• Xilin Liu, Lixin Guo, Xiangfu Ding, and Zhichen Kang.
• Department of Hand and Foot Surgery, China-Japan Union Hospital of Jilin University, Changchun, China.
• Medicine (Baltimore). 2024 Jul 5; 103 (27): e38797e38797.

RationalePulmonary sarcomatoid carcinoma (PSC), a rare tumor, comprises 0.1% to 0.4% of all malignant lung tumors. Given the rarity of PSC, its clinical course, therapeutic guidelines, and patient outcomes remain largely unknown. Therefore, it is imperative to alert clinicians to this extremely rare and instructive early-onset cancer.Patient ConcernsThis report describes a 28-year-old woman with PSC, who was initially misdiagnosed with Whipple's disease. A conclusive diagnosis of PSC was made following careful clinical examination, imaging, and histopathological evaluation of the patient's biopsy sample. Radiological imaging revealed multiple nodules and mass formations in the left upper lobe of the patient's lung, with the largest measuring of 5.4 × 3.2 cm.DiagnosisHistopathological examination indicated the presence of a malignant neoplasm associated with necrosis suggestive of sarcoma, which was pathologically staged as cT4N1M1.Interventions And OutcomesA regimen of doxorubicin and ifosfamide was administered therapeutically, resulting in a stable disease state.LessonsThe rarity and tumor origin challenge the diagnosis, which emphasizes the imperative role of histological examination, immunohistochemistry, and flow cytometry in achieving an accurate diagnosis. This report summarizes the existing publications to provide a comprehensive overview of PSC, including its clinical manifestations, radiographic imaging, pathologic features, diagnostic challenges, treatment strategies, and prognosis, and aims to improve the understanding of PSC.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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