Lixin Guo

Rationale: Pulmonary 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 concerns: This 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. Diagnosis: Histopathological examination indicated the presence of a malignant neoplasm associated with necrosis suggestive of sarcoma, which was pathologically staged as cT4N1M1. Interventions and outcomes: A regimen of doxorubicin and ifosfamide was administered therapeutically, resulting in a stable disease state. Lessons: The 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.