Résumé
PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare. The case is being reported for its rarity. METHODS: A 53-year-old female was presented with a scalp mass on vertex area. The tumor was localized in scalp skin and did not invade underlying periosteum or skull and treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. RESULTS: Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made. CONCLUSION: Authors experienced a rare case of primary scalp MPNST and report the case.
Sujets)
Femelle , Humains , Adulte d'âge moyen , Traitement médicamenteux adjuvant , Diagnostic , Immunohistochimie , Neurinome , Neurofibrome , Neurofibrosarcome , Périoste , Nerfs périphériques , Radiothérapie , Sarcomes , Cuir chevelu , Peau , CrâneRésumé
Although the head and neck region is recognized as the most common location for peripheral nerve sheath tumors, central involvement, particularly in the jaw bones, is quite unusual. Neurofibroma is one of the most common nerve sheath tumors occurring in the soft tissue and generally appears in neurofibromatosis 1 (NF1 or von Recklinghausen's disease). Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in the soft tissue. Here, we report four cases of intraosseous peripheral nerve sheath tumors occurring in the jaw bones and compare the clinical, radiologic, and pathologic findings in order to make a differential diagnosis.
Sujets)
Mâle , Humains , Femelle , Enfant , Adulte , Adolescent , Rayons X , Sarcomes/diagnostic , Neurofibromatoses/anatomopathologie , Neurofibrome/anatomopathologie , Tumeurs des gaines nerveuses/diagnostic , Mâchoire/imagerie diagnostique , Diagnostic différentiel , Tumeurs osseuses/diagnosticRésumé
A 75-year-old female presented with a 1-month history of low abd pain. A 15 x 13 cm solid mass was noted in pelvic cavity by imaging study. The patient underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic lymph node dissection and omentectomy. Immunohistochemistry aided in the final diagnosis of a malignant peripheral nerve sheath tumor. The patient had several poor prognosis factor and relapsed into tumor and died 5 month after surgery. An extremely rare case of malignant peripheral nerve sheath tumor of pelvic cavity is reported with a brief review of the literature.