Operative Procedure in a Suprasellar Paediatric Lesion of the Optic Chiasm with Hydrocephalus Caused by a Papillary Glioneuronal Tumour.

INTRODUCTION: Paediatric tumours in the sellar and parasellar regions present clinical and surgical challenges due to anatomical position and behaviour. We illustrate a rare case which caused obstructive hydrocephalus. CASE PRESENTATION: The study included a 14-year-old girl with a glioneuronal tumour (40 mm) originating from the optic chiasm, obliterating the aqueduct, with consequent triventricular hydrocephalus. The patient underwent extended endoscopic endonasal surgery and repair of the skull-base deficiency using a multi-layer technique with fascia lata. The 12-month follow-up showed no complications or recurrences, with recovery in visual acuity. CONCLUSION: The immediate placement of external ventricular drainage, in combination with an extended trans-sphenoidal approach, is a safe and feasible option to treat suprasellar paediatric lesions with hydrocephalus.

Temporal bone carcinoma: Classical prognostic variables revisited and modern clinico-pathological evidence.

AIM: Prognostic factors, rational management, and the ongoing investigations regarding temporal bone squamous cell carcinoma (TBSCC) have been critically reviewed. BACKGROUND: TBSCC is an uncommon, aggressive malignancy. Although some progress has been made in treating this aggressive tumor, the prognosis in advanced cases remains poor. MATERIALS AND METHODS: A systematic search of the literature for articles published between 2009 and October 2014 was performed using the PubMed (http://www.pubmed.gov) electronic database. RESULTS: Given the particular anatomical site of TBSCC, its prognosis is significantly influenced by any direct involvement of nearby structures. The extent of the primary tumor is generally considered one of the most important prognostic factors and it is frequently related to prognosis even more strongly than N stage. For TBSCC, biomarker investigations in surgical specimens are only just beginning to appear in the oncological literature. CONCLUSION: Given the particular features of TBSCC, the sub-specialty of otologic oncology seems to be emerging as a defined area of practice involving multidisciplinary team comprising oto-neurosurgeons, head and neck surgeons, plastic surgeons, oncologists, radiotherapists, dedicated radiologists, and pathologists.

Tezepelumab: patient selection and place in therapy in severe asthma.

Asthma is a disease characterised by heterogeneous and multifaceted airway inflammation. Despite the availability of effective treatments, a substantial percentage of patients with the type 2 (T2)-high, but mainly the T2-low, phenotype complain of persistent symptoms, airflow limitation, and poor response to treatments. Currently available biologicals target T2 cytokines, but no monoclonal antibodies or other specific therapeutic options are available for non-T2 asthma. However, targeted therapy against alarmins is radically changing this perspective. The development of alarmin-targeted therapies, of which tezepelumab (TZP) is the first example, may offer broad action on inflammatory pathways as well as an enhanced therapeutic effect on epithelial dysfunction. In this regard, TZP demonstrated positive results not only in patients with severe T2 asthma but also those with non-allergic, non-eosinophilic disease. Therefore, it is necessary to identify clinical features of patients who can benefit from an upstream targeted therapy such as anti-thymic stromal lymphopoietin. The aims of this narrative review are to understand the role of alarmins in asthma pathogenesis and epithelial dysfunction, examine the rationale underlying the indication of TZP treatment in severe asthma, summarise the results of clinical studies, and recognise the specific characteristics of patients potentially eligible for TZP treatment.

Severe hypereosinophilia in a patient treated with dupilumab and shift to mepolizumab: the importance of multidisciplinary management. A case report and literature review.

Type 2 inflammation is a heterogeneous condition due to the complex activation of different immunological pathways. Rapid progress in research to evaluate the efficacy of biologics for chronic rhinosinusitis with nasal polyps and asthma has led to the availability of effective therapeutic options. These drugs are safe, but temporary iatrogenic hypereosinophilia may sometimes be associated with clinical symptoms or organ damage. Here, we describe a case of severe hypereosinophilia in a patient with chronic rhinosinusitis with nasal polyps and asthma treated with dupilumab and a subsequent therapeutic shift to mepolizumab that led to maintenance of symptom control and concomitant normalization of blood eosinophil count.

Sellar and parasellar lesions: multidisciplinary management.

INTRODUCTION: The endoscopic endonasal transsphenoidal approach to the sella and parasellar regions is now increasingly used for removal of a variety of lesions localized in the ventral skull base. The advantage of the endoscope is enhanced visualization and improved panoramic view that can result in more complete removal of the tumor. An extensive knowledge of the anatomy is mandatory to approach this region. MATERIALS AND METHODS: From February 2009 to March 2020, the endoscopic endonasal approach was used in 153 patients with sellar and parasellar lesions, at our Institution: 136 pituitary adenomas, 7 craniopharyngiomas, 3 Rathke's cysts, a tuberculum sellae meningioma, an aneurysm of the internal carotid artery (ICA), a clivus chordoma, a papillary glioneuronal tumor, an histiocytosis, a pituitary metastasis from breast cancer and a chondrosarcoma. RESULTS: The most common surgical complications were cerebral spinal fluid leak (9), bleeding (2), pituitary abscess (2). Among endocrinological complications, the most important were diabete insipidus (23) and panhypopituitarism (3). Two patients complicated with meningitis. There were no visual worsening and no operative mortality. We had persistence of disease in 20 cases. Twelve patients underwent surgical revision for recurrence of the disease. CONCLUSIONS: Pre-operative planning and collaboration with several specialists are necessary in order to offer the patient the best treatment, minimizing complications.

Clivus Chordoma: Case Report and Current Considerations on Treatment Strategies.

Chordomas are the rare malignant bone lesions derived from the embryonic notochord. They are slow-growing tumors with a locally aggressive behavior. The clival chordomas are extradural, exophytic, and lytic lesions centered on the clivus, and are managed differently from those arising elsewhere because of the emphasis on preserving the neurological function. The gold standard for therapy is the complete resection followed by radiation therapy for a better local control of the tumor. This case report concerns a 20-year old girl with an incidental diagnosis of the clival chordoma, which was first treated via an endoscopic anterior approach to remove the exophytic portion of the tumor. The adjuvant radiation therapy was not feasible because of the macroscopic intradural residual tumor being at the level of jugular foramen. The girl was referred to our institute for the removal of the intradural residual tumor via a petro-occipital trans-sigmoid (POTS) approach followed by adjuvant proton beam therapy. The choice of the surgical approach depends on the size of the tumor, its location, and its anatomical distribution, but should also be balanced against the morbidity, considering the patient's age, and the feasibility of postoperative rehabilitation.