Singapore Med J 2013; 54(2): e31-e34; http://dx.doi.org/10.11622/smedj.2013036
Lipid-rich variant of pancreatic endocrine tumour with inhibin positivity and microscopic foci of microcystic adenoma-like areas: emphasis on histopathology
Rao AC, Monappa V, Shetty P
Correspondence: Dr Anuradha CK Rao, anuchenna@yahoo.com
ABSTRACT
Pancreatic endocrine tumours (PETs) are uncommon tumours with typical morphology characterised by relatively uniform cuboidal cells arranged in nests and festoons, with distinctive nuclear salt-and-pepper chromatin. A lipid-rich variant poses diagnostic difficulties in the midst of other pancreatic tumours and metastatic goblet cell carcinoid. A 22-year-old man presented with symptoms of abdominal pain and jaundice. His liver function test and blood glucose level were normal, but computed tomography of the abdomen suggested the presence of a tumour in the head of the pancreas. Specimen obtained by pancreaticoduodenectomy revealed an infiltrating yellow-tan tumour composed of nests and a cribriform arrangement of polygonal vacuolated cells with pyknotic nuclei, along with focal classical areas of PET. Two foci of early serous microcystic adenoma were seen. Immunohistochemistry contributed to the arrival of a conclusive diagnosis. Von Hippel-Lindau disease was excluded in our patient, as other supportive classical features of the syndrome were absent.
Keywords: lipid-rich variant, microcystic adenoma, pancreatic endocrine tumour, von Hippel-Lindau
Singapore Med J 2013; 54(2): e31–e34; http://dx.doi.org/10.11622/smedj.2013036
REFERENCES
1. Reznek RH. CT/MRI of neuroendocrine tumours. Cancer Imaging 2006; 6:S163-77.
http://dx.doi.org/10.1102/1470-7330.2006.9037 |
| |
2. Jensen RT, Berna MJ, Bingham DB, Norton JA. Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management and controversies. Cancer 2008; 113(7 Suppl):1807-43.
http://dx.doi.org/10.1002/cncr.23648 |
| |
3. Tamura K, Nishimori I, Ito T, et al. Diagnosis and management of pancreatic endocrine tumor in von Hippel-Lindau disease. World J Gastrenterol 2010; 16:4515-18.
http://dx.doi.org/10.3748/wjg.v16.i36.4515 |
| |
| 4. Neumann HP, Dinkel E, Brambs H, et al. Pancreatic lesions in Von-HippelLindau syndrome. Gastroenterology 1991; 101:465-71. |
| |
5. Blandamura S, Parenti A, Famengo B, et al. Three cases of pancreatic serous cystadenoma and endocrine tumour. J Clin Pathol 2007; 60:278-82.
http://dx.doi.org/10.1136/jcp.2006.036954 |
| |
| 6. Musso C, Paraf F, Petit B, Archambeaud-Mouveroux F, Valleix D, Labrousse F. [Pancreatic neuroendocrine tumors and Von Hippel Lindau disease]. Ann Pathol 2000; 20:130-3. French. |
| |
7. Singh R, Basturk O, Klimstra DS, et al. Lipid-rich variant of pancreatic endocrine neoplasms. Am J Surg Pathol 2006; 30:194-200.
http://dx.doi.org/10.1097/01.pas.0000184819.71752.ad |
| |
8. Verbeke CS. Endocrine tumours of the pancreas. Histopathology 2010; 56:669-82.
http://dx.doi.org/10.1111/j.1365-2559.2010.03490.x |
| |
9. Ordonez NG, Silva EG. Islet cell tumour with vacuolated lipid-rich cytoplasm: a new histological variant of islet cell tumour. Histopathology 1997; 31:157-60.
http://dx.doi.org/10.1046/j.1365-2559.1996.2230837.x |
| |
10. Loos M, Bergmann F, Bauer A, et al. Solid type clear cell carcinoma of the pancreas: differential diagnosis of an unusual case and review of the literature. Virchows Arch 2007; 450:719-26.
http://dx.doi.org/10.1007/s00428-007-0416-5 |
| |
| 11. Delellis RA, Lloyd RV, Heitz PU, Eng C, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumour of Endocrine Organs. Lyon: IARC Press, 2004: 175-207. |
