Abstract
BACKGROUND: Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare vascular tumor derived from mesenchymal cells with an incidence of about 1/1 million. The etiology remains unclear, and there are no established treatment guidelines. The tumor can occur in a variety of organs, among which the liver, lung and bone are the most commonly involved, with different clinical manifestations, mainly depending on the organ involved, but none of them is specific. Most epithelioid hemangioendotheliomas are indolent and can achieve long-term survival even without any treatment. The prognosis of patients with primary liver or lung diseases is poor, and P-EHE has the highest mortality rate. Clinical reports of airway stenosis caused by P-EHE are exceedingly rare. CASE DESCRIPTION: We report a case of a middle-aged female patient with initial diagnostic difficulties leading to misdiagnosis, which was eventually confirmed as P-EHE by pathology. Despite radiotherapy, chemotherapy, and antiangiogenic therapy, the efficacy was limited, resulting in severe airway stenosis in the advanced stage of the disease, with severe clinical symptoms. After bronchoscopic cryoablation, the oxygenation index of the patient was significantly improved, and the quality of life was significantly improved. CONCLUSIONS: This case underscores the complexity of diagnosing and managing P-EHE and illustrates the potential of cryoablation as an effective treatment for severe airway stenosis secondary to this rare tumor.