Malignant Peripheral Nerve Sheath Tumor of the Mediastinum

Shijubo, Noriharu; Sugaya, Fumiko; Imada, Akiriho; Fujishima, Takuya; Nakata, Hajime; Satoh, Masaaki; Abe, Shosaku

doi:10.1159/000029525

Cited by 1 publication

(2 citation statements)

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“…Expression of S-100 protein, a cytoplasmic marker useful for identifying Schwann cells, is often negative in MPNSTs. 5 To the best of our knowledge, there are 40 reported cases of MPNSTs occurring in the mediastinum in the English-language medical literature 2-10 ( Table 2). Ten of these cases were accompanied by NF-1.…”

Section: Discussionmentioning

confidence: 99%

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Long-term survival after removal of a malignant peripheral nerve sheath tumor originating in the anterior mediastinum

Shimoyama

Yoshiya

Yamato

et al. 2009

Gen Thorac Cardiovasc Surg

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Malignant peripheral nerve sheath tumors (MPNSTs; malignant schwannomas) rarely occur in the anterior mediastinum, and their prognosis is poor. A 75-year-old man was referred to our hospital for examination of an anterior mediastinal tumor. A computed tomography-guided percutaneous needle biopsy revealed only fibrosis. The tumor was completely excised via a median sternotomy with partial resection of the pericardium and right upper lobe of the lung. Thereafter, the tumor was diagnosed as a storiform-pleomorphic type of malignant fibrous histiocytoma. At 1 year after the surgery, a distant metastasis was found in the interlobular space between the right middle and lower lobes. The tumor was completely excised via a right posterolateral thoracotomy. Reexamination of the primary and secondary tumors revealed an MPNST. No recurrence was found up to 5 years after the second surgery without adjuvant chemotherapy or radiation therapy. However, he died from multiple lung metastases after 6 years.

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Section: Discussionmentioning

confidence: 99%

“…7,9 With complete surgical resection, the expected 5-year survival rate is 50%-75%. 5,8 However, malignant triton tumors have a signifi cantly worse prognosis than typical MPNSTs. The local recurrence rate is 42%-65%, and the distant metastasis rate is 28%-68%.…”

Section: Discussionmentioning

confidence: 99%