Gastric malignant schwannoma in a child

Malignant schwannoma (malignant peripheral nerve sheath tumour, MPNST) is a high grade sarcoma with a potential for local recurrence and distant metastasis that may occur at any site in the body where there is neural tissue. MPNST is rare in children and is unreported in the stomach in the paediatric age group. MPNST presents either as an expanding mass, with or without pain, or in the gastrointestinal tract with haemorrhage or obstruction. Many cases occur without evidence of neurofibromatosis but there is a reported association between MPNST and neurofibromatosis of up to 50%. MPNST has a higher incidence at sites of previous irradiation. Treatment is by complete surgical excision. Radiology is of value in initial diagnosis and staging prior to surgery. The definitive diagnosis is made on the histopathological appearance and immunohistochemical profile. The findings on barium meal, abdominal ultrasound and CT are presented.