Malignant and Benign Head and Neck Tumors of the Pediatric Age: A Narrative Review.

Malignant tumors of the head and neck are rare in children, but it is important to know these lesions and identify them early in order to have a good outcome for these patients. Benign lesions of the head and neck are much more frequent and have an excellent prognosis. For this reason, it is necessary to recognize the warning signs and symptoms and understand when to refer the patient to a reference center for the treatment of these pathologies. The clinical presentation of both benign and malignant lesions in children may be similar as usually, both categories have compressive effects. This confirms the fact that the clinical diagnosis is not sufficient and always requires instrumental investigations and biopsies. In this narrative review, we analyzed both malignant lesions such as lymphoma, rhabdomyosarcoma, thyroid tumors, salivary gland tumors, neuroblastoma, and nasopharyngeal carcinoma, and benign ones such as cystic dermoid teratoma, hemangioma, juvenile angiofibroma and fibrosis dysplasia. Indeed, we set out to discuss the most common lesions of this site by evaluating their characteristics to highlight the differentiation of malignant tumors from benign lesions and their correct clinical-therapeutic management. A literature search was carried out in the PubMed and Google Scholar databases to identify all narrative reviews addressing malignant and benign head and neck tumors of the pediatric age. In conclusion, the care of children affected by head and neck benign lesions and malignancy must be combined and multidisciplinary. It is essential to recognize the diseases early in order to differentiate and intervene as soon as possible for the correct clinical-therapeutic management.

The role of the pediatrician in the management of the child and adolescent with gender dysphoria.

Gender dysphoria is a clinical condition characterized by significant distress due to the discordance between biological sex and gender identity. Currently, gender dysphoria is also found more frequently in children and adolescents, thanks to greater social sensibleness and new therapeutic possibilities. In fact, it is estimated that the prevalence of gender dysphoria in pediatric age is between 0.5% and 2% based on the statistics of the various countries. Therefore, the pediatrician cannot fail to update himself on these issues and above all should be the reference figure in the management of these patients. Even if the patient must be directed to a referral center and be followed up by a multidisciplinary team, the treating pediatrician will care to coordinate the clinical and therapeutic framework. The aim of the present report is therefore to integrate literature data with our clinical experience to propose a new clinical approach in which the pediatrician should be the reference in the care of these patients, directing them towards the best therapeutic approach and staying in contact with the specialists of the referral center.

Autoimmune Polyendocrine Syndromes in the Pediatric Age.

Autoimmune polyendocrine syndromes (APSs) encompass a heterogeneous group of rare diseases characterized by autoimmune activity against two or more endocrine or non-endocrine organs. Three types of APSs are reported, including both monogenic and multifactorial, heterogeneous disorders. The aim of this manuscript is to present the main clinical and epidemiological characteristics of APS-1, APS-2, and IPEX syndrome in the pediatric age, describing the mechanisms of autoimmunity and the currently available treatments for these rare conditions.

The Impact of Oxidative Stress on Pediatrics Syndromes.

Oxidative stress is a condition determined by an imbalance between antioxidant and oxidative factors. Oxidative stress can have serious consequences on our organism. Indeed, it causes both necrosis and cell apoptosis, determining cellular aging, increased carcinogenesis, vascular stiffening, increased autoimmune diseases, and muscle decay. In the context of pediatric syndromes, oxidative stress could play a role in the first order. In fact, our review of the literature showed that in some pathologies, such as fetal alcohol spectrum disorders, oxidative stress related to the intake of ethanol during pregnancy is a main etiological factor determining the associated clinical syndrome. On the contrary, in Williams syndrome, Down syndrome, Marfan syndrome, Gaucher syndrome, ataxia-telangiectasia, autistic spectrum disorder, Fanconi's anemia, and primitive immunodeficiencies, the increase in oxidative stress is directly associated with the genetic alterations that cause the same pathologies. Although further studies are needed to better understand the relationship between oxidative stress and pediatric diseases, a better knowledge of this crucial issue encourages future therapeutic strategies.

980 nm diode lasers in oral and facial practice: current state of the science and art.

AIM: To evaluate the safety and efficacy of a 980 nm diode laser for the treatment of benign facial pigmented and vascular lesions, and in oral surgery. MATERIALS AND METHODS: 20 patients were treated with a 980 nm diode laser. Oral surgery: 5 patients (5 upper and lower frenulectomy). Fluence levels were 5-15 J/cm(2); pulse lengths were 20-60 ms; spot size was 1 mm. Vascular lesions: 10 patients (5 small angiomas, 5 telangiectases). Fluences were 6-10 J/cm(2); pulse lengths were 10-50 ms; spot size was 2 mm. In all cases the areas surrounding the lesions were cooled. Pigmented lesions: 5 patients (5 keratoses). All the lesions were evaluated by dermatoscopy before the treatment. Fluence levels were 7-15 J/cm(2); pulse lengths were 20-50 ms; spot size was 1 mm. All the patients were followed at 1, 4 and 8 weeks after the procedure. RESULTS: Healing in oral surgery was within 10 days. The melanoses healed completely within four weeks. All the vascular lesions healed after 15 days without any residual scarring. CONCLUSIONS: The end results for the use of the 980 nm diode laser in oral and facial surgery appears to be justified on the grounds of efficacy and safety of the device, and good degree of acceptance by the patients, without compromising their health and function.

Endoscope-assisted microneurosurgery for anterior circulation aneurysms using the angle-type rigid endoscope over a 3-year period.

INTRODUCTION: The use of the endoscope (fiberscope) to assist the microsurgical clipping of cerebral aneurysm was first reported by Fischer and Mustafa in 1994. The rigid endoscope has been increasingly used during aneurysm surgery in which structures around the aneurysm can be detected with high quality imaging. Our 3 years of its use now allows us to assess the endoscope's efficacy and limits in standard surgery with a pterional approach in aneurysms of the anterior circulation. The endoscope can carry out a supportive role in planning surgical manoeuvres and in verifying whether clipping has been performed correctly or not. In our view, among the aneurysms of the anterior circulation, the endoscope is particularly useful in those of the internal carotid and the anterior communicating arteries. In many cases of these aneurysms the posterior communicating artery, choroidal artery or one of the distal cerebral arteries is hidden behind the aneurysm dome. Dome retraction is often required in order to see these vascular structures with the microscope. Thus an endoscope with a 30 degrees view angle becomes very useful. The concealed areas are identified without retraction, which prevents the possibility of the aneurysm being ruptured and also reduces the use of temporary clipping. From its early use as a supportive measure that is sometimes useful in surgery for "easy" aneurysms, the endoscope has now become almost indispensable for the "difficult" aneurysms, including the large and giant ones before and after clipping. Thus, the endoscope should be kept ready for use in the operating theatre for any eventuality. OBJECTIVE: We assess the advantages and disadvantages of the use of the endoscope in the microsurgical treatment of intracranial aneurysms. METHODS: During our 3 years of experience, 52 patients with 48 ruptured and 10 unruptured aneurysms of the anterior circulation (including 6 cases of two-fold aneurysms) underwent clipping with endoscope support through a pterional approach. All ruptured aneurysms produced a Hunt and Hess Grade I or II subarachnoid haemorrhage. The endoscope was inserted before and after clipping in order to observe the conditions surrounding the aneurysm and to receive immediate confirmation that clipping had been performed correctly. RESULTS: In all cases general anatomy visualization was provided by the endoscope, and the correct clip positioning and vessel conditions were easily checked. In 4 cases the endoscope showed that the clip had been positioned incorrectly. Additional clipping was performed in these cases: in 2 cases the clip was re-applied correctly and in another case a clip was added. Only the fourth patient with a large communicating artery died (1.9%) of cerebral infarction. This was due to post-clipping stenosis of one distal cerebral artery in which it was not possible to re-position the clip correctly because of the presence of arteriosclerotic calcific plaque near the aneurysm neck. In 3 cases there was an intraoperative ruptured aneurysm dome that was not caused by the endoscope insertion. No further complications were caused by the endoscope. CONCLUSION: In certain cases endoscopic-assisted microsurgery is an exceptional aid to the surgeon and must become part of the operating theatre equipment and kept on hand ready for use. The endoscope is, in our opinion, particularly useful in certain aneurysm localisations (internal carotid artery-anterior communicating artery [ICA-ACOMA]).

Intraepithelial carcinoma arising in an endodermal cyst of the posterior fossa.

Endodermal cysts of the central neuraxis are benign, non-neoplastic epithelium-lined cysts arising from endodermal tissue that have been displaced early in fetal life. Intracranial endodermal cysts are rare and usually located in the posterior fossa. The present study involves a 36-year-old man with a typical epithelial cyst in the posterior fossa. Microscopically, the cyst has a simple columnar epithelium with mucus-producing cells, containing an area composed of dysplastic epithelium with evidence of an intraepithelial carcinoma. The atypical cells have a high proliferative fraction demonstrated by Ki-67 immunostain. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic endodermal cyst. The clinicopathologic features and a review of the literature are discussed.