Key result
Bronchoscopic resection achieves local control and 38-month survival in rare endobronchial pulmonary sarcoma.
Why the study?
Primary pulmonary undifferentiated pleomorphic sarcoma is an extremely rare, highly malignant tumor lacking specific differentiation markers, requiring thorough exclusion of other tumors for diagnosis.
Case Report (n=56)
Palliative bronchoscopic resection may offer sustained local control and improved survival for patients with PPUPS presenting as an endobronchial mass who are ineligible for radical surgery.
May support bronchoscopic resection in inoperable endobronchial PPUPS; hypothesis-generating challenge to surgical resection as sole option.
Rationale: Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) is a highly malignant tumor with extremely rare occurrence and is known for its distinct cellular pleomorphism. This tumor lacks specific differentiation markers. Consequently, PPUPS diagnosis requires thorough exclusion of other tumors through a comprehensive assessment of clinical, histopathological, and radiological outcomes. Patient concerns: A male patient of age 78 years presented to the hospital showing symptoms of continuous cough and blood-stained sputum for 1 month. Chest computed tomography detected a massive solid mass in the right lower lobe, stretching toward the right main bronchus. An endobronchial tumor blocking the right main bronchus was detected by bronchoscopy. We then conducted comprehensive histopathological and immunohistochemical analyses to elucidate the tumor's characteristics. Diagnosis: On the basis of the findings of immunohistochemical and histopathological evaluations conducted for the specimen collected during bronchoscopic resection, the diagnosis of PPUPS was confirmed after other metastatic diseases and sarcomas were ruled out. Interventions: The patient declined further surgical treatment, radiotherapy, or chemotherapy, citing personal reasons and concerns. Outcomes: Owing to the increased surgical risk and the considerably reduced capacity of the patient's lung, clinicians avoided conducting radical surgical resection; this decision was made after careful evaluation by multidisciplinary experts from the fields of respiratory medicine, anesthesiology, and thoracic surgery and consultation with the patient's family. The patient survived for 38 months postoperation. Lessons: PPUPS can develop as a solitary endobronchial mass, appearing almost identical to primary bronchogenic carcinoma. For patients considered ineligible to receive radical surgery intervention, survival may be enhanced by promoting sustained local control through minimally invasive bronchoscopic resection. This approach confronts the established belief that the sole viable treatment option is aggressive surgical resection. Accurate diagnosis of PPUPS relies on high clinical suspicion and careful elimination of other sarcomas on the basis of immunohistochemical analysis findings.
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Wei et al. (2026) conducted a case report in Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS) (n=56). Surgical resection (including minimally invasive bronchoscopic resection) vs. No surgery or incomplete resection was evaluated on Overall survival. Minimally invasive bronchoscopic resection provided sustained local control and 38-month survival in a high-risk patient with endobronchial primary pulmonary undifferentiated pleomorphic sarcoma, while literature review confirmed total surgical resection as a critical determinant of cure.
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