly differentiated neuroendocrine carcinoma(PDNEC). Well differentiated neuroendocrine tumour (WDNET) demonstrates inactivation of MEN1, genetic mutation or loss of DAXX / ATRX, altered mTOR pathway, incrimination of PTEN, TSC2, PIK3CA genes with alteration of VHL. Poorly differentiated neuroendocrine carcinoma and mixed neuroendocrine / non neuroendocrine neoplasm delineates alterations within TP53, RB, KRAS, APC, BRAF or SMAD4 / DPC4 genes. Neuroendocrine tumour delineates an organoid architecture with solid nests, trabeculae, gyri, cords, festoons, ribbons, glandular, pseudoacinar or tubulo-acinar articulations. Miniature to intermediate tumour cells are permeated with eosinophilic, amphophilic, finely granular cytoplasm, centric, spherical to elliptical, uniform nuclei with finely stippled ‘salt and pepper’ nuclear chromatin, inconspicuous nucleoli and an abundant circumscribing vascular network. Pancreatic neuroendocrine tumour is immune reactive to CK8 / CK18, CAM 5.2, OSCAR, AE1 / AE3, synaptophysin, chromogranin A, INSM1, CD56, neuron specific enolase (NSE) or CD57. Pancreatic neuroendocrine tumour pancreas requires segregation from neoplasms such as acinar cell carcinoma, pancreatoblastoma, solid pseudo-papillary neoplasm, pancreatic ductal adenocarcinoma, paraganglioma, clear cell tumours as clear cell sarcoma, metastatic renal cell carcinoma, PEComa or solid variant of serous cystadenoma. Comprehensive surgical eradication is recommended for treating well differentiated neuroendocrine tumour. Poorly differentiated neuroendocrine tumour is appropriately treated with platinum based chemotherapy.