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Cutaneous marker of an internal malignancy
Digestive and Liver Disease 43 (2011) e16
Contents lists available at ScienceDirect
Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld
Image of the Month
Cutaneous marker of an internal malignancy Pazhanivel Mohan ∗ , Ravi Ramakrishnan, Mohan Kaduganoor Ramakrishnan, Jayanthi Venkataraman Department of Gastroenterology, Stanley Medical College, Chennai, India
a r t i c l e
i n f o
Article history: Received 26 November 2010 Accepted 2 January 2011 Available online 15 February 2011
Fig. 2.
Fig. 1.
A 32-year old woman presented with progressively increasing jaundice, intractable pruritus and pale stools for 2 months. There was no fever, prodromal symptoms, abdominal pain, vomiting or gastrointestinal bleeding. She had also noticed a rapid increase in abdominal girth over the last month. Examination of her skin (Fig. 1A) showed brownish hyperpigmented macules and subcutaneous nodules suggestive of neurofibromatosis type 1 (NF1). She was deeply icteric with moderate ascites. Ultrasound of the abdomen showed a dilated common bile duct, intrahepatic biliary radicles and ascites. Side viewing endoscopy demonstrated a periampullary growth (Fig. 1B) with histology comprising of intestinal mucosa with adjacent malignant glands infiltrating the underlying submucosa and muscularis consistent with adenocarcinoma (Fig. 2A and B). Ascitic fluid cytology was positive for malignant cells.
Patients with NF1 are at increased risk of gastrointestinal malignancies like neurofibromas, gastrointestinal stromal tumours and periampullary endocrine tumours. The incidence varies between 10 and 25% [1]. The exact pathogenesis remains unclear. Carcinoid tumours constitute most of periampullary neoplasms [1]. The recent review of periampullary tumours in neurofibromatosis reported adenocarcinoma to be more common than neurofibroma in contrary to what was previously believed [1]. Reference [1] Relles D, Baek J, Witkiewicz A, et al. 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: 1052–61.
∗ Corresponding author. E-mail address: [email protected] (P. Mohan). 1590-8658/$36.00 © 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.dld.2011.01.009
Contents lists available at ScienceDirect
Digestive and Liver Disease journal homepage: www.elsevier.com/locate/dld
Image of the Month
Cutaneous marker of an internal malignancy Pazhanivel Mohan ∗ , Ravi Ramakrishnan, Mohan Kaduganoor Ramakrishnan, Jayanthi Venkataraman Department of Gastroenterology, Stanley Medical College, Chennai, India
a r t i c l e
i n f o
Article history: Received 26 November 2010 Accepted 2 January 2011 Available online 15 February 2011
Fig. 2.
Fig. 1.
A 32-year old woman presented with progressively increasing jaundice, intractable pruritus and pale stools for 2 months. There was no fever, prodromal symptoms, abdominal pain, vomiting or gastrointestinal bleeding. She had also noticed a rapid increase in abdominal girth over the last month. Examination of her skin (Fig. 1A) showed brownish hyperpigmented macules and subcutaneous nodules suggestive of neurofibromatosis type 1 (NF1). She was deeply icteric with moderate ascites. Ultrasound of the abdomen showed a dilated common bile duct, intrahepatic biliary radicles and ascites. Side viewing endoscopy demonstrated a periampullary growth (Fig. 1B) with histology comprising of intestinal mucosa with adjacent malignant glands infiltrating the underlying submucosa and muscularis consistent with adenocarcinoma (Fig. 2A and B). Ascitic fluid cytology was positive for malignant cells.
Patients with NF1 are at increased risk of gastrointestinal malignancies like neurofibromas, gastrointestinal stromal tumours and periampullary endocrine tumours. The incidence varies between 10 and 25% [1]. The exact pathogenesis remains unclear. Carcinoid tumours constitute most of periampullary neoplasms [1]. The recent review of periampullary tumours in neurofibromatosis reported adenocarcinoma to be more common than neurofibroma in contrary to what was previously believed [1]. Reference [1] Relles D, Baek J, Witkiewicz A, et al. 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: 1052–61.
∗ Corresponding author. E-mail address: [email protected] (P. Mohan). 1590-8658/$36.00 © 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.dld.2011.01.009