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Case Reports
. 2015 May;9(5):XD03-XD04.
doi: 10.7860/JCDR/2015/13318.5933. Epub 2015 May 1.

Malignant Peripheral Nerve Sheath Tumour of Small Intestine Presenting as Ileo-Ileal Intussusception - A Rare Tumour with Unusual Complication

Diwakar Pandey  1 , Ankur Verma  2 , Azaz Akhtar  3 , Ashish Arsia  3 , Nain Singh  3
Affiliations
Case Reports

Malignant Peripheral Nerve Sheath Tumour of Small Intestine Presenting as Ileo-Ileal Intussusception - A Rare Tumour with Unusual Complication

Diwakar Pandey et al. J Clin Diagn Res. 2015 May.

Abstract

Malignant Peripheral Nerve Sheath Tumours (MPNST) arises from a peripheral nerve or exhibit nerve sheath differentiation on histology. Proximal portions of the upper and lower extremities and the trunk are the most common sites of occurrence. Around 50% are associated with Neurofibromatosis Type 1 (NF1) with incidence of two to five per cent in patients with NF1. The estimated incidence in general population without NF1 is 0.0001% of which gastrointestinal MPNST are extremely rare. A 45-year-old lady without pathological antecedent for NF1 was admitted with pain in right lower abdomen and multiple episodes of vomiting for 3 months. Preoperatively intussusception was diagnosed in the small bowel with USG and CECT abdomen showing characteristic target sign. On laparotomy Ileo-ileal intussusception (proximal ileum telescoping into distal ileum) was found 2 feet proximal to ileo-caecal junction with surrounding inflammed mesentery and presence of intraluminal tumour as lead point. Resection of involved segment of ileum along with its mesentery was done followed by ileo-ileal anastomosis. Histopathology was suggestive of high grade MPNST. Postoperative course and follow up for last 10 month is uneventful. This case is unique in terms of a rare tumour presenting with unusual complication and only one case had been reported so far in western literature.

Keywords: Gastrointestinal MPNST; Ileum; Neurofibromatosis.

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Figures

[Table/Fig-1]:
[Table/Fig-1]:
CECT whole abdomen (axial view) suggestive of characteristic “target sign” in small bowel intussusception
[Table/Fig-2]:
[Table/Fig-2]:
Resected ileum along with growth acting as lead point
[Table/Fig-3]:
[Table/Fig-3]:
The intraluminal extension of growth on opening ileum
[Table/Fig-4]:
[Table/Fig-4]:
Malignant spindle cells with wavy, hyperchromatic nuclei and alternating hypercellularity with hypocellularity . (H and E, ×100)
[Table/Fig-5]:
[Table/Fig-5]:
Histopathology microphotograph (H and E,x400) tumour cells are S-100 positive
See this image and copyright information in PMC

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