Epithelioid haemangioendothelioma of the lung: clinical and pathological pitfalls

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...

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Bibliographic Details
Published in:The European respiratory journal Vol. 8; no. 9; pp. 1616 - 1619
Main Authors: van Kasteren, ME, van der Wurff, AA, Palmen, FM, Dolman, A, Misere, JF
Format: Journal Article
Language:English
Published: Leeds Eur Respiratory Soc 01-09-1995
Maney
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Summary: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.
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ISSN:0903-1936
1399-3003
DOI:10.1183/09031936.95.08091616