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Vol.58 No.3 contents | Japanese/English |
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- Case Report -
A Case of High-grade Lung Metastasis with Malignant Peripheral Nerve Sheath Tumor in a Patient with von Recklinghausen Disease
Naoki Suzuki1, Toshiaki Masaoka1, Ko Sato2, Hidenori Kawakami2, Shinya Uchigasaki3, Noriko Izumi41Department of Thoracic Surgery, 2Department of Respiratory Medicine, 3Department of Pathology, 4Department of Internal Medicine, Tsuruoka Municipal Shonai Hospital, Japan
Background. The most common metastatic site of malignant peripheral nerve sheath tumor is lung, and the long-term survival can be expected with complete resection; however, the surgical indications need to be judged carefully. Case. A 23-year-old man had been diagnosed with von Recklinghausen disease at birth. The patient had undergone resection of the nasal root tumor and been subsequently diagnosed with malignant peripheral nerve sheath tumor. Two years later, he was admitted to our hospital with a complaint of right-sided chest pain, and chest X-ray revealed a circular nodule in the lower left lung field. Computed tomography (CT) indicated a tumor in the left lower lung lobe and the left adrenal gland. Positron emission tomography-CT showed a high accumulation in both the lung and adrenal gland lesions. A bronchoscopic examination revealed no malignant cells; however, malignant tumor of the lung was suspected based on the course and imaging findings, so for a definite diagnosis and treatment, he underwent a resection of the left lung S8 area. A pathological examination showed the transition of malignant peripheral nerve sheath tumor. Immunostaining suggested that the numbers of S100 protein-positive cells were fewer than in the nasal root tumor, and the MIB-1 labeling index was high, and metastases increased over time. In June, the left adrenal gland and left kidney tumor were excised; however, rapid progression of the disease was observed, and the patient ultimately died in August. Conclusion. In the postoperative metastasis of malignant peripheral nerve sheath tumor, whether or not to perform surgery must be carefully considered based on the malignancy of the disease determined according to the pathological histology and immunostaining.
key words: Malignant peripheral nerve sheath tumor, von Recklinghausen disease, Metastasectomy, Pulmonary metastasis, Malignant conversion
Received: November 9, 2017
Accepted: March 4, 2018
JJLC 58 (3): 211-215, 2018
