Fuse Yoshinobu, Mori Shohei, Sato Shun, Kato Daiki, Shibazaki Takamasa, Nakada Takeo, Yabe Mitsuo, Matsudaira Hideki, Hirano Jun, Ohtsuka Takashi
Division of Thoracic Surgery, Department of Surgery, The Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minatoku, Tokyo, 105-0003, Japan.
Department of Pathology, The Jikei University School of Medicine, 3-25-8 Nishishinbashi, Minatoku, Tokyo, 105-0003, Japan.
Surg Case Rep. 2021 Oct 30;7(1):233. doi: 10.1186/s40792-021-01314-4.
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with malignant potential. No effective treatment other than surgical resection has been established for lung metastases of PEComas. We describe a patient who underwent complete surgical resection via bilateral lobectomy involving a two-step procedure for lung metastases 8 years after undergoing radical surgery for a colonic PEComa.
A 53-year-old woman underwent partial colectomy for a PEComa in the transverse colon 8 years ago. She presented with an abnormal chest shadow during a health examination. Chest computed tomography (CT) revealed a solid nodule 2 cm in diameter located centrally in the right lower lobe and a solid nodule 3 cm in diameter located centrally in the left upper lobe. Positron emission tomography revealed 18F-fluorodeoxyglucose uptake in these nodules. These nodules were suspected to be metastatic tumors of the colonic PEComa and were considered for complete surgical resection. Segmentectomy could not be performed because of the anatomical location of the tumors straddling the segments; therefore, bilateral lobectomy was required for complete surgical resection. Therefore, we performed two-step lobectomy safely with the expectation of pulmonary function recovery. Microscopically, the tumors were diagnosed as lung metastases of the PEComa. One year after the last surgery, no recurrence was detected, and the patient's pulmonary function improved.
This case indicates that even if multiple lung metastases of a PEComa require bilateral lobectomy, complete resection with a two-step surgery may be considered.
血管周上皮样细胞瘤（PEComas）是一种罕见的具有恶性潜能的间叶性肿瘤。对于PEComas的肺转移，除手术切除外，尚未确立有效的治疗方法。我们描述了一名患者，在接受结肠PEComa根治性手术后8年，因肺转移瘤接受了经双侧肺叶切除术的完全手术切除，该手术分两步进行。
一名53岁女性8年前因横结肠PEComa接受了部分结肠切除术。她在一次健康检查中出现胸部阴影异常。胸部计算机断层扫描（CT）显示右肺下叶中央有一个直径2厘米的实性结节，左肺上叶中央有一个直径3厘米的实性结节。正电子发射断层扫描显示这些结节中有18F-氟脱氧葡萄糖摄取。这些结节被怀疑是结肠PEComa的转移瘤，并考虑进行完全手术切除。由于肿瘤的解剖位置跨越肺段，无法进行肺段切除术；因此，需要进行双侧肺叶切除术以完成手术切除。因此，我们分两步安全地进行了肺叶切除术，期望肺功能能够恢复。显微镜检查显示，这些肿瘤被诊断为PEComa的肺转移瘤。最后一次手术后一年，未发现复发，患者的肺功能有所改善。
该病例表明，即使PEComa的多个肺转移瘤需要进行双侧肺叶切除术，也可以考虑通过两步手术进行完全切除。
