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. 2014 Nov-Dec;21(6):65-9.

Primitive neuroectodermal tumour of pancreas; second case from Asia

Khalid Hamid Changal  1 , Mohmad Hussain Mir  2 , Sheikh Aejaz Azaz  2 , Sumyra Khurshid Qadri  3 , Abdul Rashid Lone  2
Affiliations

Primitive neuroectodermal tumour of pancreas; second case from Asia

Khalid Hamid Changal et al. Malays J Med Sci. 2014 Nov-Dec.

Abstract

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.

Keywords: neuroectodermal tumors; pancreas; pancreatic neoplasms; primitive.

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Figures

Figure 1:
Figure 1:
Abdominal contrast-enhanced computed tomography (CECT) showing a hypodense mass measuring 4 × 3.7 × 2.5 cm in the head region of the pancreas.
Figure 2:
Figure 2:
Biopsy showing features of small round cell tumor with pseudorosettes infiltrating the node, suggestive of primitive neuroectodermal tumours (PNET). A mitotic count of 19/hpf was seen. The pseudorosettes are indicated with asterisks.
Figure 3:
Figure 3:
Immunohistochemistry of the tumor, positive for membranous expression of CD99.
Figure 4:
Figure 4:
Photomicrograph showing diffuse strong immunostaining for synaptophysin.
Figure 5:
Figure 5:
Photomicrograph showing positive immunostaining for vimentin.
See this image and copyright information in PMC

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