Diffuse embryoma of the testis is a very rare, distinct form of mixed germ cell tumor. I report here on a case of diffuse embryoma in a 22-year-old male who presented with painful scrotal swelling. The resected testis was entirely occupied by a non-encapsulated tumor mass. The cut surface of the tumor was grey or whitish pink, soft and granular with foci of hemorrhage and necrosis. Microscopically, the tumor was characterized by a diffuse, orderly arrangement of embryonal carcinoma and yolk sac tumor in almost equal proportions.
The yolk sac tumor component was diffusely wrapped around the embryonal carcinoma. Syncytiotrophoblasts were scattered throughout the tumor. Minor foci of immature teratoma, seminoma and intratubular germ cell neoplasia were observed.
The yolk sac tumor (YST) component was emphasized by immunoreactivity for alpha fetoprotein, whereas the embryonal carcinoma was reactive for CD30. The strong reactivity for cytokeratin in the YST component formed an outstanding contrast to the weak cytokeratin reactivity in the embryonal carcinoma.
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.
Primary mixed germ cell tumor is a very rare tumor in the cranial cavity. It is composed of varying combination of germ cell neoplasms, such as germinoma, embryonal carcinoma, choriocarcinoma and teratoma. The authors experienced a case of mixed germ cell tumor developing in the floor of the third ventricle, in a twelve years old boy. He was admitted to the hospital, because of headache, eyeball pain, diplopia and vomiting. Brain CAT scan revealed a round tumor density in the floor of the third ventricle. Tumor marker study, preoperatively checked, revealed beta-HCG, 439.8 mIU/ml and alpha-fetoprotein, under 20 ng/ml. On operation, there was a dark brown firm mass up to 3.0 cm in the third ventricle, that invaded into the foramen Monro. With microscopic examination, this tumor is composed chiefly of germinoma, with embryonal carcinomatous region, choriocarcinomatous region and focal mature teratomatous region. The authors diagnosed this case as mixed germ cell tumor.