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Anales de la Facultad de Ciencias Médicas (Asunción)

Print version ISSN 1816-8949


MARTINEZ BARRIOS, Edgar; BORTOLATTO, Léia; MARTINEZ BOGADO, Ernesto  and  NOGUERA, Oscar Gustavo. Neurofibromatosis 1 associated to malignant peripheral nerve sheath tumor: A radiological approach. An. Fac. Cienc. Méd. (Asunción) [online]. 2020, vol.53, n.2, pp.157-164. ISSN 1816-8949.


Introduction: Neurofibromatosis (NF) is characterized by ectodermal abnormality with the presence of multiple neurofibromas throughout the body. The most common form is NF1, which occurs with variable manifestations. Malignant neurilemmoma or peripheral nerve sheath malignant tumor (PNSMT) is one of the tumor formations that can be seen in the context of this pathology. The role of radiology is to characterize the injury and its invasion, as well as to assess the presence of other tumor lesions.

Case report: Adult male patient, carrier of Neurofibromatosis type I and hemorrhoids, presented with digestive hemorrhage, so an endoscopy was performed where a prepyloric stenosing lesion was found. A tomographic scan was requested where benign-looking lesions were identified in the liver, both adrenal glands, also a malignant-looking tumor lesion on the left rib cage.

Discussion: An experienced knowledge of the pathology with the radiological findings that are usually observed in these patients are of utmost importance so that the radiologist can know the location of the lesions, both classic and unusual, and orient between a character injury benign with others of malignant character.

Keywords : Neurofibromatoses; neurofibrosarcoma; diagnostic imaging; tomography..

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