RESUMEN
Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle- cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise as sporadic variants or in patients with neurofibromatosis1 (NF1). The estimated incidence of MPNSTs in the patients with NF1is 2-5% as compared to a 0.001% incidence in the general population. Herein, we are reporting a case of a giant sporadic (NF1 independent) low grade MPNST of the left thigh in 65 year female patient, which had a good prognosis.
RESUMEN
Solid pseudopapillary neoplasm of pancreas (SPN) is a rare entity; therefore proper diagnosis, evaluation and formulation of treatment protocols is difficult. A total of 14 cases were diagnosed and treated at our institute over a 10-year period (1992-2002). A retrospective study of all these cases was performed with respect to age group most frequently affected, sex ratio, common presenting symptoms and signs, investigative protocols, pathological features, treatment offered, outcome and prognosis. All the 14 patients were females, with a median age of 20 years (range 13-45 years). The commonest presenting symptom was abdominal pain. All these patients underwent surgical exploration; 13 patients underwent resection of the tumour. The average length of postoperative hospital stay was 10.3 days. Significant postoperative morbidity was seen in only one case. There was no postoperative mortality. All these patients who underwent resection were disease-free on follow-up ranging from 6 to 96 months (median 36 months).