Malignant Peripheral Nerve Sheath Tumour of Small Intestine, an Unusual Tumour: A Case Report and Review of the Literature

ABSTRACT

Neurofibromatosis type-1 (NF1), also known as von Recklinghausen disease, is a common autosomal dominant condition. It is a multisystem disorder. Abdominal involvement in patients with NF1 has been described in the form of neurofibromas within the liver, mesentery, retroperitoneum and gastrointestinal (GI) tract. Involvement of bowel is rarely seen. Malignant peripheral nerve sheath tumour of small intestine is a rare entity with very few cases reported in literature. Surgery remains the only curative option. Chemotherapy may be used as an adjuvant for advanced diseases. We report a case of malignant peripheral nerve sheath tumour of small intestine which presented as acute intestinal obstruction.