What recent primary studies tell us about ovarian teratomas in children: a scoping review.

Our knowledge of ovarian teratomas in children is still far from complete, and much remains to be discovered. Here, we conduct a scoping review of the primary research related to ovarian teratomas in pediatric age. To our knowledge, there is no published synthesis of the literature surrounding ovarian teratomas in children using scoping review methodology. We identified 24 studies from 11 countries; 18 studies were retrospective, 3 were prospective, and 3 were experimental. There were 6 studies concerning mature teratomas, 5 concerning immature teratomas, and 13 that included both tumor types. Overall, 9 out of all the studies concerned more than 50 patients. We revealed 7 major branches of research within the topic of ovarian teratoma in pediatric population: recurrence rate/relapse and follow-up strategy, malignant potential, prognostic factors, use of sparing surgery, differences between the use of laparoscopy and laparotomy, use of chemotherapy, and additional examinations to test the character of the lesion (immature vs. mature). This scoping review has revealed a number of knowledge gaps in the evidence base for pediatric ovarian teratomas. Overall, this topic has not been extensively explored, and more research dedicated exclusively to this tumor and patient population is required.

Recurrent arginine substitutions in the ACTG2 gene are the primary driver of disease burden and severity in visceral myopathy.

Visceral myopathy with abnormal intestinal and bladder peristalsis includes a clinical spectrum with megacystis-microcolon intestinal hypoperistalsis syndrome and chronic intestinal pseudo-obstruction. The vast majority of cases are caused by dominant variants in ACTG2; however, the overall genetic architecture of visceral myopathy has not been well-characterized. We ascertained 53 families, with visceral myopathy based on megacystis, functional bladder/gastrointestinal obstruction, or microcolon. A combination of targeted ACTG2 sequencing and exome sequencing was used. We report a molecular diagnostic rate of 64% (34/53), of which 97% (33/34) is attributed to ACTG2. Strikingly, missense mutations in five conserved arginine residues involving CpG dinucleotides accounted for 49% (26/53) of disease in the cohort. As a group, the ACTG2-negative cases had a more favorable clinical outcome and more restricted disease. Within the ACTG2-positive group, poor outcomes (characterized by total parenteral nutrition dependence, death, or transplantation) were invariably due to one of the arginine missense alleles. Analysis of specific residues suggests a severity spectrum of p.Arg178>p.Arg257>p.Arg40 along with other less-frequently reported sites p.Arg63 and p.Arg211. These results provide genotype-phenotype correlation for ACTG2-related disease and demonstrate the importance of arginine missense changes in visceral myopathy.

Neonatal gastric outlet obstruction by isolated pyloric atresia, an often forgotten diagnosis.

BACKGROUND: Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. METHODS: A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. RESULTS: Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. CONCLUSIONS: Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.

Fungal urinary tract infection complicated by acute kidney injury in an infant with intestino-vesical fistula.

We report one infant, who in the course of therapy of bacterial urinary tract infection developed fungal UTI and acute kidney injury. It was caused by coexistence of well-known risk factors and additionally intestino-vesical fistula. Appropriate and timely introduced treatment with intravenous fluconazole proved to be therapeutic in the patient. Our report shows that in every case detailed analysis of predisposing factors should be performed and appropriate diagnostic studies ordered, including the possible presence of other - less common - factors, e.g. defects in the gastrointestinal tract.

Various aspects of transition of care for adolescents with urological conditions.

Transition into adulthood is a common issue in many disciplines. However, urology faces additional difficulties due to different models of care and training as well as a wide diversity of pathologies. The goal of this paper is to discuss various aspects of the transition of urological care. This review provides some examples of pathologies that might require special attention of specialists. Most patients with rare diseases must be closely followed up in the long term. However, high-volume conditions may also have a huge impact on the well-being and quality of life in adulthood. Children who are cured due to oncological conditions will probably need additional attention in adulthood. The urological care during childhood is provided by a pediatric urologist, a pediatric surgeon or a urologist, depending on the local regulations and the organization of care. All patients are subsequently referred to a general urologist. Nowadays, a multidisciplinary approach is recommended in many cases, with a pediatric urologist as one of the team members. The patient, caregivers and healthcare professionals must be fully involved and focused on close cooperation to make the transition process smooth and successful.

Recommendations of procedures to follow in the case of ovarian lesions in girls.

This study presents current recommendations of the Polish Association of Pediatric Surgeons (PTChD) regarding diagnostics and treatment of ovarian lesions in girls. They are based on many years of the authors' clinical experience as well as a review of international literature and include practical clinical guidelines. The recommendations were formulated in cooperation with the Polish Association of Pediatric Oncology and Hematology (PTOHD), Polish Pediatric and Adolescent Gynecology Section of the Polish Society of Gynecologists and Obstetricians (PTG) and Polish Pediatric Section of the Polish Society of Radiology (PLTR). Only better understanding of prepubertal ovarian biology and natural history of its pathology may help to introduce efficient and safe diagnostic and therapeutic strategies for girls. The prepared document has been supplemented with treatment algorithms.

What Should Be the Topics of a Prospective Study on Ovarian Masses in Children?-Results of a Multicenter Retrospective Study and a Scoping Literature Review.

PURPOSE: to determine management problems of ovarian masses in girls in order to form a baseline for prospective randomized studies of the established topics and quality improvement of our management. MATERIALS AND METHODS: We performed a national analysis of clinical aspects of ovarian masses in girls operated on in Poland, analyzed retrospectively medical files of all consecutive patients aged 0-18 who underwent surgeries for ovarian lesions between 2012 and 2017 at 17 pediatric surgical departments and complemented the analysis with a scoping review of a recent primary research related to ovarian masses in children. RESULTS: The study group comprised 595 patients. Forty-four (7.39%) girls were diagnosed with malignant tumors. The overall preservation rate was 64.54%. The analysis revealed that positive tumor markers (OR = 10.3), lesions larger than 6 cm (OR = 4.17) and solid mass on ultrasound examination (OR = 5.34) are interdependent variables differentiating malignant tumors from non-malignant lesions (X42 = 79.1; p = 0.00000). Our scoping review revealed 10 major branches of research within the topic of ovarian masses in pediatric population. CONCLUSIONS: We have developed an overview of the field with the emphasis on the local environment. Our next step is a multi-institutional prospective study of a quality improvement project implementation based on the obtained knowledge.

The Quality of Life of Children with Myelomeningocele: A Cross-Sectional Preliminary Study.

OBJECTIVES: To investigate the relationship between the defects and symptoms caused by myelomeningocele (MMC) and quality of life. DESIGN: A cross-sectional questionnaire survey. METHODS: The subjects were 52 parents of children with MMC. Structured questionnaires were used: the Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQLTM 4.0) and a Study-Specific Questionnaire (SSQ). RESULTS: The overall PedsQLTM 4.0 score was 56.4 (SD ± 14.7). A strong, significant negative correlation was found between the children's age and emotional functioning. Functioning in this area deteriorated with age by a mean of 1.5 points per year of age. Children with no hydrocephalus functioned significantly better than those with this defect in the physical, social, and school areas (p < 0.05). Foot deformities significantly (p = 0.033) adversely affected the children's physical functioning. Living in a single-parent family had no statistically significant impact on functioning in any of the areas analyzed (p > 0.05). CONCLUSION: Understanding the QoL of children with MMC and identifying its determinants may help in planning interventions to minimize the adverse impact of the illness.

Does open ovarian biopsy in prepubertal age affect ovarian reserve in a rat model?

BACKGROUND: Partial resection of the ovary carries a possible risk of fertility reduction. We studied the influence of open ovarian biopsy on ovarian reserve, including anti-Müllerian hormone and follicle-stimulating hormone serum level evaluation, in a prepubertal rat model. METHODS: Interventions - the initial surgery was unilateral ovarian biopsy (38 rats, group B1, B2) or unilateral ovarian biopsy and ovarian resection of the contralateral gonad (38 rats, group BR1, BR2). The second operation was bilateral ovarian resection and total resection of the remaining ovary. All rats had hormone serum levels evaluated. The control group had only a blood test taken and bilateral ovarian resection done at the second intervention (30 rats, group C1, C2). The collected tissue was examined estimating follicle count and anti-Müllerian hormone immunoexpression. RESULTS: Anti-Müllerian hormone levels were significantly lower at the second intervention in the group BR2 but significantly higher in the group C2. Follicle-stimulating hormone levels were significantly higher in all but one group (BR2). CONCLUSIONS: Biopsy itself might not reduce ovarian reserve if done properly but we should know its possible negative effects in the case of a single remaining ovary.

Secondary skin neoplasms in patients after autologous and allogeneic hematopoietic stem cell transplantation procedures.

The increasing number of hematopoietic stem cell transplantation (HSCT) procedures and lower transplant-related mortality has led to a growing population of survivors facing long-term increased risk of secondary malignancy, including cutaneous neoplasms. In this review, we aim to discuss the incidence, risk factors and preventive strategies for secondary skin neoplasms after autologous and allogeneic HSCT. Cutaneous neoplasms, such as basal cell carcinoma, squamous cell carcinoma and melanoma, are among the most common solid cancers arising in patients after HSCT. Besides risk factors established in the general population, primary disease, chronic graft-versus-host disease (CGvHD), prolonged immunosuppression, especially with the use of cyclosporine and azathioprine, radiation exposure, light skin color, male sex, and young age at transplantation play a role in the development of cutaneous neoplasms in HSCT recipients. Skin cancer development after HSCT may be explained by cumulative effects of chemotherapy and radiotherapy-induced DNA damage, prolonged immunosuppressive conditions and chronic mucosal inflammation, particularly after allogeneic HSCT. Delayed immune recovery and persistent immunodeficiency in patients with graft-versus-host disease (GvHD) may also contribute to carcinogenesis. Regular dermatological surveillance and prompt recognition of precancerous and cancerous lesions is crucial for patient's prognosis and management.

Mayer-Rokitansky-Küster-Hauser syndrome as an interdisciplinary problem.

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian agenesis or aplasia, is a congenital disease manifested by the aplasia of the uterus and the upper 2/3 of the vagina; its incidence is 1 in 4,000-5,000 female live births. We can distinguish 2 types of the MRKH syndrome: type I, which is characterized by an isolated absence of 2/3 of the vagina and uterus; and type II or MURCS (Müllerian duct aplasia, unilateral renal agenesis and cervicothoracic somite anomalies), which is also associated with other symptoms. The treatment of the MRKH syndrome patients aims at creating a neovagina and enabling sexual intercourse. Non-surgical techniques are the first-choice methods, and more than 90% of patients notice an anatomical and functional improvement if they are well-prepared emotionally. If non-surgical treatment does not bring about the expected results, a surgical procedure remains an option. The surgical method is mainly determined by the surgeon's experience. There are a few types of operations, though none of them seems superior to others. The next challenge is to provide these patients with a chance to become parents. Nowadays, a uterine transplant, a surrogate or adoption are the available solutions. An interdisciplinary approach is required, and the treatment should consist of medical and psychological support. This review presents the current knowledge about the MRKH syndrome with regard to the current methods of non-surgical and surgical treatment as well as a summary of the associated psychological problems.

Safety assessment during initiation and maintenance of propranolol therapy for infantile hemangiomas.

BACKGROUND: Propranolol is an effective method of treatment for infantile hemangiomas (IH). A recent concern is a shift of the therapy into outpatient settings. OBJECTIVES: The aim of the study was to evaluate the safety of initiating and maintaining propranolol therapy for IH. MATERIAL AND METHODS: The study involved 55 consecutive children with IH being treated with propranolol. The patients were assessed in the hospital at the initiation of the therapy and later in outpatient settings during and after the therapy. Each time, the following monitoring methods were used: physical examination, cardiac ultrasound (ECHO), electrocardiography (ECG), blood pressure (BP), heart rate (HR), and biochemical parameters: blood count, blood glucose, aspartate transaminase (AST), alanine transaminase (ALT), and ionogram. The therapeutic dose of propranolol was 2.0 mg/kg/day divided into 2 doses. RESULTS: Four children were excluded during the qualification or the initiation of propranolol; a total of 51 patients were subject to the final analysis. All the children presented clinical improvement. There was a significant reduction in the mean HR values only at the initiation of propranolol. There were no changes in HR during the course of the therapy. Blood pressure values were within normal limits. Both systolic and diastolic values decreased in the first 3 months. Bradycardia and hypotension were observed sporadically, and they were asymptomatic. Electrocardiography did not show significant deviations. The pathological findings of the ECHO scans were not a contraindication to continuing the therapy. There were no changes in biochemical parameters. Apart from 1 symptomatic case of hypoglycemia, other low glucose episodes were asymptomatic and sporadic. The observed adverse effects were mild and the propranolol dose had to be adjusted in only 6 cases. CONCLUSIONS: Propranolol is effective, safe and well-tolerated by children with IH. The positive results of the safety assessment support the strategy of initiating propranolol in outpatient settings. Future studies are needed to assess the benefits of the therapy in ambulatory conditions.

Ovarian teratoma in children: a plea for collaborative clinical study.

BACKGROUND: Although teratomas are the most common histologic subtype of childhood ovarian germ cell tumors, their appropriate treatment in this age group still remains unclear. Paucity of research dedicated exclusively to both mature and immature teratomas of the ovary, contribute to decision making difficulties. Therefore, we decided to review retrospectively our experience in treatment of pediatric ovarian teratomas in order to assess the epidemiology, presenting features, and diagnostic as well as surgical management of these lesions. RESULTS: The study comprised 58 patients. Fifty percent of patients were between 9 and 15 years old. Mature teratoma was diagnosed in 55(94.83%) patients, while 3(5.17%) patients presented with immature teratoma. Twenty eight (50.91%) girls with mature teratoma had laparotomy and 23 (41.82%) had laparoscopy performed as an initial operative approach. Ovarian tissue sparing technique (preservation of the ovarian tissue of the affected gonad) was applied in only 11.11% of patients operated in the first study period (years 1999-2003) and increased to 40.54% in the second half of our study (years 2004-2016). The extent of gonadal resection was not related with the size of the lesion. Bilateral lesions were noted in 8 patients with mature teratoma. All girls with immature teratoma were subjected to formal laparotomy. Two patients had stage III of the disease and one had stage IV. They underwent at least resection of the affected gonad. Adjuvant chemotherapy was given to all girls with immature teratoma after the surgery. CONCLUSIONS: Under particular conditions ovarian-sparing surgery might be successfully applied in children with mature teratoma. Laparotomy is the treatment of choice in large masses, suspicious for malignancy and if surgical staging is required. High quality prospective multi-institutional studies are required in order to get an objective insight into biology and prognostic factors of teratomas in children.

Serum concentrations of VEGF and bFGF in the course of propranolol therapy of infantile hemangioma in children: Are we closer to understand the mechanism of action of propranolol on hemangiomas?

BACKGROUND: Propranolol has become the treatment of choice for infantile hemangiomas (IH). Neither the pathogenesis of IH nor the mechanism of action of propranolol on them are well understood. Possible explanations include the inhibition of angiogenesis by decreasing vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), induction of vascular endothelial cell apoptosis and vasoconstriction. OBJECTIVES: The aim of the study was to assess serum concentrations of VEGF and bFGF in the course of propranolol therapy of IH in children, and to assess their clinical implications. MATERIAL AND METHODS: The study included 51 children with IH treated with propranolol. The participants were assessed before, during and after the therapy with Hemangioma Activity Score (HAS), Doppler ultrasound (US) of the lesions, as well as VEGF and bFGF serum concentrations. RESULTS: All children showed clinical improvement measured in the HAS. A complete involution of the IH was reported in 32 (63%) children at the time of decision of the gradual withdrawing of propranolol, and in 28 (61%) patients at the end of the treatment (out of 46 patients present at the follow up after 1.5 months). Doppler US at the follow-up showed a complete disappearance of the blood flow in the lesion in 24 (52%) children and its reduction in 12 (26%) children. There was a significant decrease in VEGF and bFGF during and after treatment compared to pretreatment values. There was a correlation between the outcome of the Doppler US and changes in bFGF during and after treatment. Changes in VEGF during treatment did not correlate with changes in the Doppler US. CONCLUSIONS: Serum concentrations of VEGF and bFGF decreased during the propranolol treatment of IH, which may indicate the effect of propranolol on both. However, the statistical analysis showed their low prognostic value as biochemical markers of propranolol treatment. Clinical evaluation combined with Doppler US is the most valuable method of monitoring the therapy.

Guidelines of Polish National Societies Diagnostics and Treatment of Thyroid Carcinoma. 2018 Update.

Significant advances have been made in thyroid can-cer research in recent years, therefore relevant clinical guidelines need to be updated. The current Polish guidelines "Diagnostics and Treatment of Thyroid Carcinoma" have been formulated at the "Thyroid Cancer and Other Malignancies of Endocrine Glands" conference held in Wisla in November 2015 [1].

Late presenting diaphragmatic hernia: clinical and diagnostic aspects.

BACKGROUND: The congenital diaphragm hernia presents most frequently in the neonatal period. In a small group of children his defect can be diagnosed beyond the newborn age, during late infancy or early childhood. The late presenting congenital diaphragm hernia is characterized by a variable clinical picture and represents a considerable diagnostic challenge. The aim of this study was to evaluate the usefulness of imaging methods in diagnosis, monitoring and management of late presenting diaphragmatic hernias. MATERIAL/METHODS: The retrospective analysis of 58 children with congenital diaphragm hernia, aged from 1 day to 7.5 years (the average: 2.6 years) treated between 1990-2006 in the Department of Pediatric Surgery and Urology of the Medical University of Wroclaw was carried out. 19 (39%) children in whom the malformation was recognized beyond the newborn period were numbered to this group. The detailed analysis comprised the type of clinical manifestation, as well as the therapeutic and diagnostic algorithm. RESULTS: The postero-lateral variant of the defect was recognized in 15 children, the Morgagni type in 2 and the hiatal type in 2. Right-sided hernia was found in 4 children, whereas left-sided in 15. 8 infants with left hernia presented with dominant symptoms from the respiratory system; 3 infants - from the GI tract with delayed somatic growth. Among children over 1 year of age, 6 presented symptoms of respiratory origin and only 2 of gastrointestinal nature. Only one child showed associated congenital malformations. In all children the diagnosis was made on the basis of imaging modalities. The GI contrast study was decisive imaging method in 14 children, ultrasonography in 15, CT in 4. CONCLUSIONS: The lack of typical clinical presentation in cases of late presenting CDH leads to delayed diagnosis of the defect. This clinical entity should be however taken into account in the differential diagnosis of children with respiratory distress and GI disturbances. The imaging studies are essentials in every case.

Selecting treatment method for ovarian masses in children - 24 years of experience.

BACKGROUND: Epidemiology and pathology of ovarian tumors in the pediatric population are very different of these encountered in women. Few attempts have been made to analyze the whole spectrum of ovarian pathology in children, and only some of them included series of more than 200 cases. We performed a retrospective analysis of clinical and diagnostic aspects of ovarian tumors and tumor-like lesions in girls in order to identify the characteristics associated with malignancy with an attempt to elaborate a clinical management algorithm. RESULTS: The study group comprised 214 patients operated on for ovarian tumor in years 1991-2014 at the pediatric surgical center. Non-neoplastic ovarian lesion was diagnosed in 127 females. Sixty-five patients had a benign tumor and 22 had a malignant lesion. Abdominal pain was the most common symptom in the non-malignant lesion group. Patients with ovarian malignancy presented predominantly with abdominal distension and palpable mass. In the non-malignant group imaging studies revealed cystic lesion in 124 patients (68.89%) and solid mass in 10 (5.55%). Malignant lesion showed a solid or mixed structure in all cases. Positive tumor markers were noted in 14 (13.7%) patients with a benign lesion and in 14 (70%) with ovarian malignancy. Large lesions were found in 77.3% of girls with a malignant mass, while only in 32.8% of patients with a benign lesion (p < 0.001). In the group of solid tumors positive tumor marker results occurred more frequently in patients with diagnosed malignant tumors (p < 0.05). Positive tumor markers, large size of the lesion and age below 14 years were independent variables differentiating malignant tumors from non-malignant lesions (p = 0.00000). CONCLUSIONS: Predominantly solid structures noted on imaging studies, large dimension and positive tumor markers are clinical predictors of malignancy. A diagnosis of purely cystic lesions with negative markers or of a small size should be an indication for a gonad-sparing procedure. Treatment guidelines for ovarian lesions in children should be established on the basis of multicenter prospective studies and introduced as soon as possible in order to improve and unify the ovarian preservation rates across the pediatric surgical centers.

Diagnostics and Treatment of Thyroid Carcinoma.

Revised Guidelines of Polish National Societies Prepared on the initiative of the Polish Group for Endocrine Tumours approved in their final version between November 16th and 28th, 2015 by the Scientific Committee of the V Conference "Thyroid Cancer and other malignancies of endocrine glands" organised between November 14th and 17th, 2015 in Wisla, Poland; called by the following Societies: Polish Endocrine Society, Polish Society of Oncology, Polish Thyroid Association, Polish Society of Pathologists, Society of Polish Surgeons, Polish Society of Surgical Oncology, Polish Society of Clinical Oncology, Polish Society of Radiation Oncology, Polish Society of Nuclear Medicine, Polish Society of Paediatric Endocrinology, Polish Society of Paediatric Surgeons, Polish Society of Ultrasonography Gliwice-Wisla, 2015 DECLARATION: These recommendations are created by the group of delegates of the National Societies, which declare their willingness to participate in the preparation of the revised version of the Polish Guidelines. The members of the Working Group have been chosen from the specialists involved in medical care of patients with thyroid carcinoma. Directly before the preparation of the Polish national recommendations the American Thyroid Association (ATA) published its own guidelines together with a wide comment fulfilling evidence-based medicine (EBM) criteria. ATA Guidelines are consistent with National Comprehensive Cancer Network (NCCN) Recommendation. According to the members of the Working Group, it is necessary to adapt them to both the specific Polish epidemiological situation as well as to the rules referring to the Polish health system. Therefore, the Polish recommendations constitute a consensus of the experts' group, based on ATA information. The experts analysed previous Polish Guidelines, published in 2010, and other available data, and after discussion summed up the results in the form of these guidelines. It should be added that Part II, which constitutes a pathological part, has been available at the website of the Polish Society of Pathologists for acceptance of the members of the Society, and no essential comments have been proposed. The Members of the Group decided that a subgroup elected from among them would update the Guidelines, according to EBM rules, every year. The Revised Guidelines should help physicians to make reasonable choices in their daily practice; however, the final decision concerning an individual patient should be made by the caring physician responsible for treatment, or optimally by a therapeutic tumour board together with the patient, and should take into consideration the patient's health condition. It should be emphasised that the recommendations may not constitute a strict standard of clinical management imposed on medical staff. The data from clinical trials concerning numerous clinical situations are scarce. In such moments the opinion of the management may differ from the recommendations after considering possible benefits and disadvantages for the patient.

Risk of Malignancy and Need for Surgery in Pediatric Patients with Morris or Y-chromosome Turner Syndrome: A Multicenter Survey.

STUDY OBJECTIVE: The management of intersex patients with Y-chromosome Turner or Morris syndrome remains a challenge. We report our experience with a multicenter European survey. DESIGN: We collected the data on 18 patients (mean age 10.2 years, range 2-17 years) with Morris (10 patients) or Turner (8 patients) syndrome harboring the Y chromosome who were treated in 1 of 6 European centers of pediatric surgery between 1997 and 2013. All patients were evaluated by use of a multidisciplinary diagnostic protocol. All patients received a bilateral gonadectomy via laparoscopy; only 1 center performed ovarian cryopreservation. Seven patients received a concomitant genitoplasty. Operative notes and histology were reviewed for details. RESULTS: No conversions to laparotomy and no complications were recorded. For the patients receiving only the gonadectomy, the length of hospital stay was 24-48 hours, whereas for the patients receiving an associated genitoplasty, it was 6-10 days. Specimens were negative for tumors in 83.3% of cases, whereas in 3 patients (16.6%), benign abnormalities (Sertoli cell hyperplasia in 1 patient and ovotestis in 2 patients) were recorded. A malignant tumor was not recorded in our series. CONCLUSION: If the risk of malignancy is considered as the main indication for surgery in case of Turner or Morris syndrome, on the basis of our study, this indication should be reevaluated. However, based on the non-negligible rate of benign abnormalities reported in our series (16.6%), the performance of cryopreservation to preserve fertility and the possibility of performing genitoplasty during the same anesthetic procedure represent additional valid indications for surgery.