R6. Hemangiopericitomul membrelor - tumora vasculara asemanatoare sarcomului Kaposi
Hemangiopericitomul, tumora vasculara descrisa in 1942 de Storil si Cabanne, se poate dezvolta oriunde exista vase capilare. Apare sub forma de tumori eritemo-violacee, initial unice, apoi multiple, localizate de obicei la nivelul membrelor si in special la cele inferioare, cu dispozitie cel mai frecvent unilaterala. Intr-o perioada de 45 ani am intalnit 24 pacienti cu hemangiopericitom (20 de sex masculin si 4 de sex feminin), dintre care 19 cu leziuni la nivelul membrelor, cel mai frecvent la cele inferioare (14 cazuri). Leziunile s-au situat unilateral in 15 cazuri si bilateral in 4 cazuri. Alte localizari au fost reprezentate de extremitatea cefalica - 4 cazuri (regiunea fronto-parietala, occipitala, fronto-temporala si retroarticulara) si de penis - 1 caz. Desi boala este mai frecventa dupa 50 de ani, varsta pacientilor studiati s-a situat intre 18-40 ani. Unii pacienti au dezvoltat metastaze: loco-regionale, ganglionare, pulmonare, cerebrale. Din punct de vedere clinic, leziunile au pus probleme de diagnostic diferential cu forma tumorala a sarcomului Kaposi, melanomul, granulomul piogen. Diagnosticul a fost confirmat de examenul histologic: hemangiopericitomul este caracterizat prin proliferare de celule fusiforme ce inconjura capilare dilatate, deseori asociata cu atipii celulare.
R6. Hemangiopericytoma of the Limbs - Vascular Tumor Resembling Kaposi's Sarcoma
Hemangiopericytoma described in 1942 by Storil and Cabanne can develop anywhere capillary vessels exist. It appears as erythemato-violaceous, initially unique, then multiple tumors, usually localized on the limbs, especially on the lower limbs, mostly with unilateral involvement. During 45 years we have examined 24 patients with hemangiopericytoma (20 male, 4 female), 19 of them presenting lesions localized over the extremities, most frequently over the lower extremities - 14 cases. Unilateral involvement has been noted in 15 cases, but there were also simetric lesions in 4 cases. Other sites were the head - 4 cases (fronto-parietal, occipital, fronto-temporal and retroauricular region) and the penis - 1 case. The disease appears most frequently after the age of 50, however we have examined patients between 18-40 years old. Some patients developped metastases: we have noted loco-regional, lymphonodal, pulmonary and cerebral metastases. Clinically, the lesions resembled mostly the tumoral type of the Kaposi's sarcoma, the melanoma and the pyogenic granuloma. The diagnosis was established by histological examination which showed the characteristic spindle cells proliferation surrounding dilated capillaries, often associated with atypical cells.