Case report: pulmonary epithelioid haemangioendothelioma

Abstract

The patient was a 65-year-old male, a retired truck driver and chronic smoker with 60 pack year history, who presented with a chronic cough. His past medical history included diabetes, emphysema, and previous occupational exposure to asbestos. On routine CT scan of the chest, an incidental 5–6mm right lower lobe nodule was discovered. Follow up PET scan revealed the nodule was not PET avid. In view of his young age and good functional status, the decision for an excisional wedge biopsy was made, and the tissue was sent for histopathological examination. Macroscopically, the specimen consisted of a wedge of lung measuring 70×40×15mm, and on cut surface, there was a focal 6 mm, pale grey stellate lesion. The lesion was cleared of the stapled margin by 7mm. Microscopically, the lesion was composed of cords and small clusters of neoplastic epithelioid cells with ovoid nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. There was some nuclear pleomorphism, and occasional mitotic figures were noted. Occasional intracytoplasmic lumina containing red blood cells could be identified. The tumour cells were arranged in a perivascular fashion and focally extended to obliterate a number of blood vessels as demonstrated by the elastic stains. The tumour cells were positive for AE1/AE3, CD31, and negative for CK7/20, TTF1, CD45, S100, and Melan-A. The features were consistent with epithelioid haemangioendothelioma. Pulmonary epithelioid haemangioendothelioma, (formerly known as intravascular bronchioalveolar tumour, IVBAT) is an uncommon tumour of vascular endothelial origin, with an intermediate grade between haemangioma and conventional angiosarcoma. The tumour pursues an indolent clinical course; however, extensive pulmonary involvement with systemic metastases has been reported.