2008, Number 3
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ABSTRACTNeurogenic sarcomas are uncommon tumors, with an estimated incidence of 0.001% in the general population. Because its rarity, these tumors are often treated as a subcategory of soft tissue tumors. About half of neurogenic sarcoma patients also have neurofibromatosis type 1 or von Recklinghausen disease, a disorder for which an increased risk of malignancies of 3 to 15% has been reported. Other terms used for this tumor include: malignant schwannoma, malignant peripheral nerve sheath tumor, malignant neurilemmoma, and neurofibrosarcoma. The most common locations are the trunk and limbs. The case of a 27 years old male patient who went to the emergency department because of incoercible pain of the left hemithorax is presented. Physical examination showed clinical data of neurofibromatosis type 1 and a tumor of approximately 20 cm diameter in the left hemithorax, with no data of wasting syndrome. Analgesia was administered, and owing to the mass effect and excessive weight of the tumor a study protocol for its excision was begun. A surgical procedure was performed in which the tumoral mass and segments of the chest wall were resected, with placement of a Marlex mesh. During the surgical intervention, metastasis to mediastinum and involvement of the parietal pleura were observed. The postoperative evolution was acceptable, and the patient was referred to an oncologic hospital for monitoring and subsequent management; however, he died with liver metastases one year after surgery.
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