Isolated pulmonary epithelioid hemangioendothelioma: A case report

RATIONALE: Pulmonary epithelioid hemangioendothelioma (P-EHE), initially named "intravascular bronchoalveolar tumor," is an exceptionally rare malignant tumor with an incidence of <1 in a million. Diagnosis depends primarily on pathological and immunohistochemical findings, and currently, there is no established treatment standard. PATIENT CONCERNS: A 50-year-old woman had a physical examination that revealed a lung shadow, followed by a cough and sputum. A chest computed tomography (CT) scan displayed a mass shadow in the upper lobe of the left lung. 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT indicated increased uptake of a clumped shadow. DIAGNOSIS: The patient underwent a wedge resection of the left upper lung and pleural biopsy, confirming P-EHE. INTERVENTIONS: Following P-EHE diagnosis, chemotherapy, targeted therapy, and immunosuppressive therapy are administered. OUTCOMES: The patient and his family gave up the treatment because it was not satisfactory. Later, we conducted a phone follow-up and learned that the patient passed away on May 18, 2023. LESSONS: The scarcity of P-EHE complicates its diagnosis and treatment, underscoring the importance of ongoing research to enhance our understanding of this condition.