Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls

Abstract

In 1973, a 10 year old boy presented with numerous bilateral lung nodules, diagnosed as histiocytosis X by open lung biopsy. The patient was treated with prednisone until 1984. In 1993, he developed severe pain in the neck. A biopsy of the spine revealed the same tumour morphology as was seen in the lung in 1973. Immunohistological examination of the former and present biopsy led to the definitive diagnosis of epithelioid haemangioendothelioma of the lung with metastases to spine and liver. Epithelioid haemangioendothelioma of the lung is a rare soft tissue tumour of vascular origin, readily mistaken for carcinoma or, as in this case, histiocytosis. The tumour has an intermediate malignant potential. Although metastases of epithelioid haemangioendothelioma of the lung are well-known, metastatic spread to bones, as in our case, has not previously been mentioned in the literature.

Primary pulmonary epithelioid hemangioendothelioma

“…Chemotherapy is preferred in disseminated or unresectable cases 20. Radiotherapy had been proven ineffective 21. Other options include antiangiogenetic drugs, immunostimulants, lung transplantation, recombinant human endostatin and mammalian target of rapamycin inhibitors 22–27…”