Abstract

A rare case of malignant schwannoma arising in the right breast region of the thorax associated with von Recklinghausen's disease is reported. A 49-year-old female presented with a rapidly enlarging mass in the right breast. Chest CT revealed that the large tumor occupied the right breast and the bony thorax, further growing into the thoracic cavity. The chest wall resection and reconstruction by using a contralateral rectus abdominis musculocutaneous flap were performed. The tumor adhered to the lung, diaphragma and pericardial wall, and was accompanied by disseminated nodules. The excised main tumor of a dumbell-shape was 20.5×15×11.5cm in size and 2060g in weight. Histological appearance of the tumor showed a malignant schwannoma probably arising from von Reckling hausen's disease. On immunohistochemical examination S-100 proein was positive in some tumor cells. Postoperative chemotherapy was done but the patient died of recurrence and respiratory insufficiency about 5 months after surgery.

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