[Sentinel lymph nodes identification in vulvar cancer--methods and technique]. / Detekce sentinelových uzlin u karcinomu vulvy--metody a technika.

OBJECTIVE: To evaluate detection of sentinel lymph nodes (SLN) in squamous vulvar cancer with blue dye and 99mTc. The study describes technique of application, timing, management, detection rate (DR), specific side detection rate (SSDR) and false negative rate. DESIGN: Prospective clinical study. SETTING: Department of Obstetrics and Gynecology, Charles University Prague, 2nd Medical Faculty, Teaching Hospital Motol. PATIENTS AND METHODS: 46 women with squamous cell carcinoma tumors stage I or II, <4 cm with no clinical suspect lymph nodes were included. Blue dye alone was used in 16 women and the combination of 99mTc and blue dye was used in 30 women. Radiocoloid 99mTc was applied 3-5 hours and blue dye 3-5 minutes prior to ingvinal incision. RESULTS: We detected 88 SLN in 61 inguinal spaces. The detection rate in the blue dye group was 68.8 % (11 cases). One false negative SLN (6.3 %) appeared in this group. In blue dye+ 99mTc group detection rate was 100 % with no false negative SLN. CONCLUSION: Detection of SLN in squamous vulvar cancer with the combination of 99mTc and blue dye was statistically significantly more effective than using the blue dye alone.

[Hormonal suppression and sexual development in children with central precocious puberty in the first treatment cycle 12 weeks after injection of triptoreline 11.25 mg (Diphereline S. R. 11.25 mg): a pilot study]. / Hormonální suprese a pohlavní vývoj detí s centrální predcasnou pubertou v prvním lécebném cyklu 12 týdnu po injekci triptorelinu 11.25 mg (Diphereline S. R. 11.25 mg): pilotní studie.

OBJECTIVE: To evaluate hormonal suppression and pubertal development in children with central precocious puberty (CPP) after injection of triptoreline 11.25 mg (Diphereline S. R. 11.25 mg; Ipsen) in the first treatment cycle of 12 weeks. DESIGN: Pilot study. SETTING: Paediatric department, University Hospital Motol-Prague. METHODS: Serum levels of FSHmax and LHmax and basal levels of estradiol/testosterone were monitored in GnRH test before, 4, 8 and 12 weeks after triptoreline 11.25 mg injection in 3 girls and 2 boys with CPP (age 3.9-10.6 years) previously treated by triptoreline 3 mg every 4 weeks. Uterine and ovarian volume, hormonal cytology (vaginal smear), breast development and testicular volume were evaluated before and 12 weeks after triptoreline 11.25 mg injection. RESULTS: 8 weeks after triptoreline 11.25 mg, FSHmax in girls increased (2.3 IU/l vs. 1.7 IU/l before injection; median) without any other change in 12th week. In boys after initial decrease LHmax 12 weeks after injection rose to 1.7 IU/l (identical as LHmax before injection). Estradiol and testosterone levels were in prepubertal range. Pubertal development in girls did not progress, and testicular volume decreased in both boys (treated for CPP 0.3 and 0.7 years). CONCLUSIONS: Triptoreline 11.25 mg injection in 12 weeks interval can be considered as effective, useful and safe for therapy of CPP. The long-term follow-up will be necessary.

[Mixed germ cell tumours of the ovary in childhood and adolescence]. / Smísené germinální nádory ovaria u detí a dospívajících. Retrospektivní klinicko-patologická studie.

Mixed germ cell tumours of the ovary are rare malignant neoplasms containing combinations of two or more types of germ cell elements. The aim of the study was to review biopsy examinations, medical records, treatment strategy, follow-up and outcome of all girls treated for mixed germ cell tumour of the ovary at the Department of Pediatric Oncology, University Hospital Motol during the period 1979-2002. Archival slides of all tumours were reviewed and tumours were classified according to the WHO system. The clinical data on surgical treatment, chemotherapy and radiotherapy used and follow-up information were obtained in all girls. The staging was reviewed retrospectively on the basis of surgical and pathological findings and results of imaging investigations, and it was outlined according to the most recent FIGO criteria and TNM classification. Sixteen girls with mixed germ cell tumour of the ovary, age range 3 years 11 months to 17 years 8 months (median 12 years) were treated. All girls presented with unilateral tumour of the ovary and all underwent surgery as an initial treatment. The most common presenting symptom was abdominal pain, occurring in ten patients. The original diagnosis of mixed histology was confirmed in all cases; in five cases the tumour contained three histologic components, in eleven cases the tumour consisted of two germ cell types. All tumours contained elements of yolk sac tumour, followed by immature teratoma, embryonal carcinoma, dysgerminoma and mature teratoma. At the time of diagnosis three patients had stage I disease, four patients stage II, seven stage III and two stage IV disease. All patients were treated with chemotherapy after surgery, predominantly with platinum-based regimens (PVB, BEP). Three patients treated initially with MAC (metothrexate, dactinomycin, cyclophosphamide) were diagnosed in the early eighties. In seven girls with advanced disease treated in the early years, radiotherapy was administered to the pelvis or whole abdomen. Overall survival and event-free survival were 80% and 81.3% respectively (median follow-up time 7.6 years). Three patients have died from the disease, two progressed on treatment (MAC), one girl relapsed three months after finishing therapy, no further therapy was administered. One girl underwent resection of tumour of her remaining ovary 24 months after original diagnosis. Histology showed mixed serous and mucinous cystadenoma. The latest examinations revealed that all other patients were in good health. Microscopic examination should be extensive and careful to find out all types of malignant germ cell elements. Platinum based chemotherapy is effective in the management of children and adolescents with mixed germ cell tumors of the ovary. Chemosensitivity of these tumours allows most girls to have conservative surgery with possible preservation of reproductive function.

[Ovarian dysgerminoma in children and adolescents. Retrospective clinico-pathologic study]. / Dysgerminomy ovaria u detí a dospívajících. Retrospektivní klinicko-patologická studie.

BACKGROUND: To review the treatment strategy, follow up and outcome for all patients with pure ovarian dysgerminoma treated in childhood and adolescence. METHODS AND RESULTS: Twenty-one patients younger than 18 years were treated between 1979-2002 in Faculty Hospital Motol for newly diagnosed pure ovarian dysgerminoma. Patients were included into the cohort on the basis of revision of archival biopsy specimens deposited in Institute of Pathology and in Molecular Medicine tumor registry. The staging was reviewed retrospectively on the basis of surgical and pathological findings and on results of imaging investigations and outlined according to the TNM and International Federation of Gynecology and Obstetrics (FIGO) classification. The median age at the time of diagnosis was 12.5 years (range 6 years, 5 months--17 years, 11 months). There were ten FIGO stage IA tumors, one stage IB, two of stage IIC, one stage IIIB and seven IIIC tumors. All patients, except two girls with bilateral dysgerminoma, underwent unilateral adnexectomy or ovarectomy. Ten girls were treated postoperatively with chemotherapy, eight with chemotherapy and radiotherapy (eleven with cisplatine based chemotherapy). Three girls with dysgerminoma confined to the ovary (stage IA) have not received adjuvant chemotherapy. With a median follow up 7.1 years all girls remained continuously disease free. The 5-year overall and event free survival is 100%. Majority of patients does not have severe treatment sequelae, three pregnancies have occurred so far. CONCLUSIONS: Most patients with dysgerminoma, including those with metastases can expect cure when treated with conservative surgery and cisplatine based chemotherapy. Reduction of treatment toxicity and preservation of reproductive function is a main task. The girls with dysgerminoma confined to the ovary (stage IA) can be treated with fertility sparing surgery, other should be treated with cisplatine based chemotherapy.

[Growth and development in untreated girls with slowly progressing idiopathic central or borderline-onset puberty]. / Rust a vývoj nelécených dívek s pomalou progresí idiopatické centrální predcasné nebo hranicne nastupující puberty.

OBJECTIVE: To analyse growth and development of girls with slowly progressive idiopathic precocious or early puberty. DESIGN: Long-term open clinical study. SETTING: Department of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University, Prague. METHODS: In 20 untreated girls with slowly progressive puberty starting at 6-9 years neurogenic aetiology was excluded. During follow-up period 4.7 +/- 2.2 (2-8.5) years (mean +/- SD; range), sexual development (Tanner criteria), age at menarche, menstrual cycle and auxological parameters were evaluated. RESULTS: 13 girls reached menarche at 11.1 +/- 0.9 years (3.7 +/- 1.1 years after the onset of puberty), earlier than in their mothers (12.9 +/- 1.1 years) and Czech standards (P < 0.05). Menstrual cycle 28 (24-29) days was regular in all 6 girls with gynaecological age > 2 years. In one girl microprolactinoma was diagnosed, therapy with bromocryptine started at the age 14.7 years (3.5 years after menarche). At the onset of follow-up, bone age (TW20) advancement was 1.8 +/- 1.4 years above the chronologic age. Initial prediction of final height (graphic method) was 162.3 +/- 5.5 cm vs final prediction 163.7 +/- 5.1 cm. Final height 162.2 +/- 5.7 cm achieved 7 girls vs target height 163.6 +/- 5.2 cm (NS). CONCLUSION: In untreated girls, menarche occurred later after the first signs of puberty than in normal population, menstrual cycle was regular. Height potential was preserved, final height corresponded with their target height. Not all girls with early and slowly progressive puberty should be treated. Therapy is necessary in organic aetiology, rapid progressive precocious puberty and impaired growth prognosis.

[Turner syndrome: overview of problems, present status, proposals for care and a protocol for monitoring in childhood, adolescence and adulthood]. / Turneruv syndrom: prehled problematiky, soucasný stav, návrh koncepce péce a protokol sledování v detství, adolescenci a v dospelosti.

BACKGROUND: Girls and adolescents with Turner syndrome (TS) usually receive intensive medical care in a multidisciplinary team, coordinated by paediatric endocrinologist. Majority of them are discharged from specialist clinics following the induction of puberty and attainment of final height. Patients with Turner syndrome have a reduced life expectancy, they are known to have multi-system impairments in addition to the short stature and to the absence of sexual development. Aim of this study is to propos a continuous follow-up by multidisciplinary team of physicians starting in childhood and following the discharge from the paediatric care. METHODS AND RESULTS: This paper highlights the medical and psychosocial problems associated with Turner syndrome in childhood, adolescence and in adulthood. Analysis of these problems served as a background to management strategy. CONCLUSIONS: Women with Turner syndrome are at risk of number of medical problems. Quality of their life and the life expectancy can be improved with increasing awareness to comorbities associated with Turner syndrome. Assisted reproduction technologies has recently offered a chance for pregnancy and delivery of a healthy child also to women with Turner syndrome. Therefore, long-term follow-up by multidisciplinary team of physicians knowledgeable about these medical problems is necessary. Introduction of a centralised system of systematic multidisciplinary approach to patients with Turner syndrome from childhood and adolescence to adulthood seems to be very important.

[Laparoscopic neovaginoplasty--a modification of the Vecchietti operation]. / Laparoskopická neoplastika pochvy--modifikace Vecchiettiho operace.

OBJECTIVE: To asses laparoscopic approach to neovaginoplasty. DESIGN: Retrospective clinical study. SETTING: Department of Gynecology and Obstetrics, 2nd Medical Faculty of Charles University. METHODS: Vecchietti's neovaginoplasty is considered to be the most advantageous technique of creating neovagina in vaginal agenesis, because of low perioperational morbidity and quicker recovery. Authors describe own modification of laparoscopic procedure based on designed equipment of themselves. RESULTS: Study evaluates 17 cases of laparoscopic procedures, which are compared with classical laparotomic approach we were experienced in past. Time of surgery was shorter (48 min vs. 66 min) and blood loss was lower (15 ml vs. 50-100 ml) in laparoscopic procedure. Number of complications was lower in this procedure too. There was no difference in length of hospital stay and size of neovagina. CONCLUSION: Laparoscopic modification is safe procedure and comparable with classic operation as regards surgical effect. Both types of procedures are determined for workplaces with experience in special reconstructive surgery and follow-up.

[Effect of estrogens on final body height in girls with a constitutionally tall stature--risk or benefit?]. / Ovlivnení definitivní telesné výsky estrogeny u dívek s konstitucne vysokým vzrustem--rizika nebo prínos?

OBJECTIVE: Investigation of the effect of a long-term oestrogen treatment on the growth and development of children, elaboration of a theoretical model for remaining growth of girls on a long-term oestrogen therapy predicting reduction of the final height in girls with constitutionally tall stature, analysis of early and late side-effects of therapy. TYPE OF STUDY: Open clinical study. NAME AND PLACE OF DEPARTMENT: Endocrine out-patient clinic of the Second Paediatric Department and gynaecological out-patient clinic for children and adolescents of the Gynaecological-Obstetric Department, Second Medical School--Charles University and University Hospital Prague--Motol. METHOD: Elaboration of theoretical model for remaining growth of girls on a long-term oestrogen therapy was based on an analysis of the remaining growth of upper and lower body segment of healthy Czech girls. RESULTS: The extent of assumed reduction of the final height in girls with constitutionally tall stature treated with oestrogen declines with advancing age at the onset of treatment. The optimal age for the onset of treatment is between 10 and 11 years. CONCLUSION: Treatment can be recommended only in girls with growth prediction above 185 cm and serious psychological difficulties resulting from excessive height, always after careful analysis of the biological development and growth prediction. In every case it is necessary to evaluate the possible benefit of treatment in relation to potential risks. The greatest problem is according to the authors late onset and overrating of the therapeutic possibilities.

[Superficial epithelial-stromal ovarian tumors in girls]. / Povrchové epiteliálne-stromální nádory ovaria u dívek.

Superficial epithelial-stromal ovarian tumours are unusual in adolescent girls (when compared with adult women) and extremely rare before menarche. A group of 40 girls with such tumours was chosen among 180 cases (22%) of ovarian tumours in childhood and adolescent age. All of them were between 9 and 18 years with one exception of a 5-year old girl. Mucinous tumours (22 cases) prevailed a bit over the serous ones (16 cases), two tumours were seromucinous. 35 tumours were one sided, 5 bilateral belonged to serous tumours. One sided tumours comprised 5 cystadenomas, 5 cystadenofibromas and 1 borderline serous tumour. Bilateral neoplasms were represented by 3 serous cystadenofibromas, 1 borderline serous tumour and 1 serous cystadenocarcinoma. Mucinous tumours comprised 16 benign cystadenomas, 4 borderline intestinal type tumours (one with identifiable eosinophilic indifferent cells) and 2 mucinous intestinal type cystadenocarcinomas. Both mixed tumours had the structure of seromucinous cystadenoma.