Epithelioid haemangioendothelioma and paraneoplastic hypercorticism

S. El Mahou, B. Jamard, A. Constantin, A. Cantagrel, B. Mazieres and M. Laroche

CHU RANGUEIL, Service de Rhumatologie Professeur Mazieres, 1 avenue Jean Poulhes, Toulouse 31059, France.

Correspondence to: S. El Mahou. E-mail: elmahousoumaya2003{at}yahoo.fr

SIR, A 24-yr-old man was followed for 13 yr for Cushing's syndrome. He had two hypophysectomies (the first was partial and the second complete). Histological examination did not confirm an adenoma and the surgery did not bring about a decrease of secretion of adrenocorticotrophic hormone (ACTH). The outcome extension was negative. Bilateral adrenal gland surgery did not improve the Cushing's syndrome nor the ACTH secretion.

In 2002, after a small trauma, the patient presented with violent pain in the right leg, mainly in the ankle. On examination he was found to have oedema of the right calf. The remainder of the physical examination was normal except for the Cushing's syndrome which was already known. Right ankle radiographs showed a radiolucent area with a well-defined rim of bone. Magnetic resonance imaging (Fig. 1) showed multiple areas of bone involvement with increased intensity on T2-weighted images; there were the same lesions in muscle. Biopsies of the bone and muscle lesions confirmed the diagnosis of epithelioid haemangioendothelioma (HE), characterized by the presence of epithelioid and histolytic endothelial cells with primitive vascular differentiation. The tumor was surgically removed and the patient treated with radiation therapy. Excision of the tumour mass permitted prompt biochemical remission of hypercortisolism and normalization of ACTH secretion for the first time for more than 13 yr. The clinical presentation and biological evolution favoured a diagnosis of paraneoplastic Cushing's syndrome.



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FIG. 1. Ankle RMN: multiple areas of bone involvement.

 
HE is a rare malignant vascular neoplasm described in soft tissue and bone. It seems to have a predilection for males and to affect all age groups, but mainly patients in the second and third decade. The most frequent symptoms are pain and fracture. Multicentric involvement has been reported in 22 to 64% of patients.

Paraneoplastic Cushing's syndrome is also a rare entity; it results from ectopic corticolipotrophic secretion. In some cases the primary tumour presents late. Some studies have demonstrated the synthesis of corticotrophin-releasing factor in non-neuroendocrine tissues.

The authors have declared no conflicts of interest.

Accepted 6 July 2004





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