Yun Takamasa, Suzuki Hidemi, Mizobuchi Teruaki, Sakairi Yuichi, Nagato Kaoru, Nakajima Takahiro, Iwata Takekazu, Yoshida Shigetoshi, Nakatani Yukio, Yoshino Ichiro
Department of General Thoracic Surgery, Chiba University Graduate School of Medicine, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670 Japan.
Department of Pathology, Chiba University Graduate School of Medicine, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670 Japan.
Surg Case Rep. 2015;1(1):37. doi: 10.1186/s40792-015-0037-1. Epub 2015 Apr 24.
A 27-year-old female presented with a history of a right chest wall tumor at 3 years of age. At that time, the tumor was surgically resected and diagnosed as Ewing's sarcoma (EWS), and postoperative chemoradiotherapy was administered. The patient remained disease-free for 25 years. At age 27, chest computed tomography revealed a mass adjacent to the anterolateral thoracic wall. After surgery, the diagnosis was primitive neuroectodermal tumor (PNET). She died of the disease 10 months later. PNET and EWS were integrated into a single item in the 2002 WHO classification; thus, they are considered clinically and pathologically identical. The morphologic, immunohistochemical, and molecular biological characteristics of both specimens showed that the second tumor was a local recurrence of Ewing's sarcoma family of tumors (ESFT). Our case is the longest duration local recurrence reported. Long-term recurrences of ESFT and patients with recurrent ESFT have a poor prognosis; thus, long-term follow-up is necessary.
一名27岁女性有3岁时右胸壁肿瘤病史。当时，肿瘤经手术切除，诊断为尤因肉瘤（EWS），并进行了术后放化疗。患者25年无病生存。27岁时，胸部计算机断层扫描显示前胸壁外侧有一肿块。手术后，诊断为原始神经外胚层肿瘤（PNET）。10个月后她死于该病。PNET和EWS在2002年世界卫生组织分类中被归为一项；因此，它们在临床和病理上被认为是相同的。两个标本的形态学、免疫组织化学和分子生物学特征表明，第二个肿瘤是尤因肉瘤家族肿瘤（ESFT）的局部复发。我们的病例是报道的局部复发持续时间最长的。ESFT的长期复发以及复发性ESFT患者预后较差；因此，需要长期随访。
