Exclusive or combined endoscopic approach to tumours of the lower lacrimal pathway: review of the literature.

A literature review was conducted to assess the role of minimally-invasive endoscopic treatments for lacrimal pathway neoplasms. The study involved the analysis of 316 patients with benign or malignant tumours affecting the lacrimal drainage system. Histologically, the analysis revealed a prevalence of squamous cell carcinoma, followed by lymphatic neoplasms and melanomas. In terms of treatment, minimally-invasive endoscopic approaches, such as endoscopic dacryocystorhinostomy, play a predominant role in managing early-stage tumours, rather than merely obtaining samples for histological analysis. For more extensive tumours, which constitute the majority of cases, more aggressive external approaches are required, along with the use of adjuvant radiotherapy and chemotherapy. The lack of universally shared staging systems poses a limitation in standardisation and comparison of results. Treatment of these tumours remains complex due to their rarity and histological heterogeneity. A multidisciplinary approach is mandatory to optimise outcomes.

Sinonasal Malignancies Involving the Frontal Sinus: A Mono-Institutional Experience of 84 Cases and Systematic Literature Review.

Frontal sinus involvement by malignant tumors is a rare finding. Therefore, a systematic literature review along with a personal case series may contribute to defining more accurately the epidemiology, treatment options, and outcomes of these neoplasms. This is a retrospective review of patients affected by frontal sinus malignancies surgically treated in a tertiary-care referral center over a period of 20 years. Moreover, a systematic literature review of studies describing frontal sinus cancers from 2000 to date was performed according to PRISMA guidelines in order to analyze current evidence about the treatment and outcomes of such a rare disease. Our retrospective review was basedon 84 cases, treated with an exclusive endoscopic approach in 43 cases (51.2%), endoscopic approach with frontal osteoplastic flap in 6 cases (7.1%), and transfacial or transcranial approaches in 35 cases (41.7%). The five-year overall, disease-specific, disease-free, and recurrence-free survivals were 54.6%, 62.6%, 33.1%, and 59.1%, respectively. Age, dural involvement, type of surgical resection, and surgical margin status were significantly associated with the survival endpoints. In conclusion, the involvement of the frontal sinus is associated with a poor prognosis. Multidisciplinary management, including specific histology-driven treatments, represents the gold standard for improving outcomes and minimizing morbidity.

Solitary fibrous tumor: A rare lesion with an unusual paravertebral presentation.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare spindle-cell neoplasm [1]. Although typically originating from pleura, head and neck presentation accounts for about 6-18 % and very few cases have been described in paravertebral and posterior neck spaces [2]. Both computed tomography (CT) scans and magnetic resonance imaging (MRI) help in differential diagnosis of such lesion [3]. However, only histological and immunohistochemical studies give a conclusive diagnosis: CD34, BCL-2, and in particular STAT6 stainings are strongly orientative [4]. Radical surgery with free margin excision is the first treatment option, rarely requiring adjuvant therapy [2]. Prognosis is typically good and strictly related to histological risk assessment [5]. Rare cases of local recurrence and distant metastasis have been described in literature [2]. OBJECTIVE: The purpose of this video is to describe the operative technique of a transcervical removal of a rare case of right paravertebral SFT. We present the diagnostic flowchart, management strategies, surgical technique and we provide anatomical dissection parallelism, which might be of interest to the readers. MATERIALS AND METHODS: A 59-years-old man with a one-year right cervical asymptomatic swelling was referred to our department. A contrasted MRI documented an expansive 6.5 cm capsulated lesion in the deep posterior neck spaces with diffuse contrast enhancement and inhomogeneous appearance. A core needle biopsy was performed, and the results from the initial immunohistochemical panel were not univocal showing positivity for cytokeratins AE1/AE3, PAX8, and no reactivity for CD34. A second immunohistochemical panel was then performed, displaying diffuse nuclear positivity for STAT6, which is a surrogate marker for the NAB2-STAT6 gene fusion, a specific driver mutation of SFT. Therefore, a radical excision was performed via transcervical approach (Video 1). No post-operative complications neither cranio-cervical neurological deficit occurred. RESULTS: In comparison to pre-operative histopathologic study, the definitive histological examination of the whole mass revealed a more classical morphology of SFT. It was classified as an intermediate risk SFT [5]. A complete free margin excision was confirmed. After a multidisciplinary discussion, no adjuvant therapies were suggested. A six- and twelve-months radiological follow-up with MRI showed no evidence of disease. CONCLUSION: SFT may represent a misdiagnosed entity in head and neck spaces and a correct diagnosis through immunohistochemistry is mandatory. Radical excision with free surgical margins should be pursued as adequate goal. Since SFTs show variable risk of metastatic disease, adjuvant radiotherapy should be contemplated in high-risk diseases and a clinico-radiological follow-up with MRI is required.

Molecular Biomarkers in Sinonasal Cancers: New Frontiers in Diagnosis and Treatment.

PURPOSE OF REVIEW: Sinonasal tumors are rare and heterogeneous diseases which pose challenges in diagnosis and treatment. Despite significant progress made in surgical, oncological, and radiotherapy fields, their prognosis still remains poor. Therefore, alternative strategies should be studied in order to refine diagnosis and improve patient care. RECENT FINDINGS: In recent years, in-depth molecular studies have identified new biological markers, such as genetic abnormalities and epigenetic variations, which have allowed to refine diagnosis and predict prognosis. As a consequence, new histological entities have been described and specific subgroup stratifications within the well-known histotypes have been made possible. These discoveries have expanded indications for immunotherapy and targeted therapies in order to reduce tumor spread, thus representing a valuable implementation of standard treatments. Recent findings in molecular biology have paved the way for better understanding and managing such rare and aggressive tumors. Although further efforts need to be made in this direction, expectations are promising.

Ectopic Primary Olfactory Neuroblastoma: Case Series and Literature Review.

OBJECTIVE: Olfactory neuroblastoma (ONB) is a rare malignant tumor arising in the upper nasal cleft. Rarely, ONB may originate in ectopic sites and the impact of this on prognosis and treatment strategies continues to be debated. METHODS: A retrospective analysis was undertaken of patients with ectopic ONB treated between 2000 and 2020 in a tertiary-care referral center for skull base tumors. Three patients were included in this analysis: a 37-year-old woman with ONB arising from the bulla ethmoidalis; a 28-year-old man with inappropriate secretion of antidiuretic hormone caused by a maxillary sinus ONB; and a 41-year-old man with lacrimal sac ONB. Preoperative workup, surgical approach, adjuvant treatments and postoperative surveillance were analyzed. Relevant literature published between 2000 and January 2021 was fully reviewed to investigate oncologic outcomes and delineate the standard of care for such rare tumors. RESULTS: All patients were treated via endoscopic endonasal resection with radical intent, followed by adjuvant treatments when required. No recurrences of disease were observed after a mean follow-up time of 32 months (range, 12-60 months). Data emerging from the literature suggest that a multidisciplinary treatment approach, including free-margins surgical resection followed by adjuvant radiotherapy or radiochemotherapy, is recommended. Olfactory bulb and dura preservation should be attempted whenever feasible. CONCLUSIONS: Endoscopic endonasal surgery should be preferred, when possible, to achieve complete excision to minimize patients' morbidity. The ectopic site of origin affects prognosis and should be considered when selecting the appropriate multimodal treatment strategy.

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.