Current surgical approach: Extracranial malignant germ cell tumors.

Germ cell tumors (GCT) are a complex, heterogeneous collection of tumors that may present in either gonadal or extragonadal sites. They consist of a variety of benign and malignant histologies that can occur at several locations throughout the body. An important component of treatment is surgical resection, and while the key components of resection are site specific, the universal goals of GCT resection include the complete resection of tumor without violating the tumor capsule, while preserving function of surrounding organs, minimizing morbidity, and assessing for regional spread.

The future of paediatric and adolescent gynaecology in Europe.

Paediatric and Adolescent Gynaecology (PAG) is a subspecialty under the umbrella of Obstetrics and Gynaecology but linked to other branches of medicine including Paediatrics, Surgery, Endocrinology and Urology. Therefore future developments in clinical care and education requires a multidisciplinary approach combining aspects of all the above medical specialties, and also with inputs from Public Health, Genetics, Radiology and Psychology. A multidisciplinary collaboration among different specialists is as important as the establishment and adoption of standards in education, training and management. PAG in Europe has evolved from its first steps and it is still growing with the aim of providing increasing protection of the gynaecological and reproductive health of female children and adolescents. In fact, without proper advice and care, inappropriate management of gynaecological issues in childhood and adolescence can be expected to have significant repercussions throughout later years, and into adulthood. The aim of this third paper in this mini-symposium is to explore how PAG should develop in Europe in the near future.

Glutathione S-transferase (GST) polymorphism could be an early marker in the development of polycystic ovary syndrome (PCOS) - an insight from non-obese and non-insulin resistant adolescents.

INTRODUCTION: It has been supposed that endocrine disturbances might be responsible for polycystic ovary syndrome (PCOS)-associated oxida-tive stress, with special emphasis on hyperandrogenism. Considering the potential relationship between hyperandrogenism and increased free radical production, parameters of oxidative stress were determined in non-obese normoinsulinemic adolescent girls newly diagnosed with PCOS. MATERIALS AND METHODS: Nitrotyrosine, thiol group concentrations, glutathione peroxidase, and superoxide dismutase activities were determined under fasting conditions and during oral glucose tolerance test (OGTT) in 35 PCOS patients and 17 controls. Insulin resistance was assessed by the homeostasis model (HOMA-IR), HOMA ß, insulinogenic index (IGI), Matsuda insulin sensitivity index (ISI), and AUC for glucose. Glutathione S-transferases (GSTs) polymorphisms were determined by PCR. RESULTS: Under fasting conditions, no significant difference of oxidative stress parameters was found between PCOS and controls. Acute hyperglycaemia during OGTT induced significant alteration in parameters of oxidative protein damage in PCOS patients. Alteration in nitrotyrosine concentrations correlated with testosterone, DHEAS, androstenediones, FAI, and LH, while changes in thiol groups cor-related with DHEAS. Significant inverse association was found between LH and ISI, as well as AUC glucose and thiol groups. PCOS girls, carriers of GSTM1-null genotype, had significantly lower testosterone in comparison to ones with GSTM1-active genotype. CONCLUSIONS: PCOS girls exhibited high free radical production together with unchanged antioxidant enzymatic capacity, independently from obesity and insulin resistance. Based on associations between oxidative stress parameters and testosterone, DHEAS, and androsten-edione, it can be suggested that increased free radical production, probably as a consequence of hyperandrogenaemia, is an early event in the development of PCOS.

Ovarian Preservation from Tumors and Torsions in Girls: Prospective Diagnostic Study.

STUDY OBJECTIVE: To develop a new decision tree system (DTS) for the management of adnexal masses in prepubertal and adolescent girls, aimed to improve the distinction between benign and malignant masses, help preserve affected ovaries during surgery, and reduce the rate of surgical management of uncomplicated functional ovarian cysts. DESIGN: A prospective diagnostic study using clinical and ultrasound data collected for all patients younger than 19 years of age with adnexal masses managed between 2006 and 2015. SETTING: Mother and Child Health Institute of Serbia 'Dr Vukan Cupic' (Belgrade, Serbia). PARTICIPANTS: Patients (N = 1499) with adnexal masses, of whom 318 were surgically treated. INTERVENTIONS AND MAIN OUTCOME MEASURES: Ultrasonographic characteristics (Ueland's Morphology Index [MI] and the ovarian crescent sign [OCS]). Patients were managed expectantly or surgically, in line with 3 rules of the DTS. Rule 1: asymptomatic patients having a mass with MI of 4 or less and OCS present, were managed expectantly. Rule 2 (emergency): malignancy was suspected if the MI was 7 or more and no edema of the OCS was present. Rule 3 (nonemergency): malignancy was suspected if the OCS was absent and MI was 5 or more. The diagnostic accuracy was assessed using sensitivity and specificity (P < .05). RESULTS: No malignancy was found in the group of 1236 patients selected according to the DTS rule 1. Torsion was confirmed in 36% of surgically treated masses (n = 115). The OCS was present in 96% of benign masses in the non-emergency group (n = 149) and in three with microscopic malignancy. In predicting malignancy, the DTS (rules 2 and 3) showed a sensitivity of 93 (95% confidence interval [C.I.]: 82-98); and a specificity of 97 (C.I.: 95-99). Ovarian tissue was preserved from benign (n = 254, 93%) and malignant tumors (n = 3, 7%). Only five (2%) uncomplicated ovarian cysts were surgically treated. CONCLUSION: The DTS with 3 rules is a very accurate diagnostic tool in the differentiation between benign and malignant ovarian masses. The DTS rule 1 reduces the number of surgical procedures on functional cysts, rules 2 and 3 are very useful in choosing the optimal treatment of adnexal masses, whether or not they are twisted.

Torsion of malignant ovarian tumors in childhood and adolescence.

AIM: To investigate the frequency of torsion of malignant ovarian tumors in children and adolescents. METHODS: We evaluated all patients treated for adnexal torsion in a tertiary care referral pediatric institution during the last 20 years. Presentation, tumor markers and pathology reports were evaluated. We reviewed the literature on torsion of malignant ovarian tumors. RESULTS: Ninety-two girls (age 6 months to 19 years), 41 of them premenarchal, were surgically treated for adnexal torsion. Symptoms and signs that led to clinical investigation and subsequent surgery were not specific. Histological findings of torsioned masses showed 69 non-neoplasms and 23 tumors, including five malignant. Origin of the malignant disease included four germ cell tumors and one sex-cord stromal tumor. The morphology index score for malignant tumors was > or = 7 in all five patients. Tumor markers were elevated in 12 patients, including four of the patients with malignant tumors. Complete staging was performed in three adolescents with stage Ia, IIa and IIIa of disease. We found tumor origin for 11 previous reported patients with torsion of malignant ovarian tumor, including seven germ cell and four granulosa cell tumors. CONCLUSION: Torsion of malignant ovarian tumors in pediatric and adolescent patients occurs very rarely, but it is nevertheless possible at any stage of disease. The most common torsioned malignant ovarian tumors were of germ cell origin, in both premenarchal and postmenarchal girls. A torsioned adnexal mass with index > or = 7 needs to be considered as a potential malignant tumor.

Pre-operative differentiation of pediatric ovarian tumors: morphological scoring system and tumor markers.

OBJECTIVE: To investigate the importance of morphological scoring systems in differentiation of ovarian tumors in childhood. METHODS: Morphological assessment using DePriest's index was performed for all patients with histopathological confirmation of ovarian tumor, with evaluation of tumor markers, from January 1997. RESULTS: Fifty-three girls (age range 13 months to 19 years) were surgically treated for 59 ovarian tumors, including six bilateral. All lesions with cystic appearance on ultrasonography were benign, 23 of 35 semisolid, and four of ten solid tumors were also benign. Stage of malignant disease was as follows: stage I, ten; stage II, two; stage III, six. Sensitivity, positive predictive value and accuracy by DePriest's and Ueland's indexes for benign tumors (score <7) were: 0.88, 0.79; 0.89; and 0.94, 0.84; 0.93; respectively. Elevated levels of tumor markers were observed in 17 patients, including four patients with endocrine manifestations. In 24 patients ovaries were successfully preserved, including two patients with foci of immature teratoma in a dermoid cyst. CONCLUSION: Ultrasonographic assessment with morphological analysis recommended by DePriest and Ueland is a very useful procedure for differentiating benign from malignant ovarian tumors in children. Tumor markers and endocrinological investigation are also useful for preoperative evaluation.

Rapidly growing bilateral ovarian cystadenoma in a 6-year-old girl: case report and literature review.

BACKGROUND: Benign ovarian neoplasms originating from epithelial tissue are common tumors in adult women. However, they are rarely seen in the pediatric population, especially in the first decade of life. CASE: We report a case of a 6-year-old, premenarchal girl, previously healthy, with frequent micturition lasting 3 days prior to the first examination, without discomfort or pain. Laboratory analyses of blood and urine showed no abnormalities. Repeated ultrasonographic examinations revealed bilateral, cystic, rapidly growing ovarian masses. Cysts were surgically removed, with preservation of normal ovarian tissue, and histopathologic findings showed a serous cystadenoma of both ovaries.