Primary Intracranial Adenoid Cystic Carcinoma: Report of Three Cases.

Objective Nasal cavity and paranasal sinuses host a variety of malignant tumors with adenoid cystic carcinoma (ACC) being the most frequent cancer of salivary gland origin. The histological origin of such tumors virtually precludes primarily intracranial localization. The aim of this study is to report cases of primarily intracranial ACC without evidence of other primary lesions at the end of an exhaustive diagnostic workup. Methods An electronic medical record search complemented by manual searching was conducted to identify prospective and retrospective cases of intracranial ACCs treated in Endoscopic Skull Base Centre Athens at the Hygeia Hospital, Athens from 2010 until 2021 with a mean follow-up time of at least 3 years. Patients were included if after complete diagnostic workup there was no evidence of a nasal or paranasal sinus primary lesion and extension of the ACC. All patients were treated with a combination of endoscopic surgeries performed by the senior author followed by radiotherapy (RT) and/or chemotherapy. Results Three unique illustrative cases (ACC involving the clivus, cavernous sinus and pterygopalatine fossa, one orbital ACC with pterygopalatine fossa and cavernous sinus involvement and one involving cavernous sinus, and Meckel's cave with extension to the foramen rotundum) were identified. All patients underwent subsequently proton or carbon-ion beam radiation therapy. Conclusions Primary intracranial ACCs constitute an extremely rare clinical entity with atypical presentation, challenging diagnostic workup and management. The design of an international web-based database with a detailed report of these tumors would be extremely helpful.

Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review.

Objective: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade cancer that was included from the 4th edition of WHO classification of head and neck tumours. The purpose of this study is to analyse clinical behaviour, pattern of recurrences and survival outcomes of this neoplasm. Methods: Retrospective review of patients affected by BSNS who were treated via an endoscopic-assisted approach in 6 European tertiary-care referral hospitals. Cases of BSNS described in literature since 2012 to date were fully reviewed, according to PRISMA guidelines. Results: A total of 15 patients were included. Seven patients were treated via an endoscopic endonasal approach, 4 with endoscopic transnasal craniectomy, and 4 via a cranio-endoscopic approach. Adjuvant treatment was delivered in 2 cases. After a mean follow-up of 27.3 months, systemic metastasis was observed in 1 case; the 5-year overall survival and disease-free survival rates were 100% and 80 ± 17.9%, respectively. Conclusions: BSNS is a locally aggressive tumour with a low recurrence rate and encouraging survival outcomes if properly treated with surgical resection and free margins followed by adjuvant radiotherapy for selected cases. Endoscopic-assisted surgery is safe and effective as an upfront treatment within a multidisciplinary care protocol.

Polyps, asthma, and allergy: what's new.

PURPOSE OF REVIEW: Although chronic rhinosinusitis with nasal polyps, asthma, and allergy share common inflammatory mechanisms, there is no evidence of cause-and-effect relationship. In this review, we present new studies investigating the complex immunology that links these diseases. Advances in new therapies as well as evidence regarding indication and timing of surgery, especially of more complex cases, are highlighted. RECENT FINDINGS: New studies have endotyped patients in an effort to describe the exact inflammatory profile of each phenotype, whereas described cytokines seem to play a significant role in amplification of T2 inflammation, directly or via innate lymphoid cells. New mAbs that block specific cytokines of these pathways have been developed and seem to show reduced asthma severity as well as improved sinonasal outcomes. Moreover, it has been shown that operating early in the course of disease leads not only to bigger improvements in SNOT-22 outcomes but also to reduced asthma incidence postoperatively in refractory cases. SUMMARY: Applying data from current studies in clinical practice, we could better manage refractory cases with asthma and polyps, both medically and surgically. Treatment has to be patient-centered, and this demands a multidisciplinary-team approach of the airway diseases.