Minagawa Tomohiro, Shioya Ryuta, Sato Chigusa, Shichinohe Ryuji, Yasui Go, Ishikawa Kohsuke, Takahashi Hiroko
Department of Plastic and Reconstructive Surgery, Asahikawa-Kosei General Hospital, 1 Jo-dohri 24-chome 111, Asahikawa City, Hokkaido 078-8211, Japan.
Case Rep Oncol Med. 2011;2011:705345. doi: 10.1155/2011/705345. Epub 2011 Sep 6.
Malignant peripheral nerve sheath tumor (MPNST) is a rare high-grade soft tissue sarcoma. The epithelioid variant accounts for 5% or less of MPNSTs; the clinical behavior of this variant is unclear. Reports of approximately 40 cases are available in the English literature; however, most reports addressed clinicopathological features rather than therapeutic procedures or clinical courses. We describe a case of a 62-year-old male with an epithelioid MPNST of the left foot. Multiple lung metastases developed after radical surgery on the primary lesion. The response to adjuvant chemotherapy including doxorubicin and ifosfamide was favorable, and thoracoscopic resection was subsequently performed on the remaining three metastases. No evidence of recurrence or metastasis was observed at the 12-month followup after the first operation. Further followup and chemotherapy may be required.
恶性外周神经鞘膜瘤（MPNST）是一种罕见的高级别软组织肉瘤。上皮样变体占MPNST的5%或更少；该变体的临床行为尚不清楚。英文文献中有大约40例相关报道；然而，大多数报道关注的是临床病理特征，而非治疗方法或临床病程。我们描述了一例62岁男性，患有左足上皮样MPNST。原发灶根治性手术后出现了多发肺转移。对包括阿霉素和异环磷酰胺在内的辅助化疗反应良好，随后对其余三处转移灶进行了胸腔镜切除术。首次手术后12个月的随访未发现复发或转移迹象。可能需要进一步随访和化疗。
