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Fertility preservation (FP) in pediatric patients with cancer is an evolving field. https://www.selleckchem.com/products/gw4869.html In this review, we give a short update on recent scientific advances in the practice of pediatric oncofertility, particularly related to the research involving gonadal tissue cryopreservation from prepubertal patients, which remains experimental. We then focus on recent advances in the implementation of formal pediatric oncofertility programs and barriers in the delivery of FP in this patient population. Finally, we include some of the more recent outcomes data from established oncofertility programs that treat pediatric patients.Differences of sexual development (DSD) are known to be associated with an elevated risk of malignant and pre-malignant tumors. However, given the rarity of DSD and tumors in patients with DSD, more robust, large scale, prospective literature is required to truly determine the extent of this association, long-term outcomes and the nuances associated with the wide variety of DSD diagnoses. In addition, the spectrum of diagnoses and nomenclature has been ever-changing, limiting assessment of long-term patient outcomes. This review aims to provide an overview of the pathogenesis of DSD conditions, potential malignancies associated with the diagnoses, the available screening for malignancy, and the most recent data on stratification for each DSD diagnosis and association with malignancy.While radical orchiectomy remains the gold standard for testicular cancer, partial orchiectomy has become a well-accepted organ-sparing approach for benign testicular tumors in pre-pubertal patients. The aims of testicular-sparing surgery include prevention of over-treatment, preservation of future hormonal and reproductive function, and provision of a durable cure. For pre-pubertal patients, who have a high likelihood of benign lesions, partial orchiectomy provides effective treatment, owing to the high reliability of scrotal ultrasound (US) and intraoperative frozen section. In adolescent and young adult patients, who are more likely to harbor malignant pathology, the role of partial orchiectomy is less clear. Testis-sparing surgery is being reported with greater frequency in the adult literature for small testicular masses and for situations in which radical orchiectomy would result in an anorchia. More recently, a testis-sparing approach has also been described for carefully-selected post-pubertal pediatric patients. This review will highlight the role of partial orchiectomy in pediatric patients ( less then 18 years old).The treatment modalities for testicular tumors (surgery, chemotherapy, and radiotherapy), have different associated gonadotoxic risks and the overall survival for most pediatric patients with testicular tumors is very good. However, necessary treatments may lead to the development of lasting gonadal dysfunction and subsequent negative health and quality of life impact. Research with long-term follow-up for patients who have undergone surgery as the sole treatment modality for testicular tumors in childhood are lacking. It is currently unclear if surgery leads to long-term negative functional outcomes. Alkylating agents (e.g., cyclophosphamide) have long been known to increase risk of infertility; platinum-based therapies used frequently for patients with germ-cell tumors (GCTs) also seem to carry some risk of gonadotoxicity, although they have not been as well studied. Radiotherapy to the gonads is toxic and Leydig cells are particularly sensitive to high doses of radiation (>12 Gy). Long-term fertility and hcular function. As research continues in this field, it grows more important for urologists to be aware of the outcomes and options for their patients.Overall survival (OS) for children with Wilms tumor (WT) currently stands at around 90%. This is markedly improved from the survival rates of around 30% reported in the middle of the last century. This improvement is due to the development of multimodal treatment for this disease, based on the evidence yielded through international collaboration on trials conducted by the Société Internationale d'Oncologie Pédiatrique (SIOP) and the Children's Oncology Group (COG). In this article, we review some of the current surgical controversies surrounding the management of WT.Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 9-17% of all cases of the malignancy. Due to an earlier age at WT diagnosis and an increased incidence of bilateral and metachronous disease, management of syndromic WT warrants a distinct approach from that of non-syndromic WT. This review of English-language manuscripts about WT focuses on the most common syndromes, surveillance protocols and current treatment strategies. Highlighted syndromes include those associated with WT1, such as WAGR (Wilms-Aniridia-Genitourinary-mental Retardation), Denys-Drash syndrome (DDS), and Frasier syndrome, 11p15 defects, such as Beckwith-Wiedemann syndrome (BWS), among others. General surveillance guidelines include screening renal or abdominal ultrasound every 3-4 months until the age of 5 or 7, depending on the syndrome. Further, some of the predisposing conditions also increase the risk of other malignancies, such as gonadoblastoma and hepatoblastoma. With promising results for nephron-sparing surgery in bilateral non-syndromic WT, there are increasing reports and recommendations to pursue nephron-sparing for these patients who are at greater risk of bilateral, metachronous lesions. In addition to the loss of renal parenchyma from malignancy, many patients are at risk of developing renal insufficiency as part of their syndrome. Although there may be some increase in the complication rate, recurrence free survival seems equivalent. Some conditions require specialized approaches to adjuvant therapy, as their syndrome may make them especially susceptible to side effects.While the worldwide incidence of newborns with spinal dysraphism is decreasing, the majority of children now survive and reach adulthood, creating new challenges in patient management and long-term care. Despite medical and surgical advances with a subsequent decline in mortality, spina bifida continues to be a major cause of chronic disability and affected individuals face increased mortality in adulthood. The majority of spina bifida patients have neurogenic bladder. Reconstructive and diverting procedures may increase the neoplastic risk in the urinary tract over time, however bladder dysfunction itself may be a risk factor, therefore patients, caregivers, and physicians must be aware of the baseline risk of cancer in patients with neurogenic bladder, in addition to routine cancer screening. Optimal treatment of myelomeningocele requires a multidisciplinary care team to prevent and manage potential sequelae that may affect function, quality of life, and survival.
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