[Hypertrophic pulmonary osteo-arthropathy revealed by a pulmonary epithelioid hemangioendothelioma in a 15-year-old girl].
Pulmonary epithelioid hemangioendothelioma is a rare vascular tumor of the lung, that may be revealed by hypertrophic pulmonary osteo-arthropathy.


METHODS
A 15-year-old patient was admitted because of a 1-month history of pneumonia associated with clubbing. A hypertrophic pulmonary osteo-arthropathy due to a paraneoplastic syndrome associated with lung epithelioid hemangioendothelioma was diagnosed. Despite surgical resection and chemotherapy, the patient died 6 months later.


CONCLUSIONS
Hypertrophic pulmonary osteo-arthropathy is a rare syndrome in childhood, and is frequently associated with chronic respiratory failure. Diagnosis is confirmed by long bones proliferative periostitis on standard X-ray examination. Pulmonary epithelioid hemangioendothelioma is a rare vascular malignant tumor, that spreads from the vascular pulmonary or hepatic tissues. The prognosis is variable, depending on the malignancy of the tumor. In the absence of a chronic cardiorespiratory disease, the diagnosis of a pulmonary hypertrophic osteo-arthropathy in childhood should prompt the search for a tumoral cause.
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Clinique de pédiatrie, unité de pneumologie, hôpital Jeanne-de-Flandre, CHRU de Lille, avenue Oscar-Lambret, 59037 Lille cedex, France.
Barbier
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Thumerelle
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Santos
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Desfachelles
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Devisme
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Deschildre
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D000293:Adolescent; D003937:Diagnosis, Differential; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000175:diagnosis*; D010004:Osteoarthropathy, Primary Hypertrophic / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology