Changal Khalid Hamid, Mir Mohmad Hussain, Azaz Sheikh Aejaz, Qadri Sumyra Khurshid, Lone Abdul Rashid
Department of Internal Medicine, Sher-i-Kashmir Institute of Medical Sciences, Srinagar 190011, India.
Department of Medical Oncology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar 190011, India.
Malays J Med Sci. 2014 Nov-Dec;21(6):65-9.
Primitive neuroectodermal tumours (PNETs) are malignant tumours composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNETs originating in the pancreas are extremely rare; previous to this report, only 14 cases were reported worldwide, making this case the fifteenth in the world and the second in Asia. We present the case of a painful pancreatic lump diagnosed as PNET of the pancreas after a thorough workup. The diagnosis of PNET is made according to the overall clinical picture, imaging, histopathology, cytogenetics, and immunohistochemistry, as in the case we present. It is essential to differentiate primary pancreatic PNET from a secondary involvement. A review of all of the cases diagnosed worldwide thus far is also provided.
原始神经外胚层肿瘤（PNETs）是由神经外胚层起源的小圆形细胞组成的恶性肿瘤，可累及软组织和骨骼。起源于胰腺的PNETs极为罕见；在本报告之前，全球仅报道过14例，此病例为世界第15例、亚洲第2例。我们报告一例经全面检查后诊断为胰腺PNET的疼痛性胰腺肿块病例。如我们所报告的病例一样，PNET的诊断依据整体临床表现、影像学、组织病理学、细胞遗传学和免疫组织化学来做出。将原发性胰腺PNET与继发性累及相鉴别至关重要。本文还对迄今为止全球诊断的所有病例进行了综述。
