Primary Pulmonary Epithelioid Hemangioendothelioma: A Case Report and Literature Review

Introduction: Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare vascular tumour of borderline malignancy with a clinical course that lies between a benign hemangioma and an angiosarcoma. The aetiology of P-EHE is still unknown.Method: We report a case of an incidental diagnosis of P-EHE in a 54-year-old female with multiple bilateral nodules discovered after a routine chest x-ray following a 1 month history of cough, dyspnea and persistent fatigue diagnosed with a right wedge resection.Discussion: Clinical presentation is heterogeneous, with the majority of patients being asymptomatic at the time of diagnosis. A diagnosis is usually made after the discovery of abnormal findings in chest x-rays during routine health examinations. To this day, there is no approved standardised treatment protocol for P-EHE. Therapeutic options range from regular follow-ups with no active therapy in asymptomatic patients, to surgical resections, corticosteroids, azathioprine and combination chemotherapy in symptomatic persons. Reports on survival vary greatly. Three separate studies have shown a mean survival of 4.6 years with a range of 6 months to 24 years.Conclusion: P-EHE is a rare tumour that requires further research to correlate its radiological findings to its molecular and genetic characteristics in order to assess their prognostic value and devise standardised treatment protocols.