[Juvenile granulosa cell tumor with subsequent occurrence of gastrointestinal polyposis, subcutaneous lipomatosis and nodular goiter]. / Juvenilní nádor z bunek granulózy s následným výskytem polypózy zazívacího traktu, subkutánní lipomatózy a nodózí strumy.

A juvenile tumour from granulosa cells (M-8622/1), 13 x 8 x 6 cm, in the right ovary in a three-month-old girl produced some symptoms of pseudopubertas praecox isosexualis which disappeared after operation. Microscopic examination of the tumour revealed in addition to typical structures a less common differentiation to Sertoli cells. Despite actinotherapy and chemotherapy one and a half years after the onset of the disease X-ray examination revealed metastases in the lungs which were successfully cured by further doses of the above two types of treatment. Between the age of 6 and 15 years the girl developed successively polyposis of the stomach, small and large intestine (M-7564/0), subcutaneous lipomatosis of the trunk and left lower extremity (M-8881/0) and nodular goitre (M-7164/0), predominantly quiescent. In the literature a connection between gonadal stromal ovarian tumours and mesenchymal tumours, intestinal polyposis and disorders of the thyroid gland is described, but in different patients. The authors' observation is unique by the successive incidence of these changes in a single patient surviving 15 years after operation; and thus genetically conditioned associations could be involved.

[An ovarian tumor with structural gonadoblastoma, dysgerminoma and choriocarcinoma]. / Ovariální nádor se strukturami gonadoblastomu, dysgerminomu a choriokarcinomu.

A 14 1/2-year old girl with menometrorrhagia followed by amenorrhea and abdominal pain had a pelvic resistance with limited mobility. Histology of a left ovarian tumour showed gonadoblastoma turning to dysgerminoma and associated with choriocarcinoma (M-9073/1, M-9060/3, M-90101/3). Genuine ovarian tissue was hypoplastic on both sides. In the right ovary, there were additional thecalutein cysts. The patient's karyotype was 46 XX. She has been without any relapse 2 1/2 years after chemotherapy and actinotherapy.

Mixed germ cell-sex cord stromal tumor with heterologous structures.

A mixed germ cell-sex cord stromal tumor developed in the right ovary of a 4-year-old girl. The patient's cells contained sex chromatin and the karyotype was 46,XX. Clinically, she exhibited mild isosexual pseudopuberty. Some of the tumor cells showed differentiation to Sertoli cells, whereas others had characteristics of germ cells. The neoplasm was composed of solid cords consisting of a mixture of the two tumor cell types and surrounded by a delicate connective-tissue network. In one small area, the tumor contained heterologous development in the form of glands and cysts lined by columnar mucinous epithelium containing numerous goblet cells and occasional argyrophilic neuroendocrine cells. Normal ovarian tissue was present at the periphery of the tumor. Electron microscopic study confirmed the presence of both Sertoli-like and germ cell-type cells. Immunohistochemical studies demonstrated vimentin positivity in both cell types (with preference for the Sertoli-like cells) and cytokeratin positivity in the Sertoli-like cells. The patient was symptom free 4 years after right oophorectomy, radiotherapy, and chemotherapy.

Recidivous offence in sadistic homosexual pedophile with karyotype 48, XXXY after testicular pulpectomy. A case report.

The case of recidivous sexual offender with genetically caused mental retardation and primary hypogonadism (Klinefelter's syndrome with karyotype 48, XXXY) is described. He was examined after sadistic abuse of a boy aged 13 that he had committed 19 years after performed testicular pulpectomy. Plasmatic level of testosterone was found 4x higher than mean level in men after orchidectomy. Histological examination of residual scrotal tissues proved that the source of androgens were hyperplastic nodules of extratesticular Leydig cells.

[Fertility in gonadal dysgenesis]. / Fertilita u gonadální dysgeneze.

At the Second Gynaecological and Obstetric Clinic in Prague there are at present 78 phenotypic women with dysgenetic gonads in dispensary care. In 61.5% the main reason for examination in a specialized department was infertility. Based on a comprehensive examination, the authors demonstrate the relationship between chromosomal complement and histology of the gonads on one side and fertility on the other. 31% of women with dysgenetic sclerocystic gonads became pregnant after surgical and hormonal treatment. In 27% of the dispensarized group genital and extragenital tumours were detected.

[The relation of nucleoli to precancerous conditions of the cervix]. / Vztah nukleolu k prekancerózám cervixu.

Oestrogens act at the nuclear level. To these steroids also a cancerogenic effect is ascribed. By staining for nuclear structures according to Smetana and Busch the authors proved morphological structures of the nucleolus in vaginal epithelia and in cells from cervical lesions. This applies to solid nucleoli -- which are active, annular nucleoli which are resting and nucleoli which have completed their activity -- micronucleoli. Hyperoestrogenic women have in their vaginal epithelia of the upper third of the vagina more active nucleoli (11.4%) than hypooestrogenic women (2.1%). Women suffering from cervical carcinoma during the menopause have more active nucleoli in the vaginal epithelia (30.8%), as compared with healthy hyperoestrogenic women of productive age. Patients during the menopause with grade III dysplasias have fewer active nucleoli in cervical epithelia than women with cervical cancer (25.0%), however, as compared with hyperoestrogenic women of productive age, much more. A large number of active nucleoli was proved by staining by Smetana's method also in cells taken from cervical lesions carcinoma type and grade III dysplasia in postmenopausal patients. Evaluation of morphological changes of the nucleoli suggests their activity, the activity of the whole nucleolus and is a sensitive indicator of the activity of the whole cell.

[Juvenile granulosa cell tumor]. / Juvenilní nádor z bunek granulózy.

Granulosa cell juvenile tumours, defined in 1976 by Scully as a separate unit are found mostly in girls during the first two decades of life. On inspection under an optic microscope they are characterized by the presence of non-differentiated "blastemoid" structures and the formation of solid follicular formations and cysts. In our first case we observed a bilateral juvenile tumour made up of granulosa cells in a 8-month-old girl where the tumour produced symptoms of pseudopubertas praecox isosexualis. In the second case in a 9-month-old girl also with symptoms of pseudopubertas praecox isosexualis the authors detected in the left ovary a tumour which probably is a less differentiated variety of the juvenile granulosa cell tumour. These tumours belong into the group of little differentiated gonadal-stromal tumours with ICD-O code M-8590/1.

Effect of etomidate on adrenal function in rats.

Etomidate, an imidazole derived hypnotic was applied to rats in order to find out whether repeated treatment with the drug, in doses exceeding those usually given to human, does alter adrenal morphology and function in terms of corticosterone and 11 beta-hydroxyandrostenedione (an androgen of exclusively adrenal origin) production, both in vivo and in vitro. A slight, dose-related decrease of plasma corticosterone and 11 beta-hydroxyandrostenedione was observed from etomidate concentrations 0.3 resp. 1.4 mg kg-1 body weight, respectively. In vitro, corticosterone production was reduced in the groups receiving 0.7 mg of the drug or more. Morphologically, only minute lipid hyperplasia was found after the highest dose of etomidate, associated with a significant increase of relative body weight. Administration of low dose of etomidate (approx. one half of that given usually to human) led to an increase of corticosterone production, indicating the reversibility of such effect. Etomidate did not stimulate enzymic oxidation of 11 beta-hydroxysteroids to the corresponding 11-oxosteroids, as do some blockers of 11 beta-hydroxylase.

[Classification of germinal tumors of the testis]. / Klasifikace germinálních nádoru varlete.

Based on the collection of more than 500 personal as well as consiliary cases of TGCT and a thorough review of the existing classifications, the essential synonyms used for designating TGCT entities have been subjected to a critical reappraisal. The tumour entities have been defined exactly to facilitate their coding according to the ICD-O system. The existing classification system have been subjected to a critical reappraisal and compared one to another as well as to our own classification as suggested by ourselves in 1974 and used since then. A detailed discussion and exact coding of well-defined entities is believed to permit an almost complete interconvertibility of the most significant classifications with that of ours and thus to form a reliable basis for amy form of comparison, including the therapeutic results. The bioptic examination of surgical specimens obtained by orchiectomy (other samples are regarded unsuitable) must be carried out in a standardized manner. This should permit not only the usual typing of the respective tumour but also the data necessary for proper tumour staging which today appears to be a natural prerequisite for rational treatment and its monitoring. The main principles of bioptic examination of testicular tumours have been summarized in brief.

The significance of vaginal hormonal cytology and the leukocyte adherence inhibition test for the detection of malignancy.

Raised leukocyte adherence inhibition (LAI) test values were found for 25 women with endometrial carcinoma and 12 women with benign hyperplastic endometrium. Of 42 patients with oligomenorrheic and polymenorrheic disturbances of the menstrual cycle and whose hyperestrogenism was assessed by cytologic indices (i.e., eosinophilic and karyopyknotic indices), 38 exhibited positive or borderline LAI test values with either one or both of the antigens used. The LAI test was negative with both antigens in four women with hyperestrogenism associated with a 28-day menstrual cycle. The high correlation between hyperestrogenism and positive LAI test results as well as between the LAI test and the presence of tumors suggests that both hormonal cytology and the LAI test might be useful as screening methods for evaluating risk factors for the development of malignant tumors of the female reproductive tract. Both methods are fairly simple, reproducible and convenient for long-term follow-up.