RESUMEN
We experienced five cases of solitary fibrous tumor; two in the pleura, two in the orbital soft tissue, and one in the lung parenchyma. Three patients were male, and the age of the patients ranged from 38 to 71 years (mean age: 53.6). Grossly, the masses were well circumscribed and had varying sizes from 2.5 to 30.0 cm. The cut surfaces were grayish-yellow firm with focal variegated hemorrhage, necrosis, cystic change, and myxoid area. Microscopically, these were characterized by a haphazard proliferation of spindle cells or polygonal cells separated by variable amounts of hyalinized collagen and showed a prominent vascular channels reminiscent of hemangiopericytoma in foci. Immunoperoxidase stains showed a strong reactivity for CD34, and were weakly positive for vimentin. Electron microscopical examination revealed features of fibroblast; spindle to round tumor cells were arranged in groups and surrounded by collagen. Nucleoli were seldom prominent. The cytoplasm contained many microfilaments and a moderate number of cisternae of rough endoplasmic reticulum.
Asunto(s)
Humanos , Masculino , Citoesqueleto de Actina , Colágeno , Colorantes , Citoplasma , Retículo Endoplásmico Rugoso , Fibroblastos , Hemangiopericitoma , Hemorragia , Hialina , Pulmón , Necrosis , Órbita , Pleura , Tumores Fibrosos Solitarios , VimentinaRESUMEN
Turner's syndrome results from complete or partial monosomy of the X chromosome and is characterized by hypogonadism or related other congenital anomalies in phenotypic females. In these patients, there are failure to develop normal secondary sex characteristics, amenorrhea, or short stature at puberty and the ovaries are reduced to atrophic fibrous strands devoid of ova and follicles(streak gonads). Individuals with this condition are particularly prone to the development of gonadoblastoma. For this reason, the gonads should be early removed and supplemental estrogen therapy given. We experienced a case of Turner's syndrome, 45, XO/46, XY karyotype in a 20-year-old phenotypic female complained an amenorrhea. On the exploratory laparotomy, the right gonadal mass is sevearly adhered to the adjacent organs and measures 8 x 5 x 5 cm in dimension and 75gm in weight and shows multiple foci of hemorrhage with necrosis. The left streak gonad measures 3.5 x 2 x 1.5 cm in dimension and shows multiple foci of calcification. Microscopically, the right gonadal mass reveals malignant mixed germ cell tumor, composed of endodermal sinus tumor, composed of endodermal sinus tumor with dysgerminoma and gonadoblastoma. The left streak gonad consists of mainly dense fibrous connective tissue and shows some foci of calcification associated with gonadoblastoma. On immunohistochemical and special stainings, the cytoplasm and hyalin droplets of the endodermal sinus tumor component reveal strong positivity to the a-fetoprotein and PAS. After removal of both gonads, the serum level of the a-fetoprotein is markedly down from 1742ng/ml to 2.6 ng/ml.