A Case of Pulmonary Metastasis of Malignant Nerve Sheath Tumor with Neurofibromatosis Type 1

KIDOKORO Yoshiteru, SUZUKI Yoshimasa, KADONAGA Taichi, YOSHIDA Haruhiko, NAKAMURA Hiroshige

doi:10.3919/jjsa.78.1267

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神経線維腫症1型に合併した悪性末梢神経鞘腫瘍肺転移の1例
症例神経線維腫症1型に合併した悪性末梢神経鞘腫瘍肺転移の1例
ショウレイシンケイセンイシュショウ 1ガタニガッペイシタアクセイマッショウシンケイサヤシュヨウハイテンイノ 1レイ

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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma. The lung is the most common metastatic site of MPNST. The patient was a 79-year-old woman who had been diagnosed with neurofibromatosis type 1 several decades previously. She underwent extended tumorectomy with irradiation (66 Gy) for MPNST in the left femur in the Department of Orthopedics 6 months earlier. Chest X-ray showed a 2-cm nodule in the left upper lung field. Chest computed tomography showed a 17×14mm solitary tumor in the left upper lobe (S¹⁺²) of lung. However, no local recurrence was found. The lung tumor which invaded from the visceral pleura to the lower lobe was resected via video-assisted thoracic surgery, and histologically diagnosed as pulmonary metastasis of MPNST. No adjuvant therapy was conducted. Multiple lung metastases and chest wall recurrence occurred 2 months after pulmonary metastasectomy. Despite palliative treatments including pain control, the disease progressed and she died 4 months after the second surgery.

Journal

Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)

Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 78 (6), 1267-1271, 2017

Japan Surgical Association

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