A Case of Pancreatic Neuroendocrine Tumor presenting Iron Deficiency Anemia in a Patient with Neurofibromatosis Type 1

A Case of Pancreatic Neuroendocrine Tumor presenting Iron Deficiency Anemia in a Patient with Neurofibromatosis Type 1

Loading document ...
Page
of
Loading page ...

Author(s)

Author(s): Xueyu Sun, Yanan Yu, Yueping Jiang, Deming Li

Download Full PDF Read Complete Article

DOI: 10.18483/ijSci.1210 261 892 51-56 Volume 6 - Mar 2017

Abstract

Neurofibromatosis type 1 (NF1) is a dominantly inherited disease characterized by cafe´-au-lait spots in the skin and dermal neurofibromas. We report an unusual case of a 38-year-old women with NF1 presented with abdominal pain and iron deficiency anemia. Imaging studies revealed a lesion in the head of the pancreas. At surgery, a 20 mm neuroendocrine tumor was found in the head of the pancreas. We present this case of a patient with pancreatic neuroendocrine tumors (PNETs) associated with NF1 and review the literatures that have been reported to discuss the characteristics of these tumors to improve early differential.

Keywords

Neurofibromatosis Type 1, Von Recklinghausen's Disease, Neuroendocrine Tumor, Pancreatic Neuroendocrine Tumor, Carcinoma, Familial Syndrome

References

  1. Agaimy A, Vassos N, Croner R S. Gastrointestinal manifestations of neurofibromatosis type 1 (Recklinghausen’s disease): clinicopathological spectrum with pathogenetic considerations. Int J Clin Exp Pathol. 2012. 5(9):852-862.
  2. Uusitalo E, Leppävirta J, Koffert A, et al. Incidence and mortality of neurofibromatosis: a total population study in Finland. J Invest Dermatol. 2015. 135(3): 904-906.
  3. Reynolds R M, Browning G G P, Nawroz I, Campbell I W. Von Recklinghausen’s neurofibromatosis: neurofibromatosis type 1. Lancet. 2003. 361: 1552–1554.
  4. Anlauf M, Garbrecht N, Bauersfeld J, et al. Hereditary neuroendocrine tumors of the gastroenteropancreatic system. Virchows Arch. 2007. 451 Suppl 1: S29-38.
  5. Gut P, Komarowska H, Czarnywojtek A, et al.Familial syndromes associated with neuroendocrine tumours. Contemp Oncol (Pozn). 2015. 19(3): 176-183.
  6. Crona J, Skogseid B. GEP- NETS UPDATE: Genetics of neuroendocrine tumors. Eur J Endocrinol. 2016. 174(6): R275-290.
  7. Relles D, Baek J, Witkiewicz A, Yeo CJ. Periampullary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases. J Gastrointest Surg. 2010. 14(6): 1052-1061.
  8. Katsinelos P, Lazaraki G, Chatzimavroudis G. An unusual case of obscure gastrointestinal bleeding in a patient with neurofibromatosis type 1. Clin Gastroenterol Hepatol. 2015. 13(3): A24-25.
  9. Coskey R L, Tranquada R E. Insulinoma and Multiple Neurofibromatosis : Report of a Case. Metabolism. 1964. 13(4): 312.
  10. Fung J W, Lam K S. Neurofibromatosis and insulinoma. Postgrad Med J. 1995. 71: 485-486.
  11. Fujisawa T, Osuga T, Maeda M, et al. Malignant endocrine tumor of the pancreas associated with von Recklinghausen’s disease. J Gastroenterol. 2002. 37:59–67.
  12. Perren A, Wiesli P, Schmid S, et al. Pancreatic endocrine tumors are a rare manifestation of the neurofibromatosis type 1 phenotype: molecular analysis of a malignant insulinoma in a NF-1 patient. Am J Surg Pathol. 2006. 30(8): 1047-1051.
  13. Takai A, Setoyama T, Miyamoto S. Pancreatic somatostatinoma with von Recklinghausen's disease. Clin Gastroenterol Hepatol. 2009. 7(5): A28.
  14. Bukkems SF, Stoot JH, Driessen A, Beets-Tan RG, van Nieuwenhoven MA, Masclee AM. A rare cause of obstructive jaundice and weight loss in Von Recklinghausen's disease. Neth J Med. 2010. 68(12): 414-417.
  15. Nishi T, Kawabata Y, Hari Y, et al. A case of pancreatic neuroendocrine tumor in a patient with neurofibromatosis-1. World J Surg Oncol. 2012. 10: 153.
  16. Rogers A, Wang LM, Karavitaki N, Grossman AB. Neurofibromatosis Type 1 and pancreatic islet cell tumours: an association which should be recognized. QJM. 2012. 108(7): 573-576.

Cite this Article:

International Journal of Sciences is Open Access Journal.
This article is licensed under a Creative Commons Attribution 4.0 International (CC BY 4.0) License.
Author(s) retain the copyrights of this article, though, publication rights are with Alkhaer Publications.

Search Articles

Issue June 2023

Volume 12, June 2023


Table of Contents



World-wide Delivery is FREE

Share this Issue with Friends:


Submit your Paper