Abstract
BackgroundPerivascular 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.Case presentationA 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.ConclusionsThis case indicates that even if multiple lung metastases of a PEComa require bilateral lobectomy, complete resection with a two-step surgery may be considered.
Highlights
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with malignant potential
We report a patient who underwent complete surgical resection via left upper and right lower lobectomy involving a two-step procedure for bilateral lung metastases detected 8 years after undergoing radical surgery for a PEComa in the colon
We considered that the patient would tolerate bilateral lobectomy because the predicted postoperative % forced expiratory volume in one second (FEV1) was 56%
Summary
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms originating in various organs, including the retroperitoneum, uterus, uterine cervix, gastrointestinal tract, kidneys, liver, breasts, and lungs [1]. We report a patient who underwent complete surgical resection via left upper and right lower lobectomy involving a two-step procedure for bilateral lung metastases detected 8 years after undergoing radical surgery for a PEComa in the colon. 18F-fluorodeoxyglucose-positron emission tomography revealed maximum standardized uptake values of 8.3 and 5.7 in each lesion (Fig. 1c, d) These nodules were suspected to be metastatic tumors of the colonic PEComa and were considered for complete surgical resection. Immunohistochemistry assessment revealed that the tumor cells tested positive for melan-A (Fig. 2c), human melanoma black-45 (HMB-45), caldesmon, and S100 and negative for cluster of differentiation (CD), chromogranin A, synaptophysin, AE1/AE3, desmin, α-smooth muscle actin, CD34, CD117, and CAM5.2 Based on these findings, the tumor was diagnosed as lung metastasis of the PEComa.
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