Salvage endoscopic nasopharyngectomy for recurrent nasopharyngeal carcinoma in a non-endemic area.

PURPOSE: To analyze oncological outcomes of endoscopic surgical treatment of locally recurrent EBV-related undifferentiated non-keratinizing nasopharyngeal carcinoma (uNK-NPC) in a non-endemic area. METHODS: Retrospective review of patients affected by recurrent uNK-NPC treated with nasopharyngeal endoscopic resection (NER) in a tertiary-care referral center from 2003 to 2022, by evaluating survival rates, prognostic factors, and follow-up strategies. RESULTS: The oncological outcomes of 41 patients were analyzed, over a mean follow-up period of 57 months. The 5-year overall, disease-specific, and disease-free survival of the cohort were 60.7% ± 8.9%, 69% ± 9%, and 39.7% ± 9.2%, respectively. The local (rT) and regional (rN) extension of recurrent disease, stage of disease, and status of resection margins appeared to significantly influence survivals. After a mean follow-up period of 21 months, a further recurrence after NER was observed in 36.6% of cases. Skull base osteonecrosis induced by previous irradiation and post-surgical bone remodeling represent the major challenges for early detection of further local relapses during postoperative follow-up. CONCLUSION: NER appeared as a safe and effective treatment for recurrent uNK-NPC. The adequate selection of patients eligible for NER is essential, to maximize the chances to cure and minimize the risk of local complications.

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.

Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.

OBJECTIVE: This paper highlights the management of 5 patients affected by symptomatic ecchordosis physaliphora (EP), treated via endoscopic endonasal transsphenoidal-transclival approach and contextual multilayer skull base reconstruction. A detailed analysis of each case is provided, along with the review of the current body of literature. METHODS: A retrospective review of patients treated by means of endoscopic endonasal approach for EP from 2010 to 2020 in the Otolaryngology and Neurosurgery Departments of a tertiary-care referral center for endoscopic skull base surgery was analyzed. Only adult patients with a definitive histopathological and immunohistochemical diagnosis of EP were included in the study. A systematic literature review according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed for EP. RESULTS: Five cases of EP were retrieved and included in the study. Four patients presented with CSF leakage: in two cases after minor head trauma, in one case with associated bacterial meningitis, and in one case as only referred symptom. One patient complained diplopia due to VI cranial nerve palsy. No complications or recurrences of the disease were observed after a median follow-up of 37.2 months (range, 18-72 months). A total of 27 studies were identified with the systematic literature review, encompassing 30 patients affected by symptomatic EP who were addressed to surgical treatment. Twenty-five patients underwent complete surgical removal of the EP, while in 5 cases, only subtotal resection was performed. CONCLUSIONS: EP might result in a "locus minoris resistentiae" of the skull base, predisposing, in rare cases, to CSF leakage and meningitis, spontaneously or after minor trauma. In cases of symptomatic EP, endoscopic endonasal transsphenoidal-transclival approach represents a safe and effective technique for both EP resection and contextual skull base reconstruction.

Medial femoral condyle free flap in combination with paramedian forehead flap for total/subtotal nasal reconstruction: Level of evidence: IV (therapeutic studies): Level of evidence: IV (therapeutic studies).

BACKGROUND: The nose is a functionally complex organ with also a critical role in aesthetics. For reconstruction of full thickness nasal defects, multiple stages are needed and there is risk for resorption resulting in residual deformity. The aim of this report was to develop and evaluate a new method for full thickness total/subtotal nose reconstruction using the medial femoral condyle free flap (MFCFF) in combination with a paramedian forehead flap. METHODS: Between November 2015 and January 2018, eight patients (four males, four females) mean age 52 years (range 40-73 years) undergoing a total/subtotal nasal excision and subsequential reconstruction with MFCFF plus paramedian forehead flap were enrolled. Six cases were squamous cell carcinomas while two were basal cell carcinomas. The MFCFF was stabilized, with the periosteum as inner layer, with plates and a paramedian forehead flap was used as external skin coverage. All patients were evaluated for with postoperative nasal endoscopy and CT scan. A postoperative questionnaire was given 6 months after surgery. RESULTS: The mean MFCFF size was 2-3.8 cm × 2.25-2.5 cm with a mean pedicle length of 6.3 cm (range 4.1-9.4 cm). The postoperative period was uneventful. The mean follow-up was 16 months, no bone displacement or resorption was observed at the CT scan, no evidence of nasal stenosis occurred. All patients had a satisfying aesthetic evaluation and a good subjective nasal function. CONCLUSIONS: In this series, the MFCFF in combination with the paramedian forehead flap appeared to provide a valid subtotal nose reconstruction, allowing for the recreation of all the three nasal layers and maintaining the nose projection and airway patency in the long term.

Frontal Sinus Preservation During Cerebrospinal Fluid Leak Repair.

BACKGROUND: To validate the repair of frontal sinus cerebrospinal fluid (CSF) leaks with preservation of frontal sinus drainage pathway (FSDP), independently of the approach used, and to establish the indications and limits of the endoscopic approach. METHODS: A retrospective evaluation of patients undergoing surgery for frontal sinus CSF leaks was performed. Demographics, indications for both endoscopic/combined endoscopic and open approaches, complications, methods for maintaining FSDP, and perioperative outcomes were examined. RESULTS: Fifty-three patients (average age 45 years) with frontal sinus CSF leaks were treated surgically from 2000 to 2018. All CSF leak repairs were successful at the 1st attempt with a mean follow-up of 76.8 months (range 4-227 months). Etiology was spontaneous (14), traumatic (26), and iatrogenic (13). In 17 patients, a purely endoscopic approach was performed, while an endoscopic endonasal orbital transposition procedure was added in 4 out of this group. In 23 patients, a combined endoscopic and osteoplastic procedure was employed, while in 13 patients a combined craniotomy and endoscopic procedure was performed. All frontal sinuses remained patent except for 2 cranialization patients. CONCLUSION: With contemporary endoscopic instrumentation and techniques, endoscopic closure of frontal sinus CSF leaks even in the far superolateral wall is feasible in selected patients. However, the surgeon should consider the option of combining an external approach when required. Preserving the FSDP should be the main aim regardless of the approach.

Endoscopic Treatment of Ewing Sarcoma of the Sinonasal Tract.

The extra-skeletal form is an unusual type of Ewing sarcoma (ES) arising from soft tissue and in the literature there are reports of less than 50 patients describing the tumor in the paranasal sinuses and skull base. The histological diagnosis is crucial to plan the correct treatment and the molecular confirmation is mandatory in equivocal patients. A multimodality treatment with chemotherapy, surgery and radiotherapy improved the outcomes of these diseases during the last decades and a free-margin resection with the endoscopic transnasal technique is one of the most recent ways to manage these pathologies in selected patients, reducing the morbidities of the external approaches and preserving the quality of life of the patient.Here, the authors present the first patient of primary sinonasal ES free from disease after 5 years of follow-up and treated with an endoscopic endonasal approach and a second patient of sinonasal metastases of ES treated with and endoscopic transnasal approach.

Comparative genomic hybridization on microarray (a-CGH) in olfactory neuroblastoma: Analysis of ten cases and review of the literature.

Olfactory neuroblastoma is a rare tumor arising from the basal layer of the olfactory epithelium in the superior recesses of the nasal cavity. The rarity of this tumor, and the difficulties in culturing tumor cells has limited the generation of conventional cytogenetic data, whereas consistent results have been obtained by recent molecular methods. We report the results of an array-based comparative genomic hybridization analysis (a-CGH) obtained on 11 samples from 10 subjects: 8 primary and 3 relapsed tumors. In one patient, both the primary and relapsed tumors were available. Our results on chromosome imbalances highlight the highly heterogeneous presentation: six of eleven samples showed multiple numerical changes and very few structural ones, while four samples showed an opposite pattern; one sample out of eleven showed no imbalances. We did not reach firm evidence of any recurrent specific imbalances either at level of entire chromosomes or chromosome segments. A review of the literature indicates a number of recurrent gains, and losses, mostly not confirmed by our results. Gain of chromosome 19 was the only correspondence with literature data concerning an entire chromosome, and most segmental gains and losses found in our cohort of patients were different from those indicated in the literature: the only similarities concerned the gain of 20q13 and the loss of segments of chromosomes 15 and 22.

Treatment strategies for primary early-stage sinonasal adenocarcinoma: A retrospective bi-institutional case-control study.

OBJECTIVE: To investigate different treatment strategies for primary early-stage (pT1-T2) sinonasal adenocarcinomas. METHODS: Retrospective case-control study. From 2000 to 2011, 61 cases were radically resected using an endoscopic endonasal approach. Surgery as a single treatment modality was adopted for 33 patients (study group) while it was followed by postoperative radiotherapy (poRT) in 28 patients (control group). RESULTS: Median follow-up was 61 and 67 months for the study and control group respectively. Patients were stratified according to the pT classification and no statistically significant differences were found in terms of Overall (OS) and Recurrence-free (RFS) survival. When analyzing the high-grade tumors (47 cases), statistically significant differences were observed between the control and study groups both in terms of OS (90.5% ± 6.5% versus 57.6% ± 15.4%, P = 0.03) and RFS (92.3% ± 7.39% versus 80.2% ± 8.88%, P = 0.05). Using multivariate analysis, OS was independently determined by poRT (Hazard Ratio = 0.16; P = 0.03) thus confirming its protective role for high-grade adenocarcinomas. CONCLUSION: Our preliminary results suggest that endoscopic endonasal surgery could be used as a single treatment modality for primary early-stage low-grade sinonasal adenocarcinoma, resected with negative margins. Surgery followed by poRT offers the best treatment strategy not only for advanced-stage lesions but also for high-grade adenocarcinomas, regardless of the stage of disease at presentation.

Newly designed double-vascularized nasoseptal flap to prevent restenosis after endoscopic modified Lothrop procedure (Draf III): laboratory investigation.

Despite advances in endoscopic approaches, instrumentations and imaging guidance systems, the management of frontal sinus is still challenging. Failure of the endoscopic procedure and restenosis of the newly formed ostium have been demonstrated even with large frontal sinusotomy. We describe a newly designed double nasoseptal flap to cover the bare bone after endoscopic modified Lothrop procedure (EMLP). Five fresh, double-injected cadavers were dissected through an endoscopic endonasal approach. Posteriorly based nasoseptal flap on one side and laterally based nasoseptal flap on the other side were harvested before performing wide EMLP. Feasibility of the procedure, versatility of the flap, coverage area and measurements were calculated. Harvesting the mucoperichondrial/mucoperiosteal flap over the septectomy site was straightforward step. Two different designed flaps (one on each side) are more practical to avoid torsion of the flap. The flap measures 2 × 3 cm on average that was able to cover the bare bone of the anterior and posterior wall of frontal sinus. Osteoneogenesis and progressive osteoplastic activity after EMLP plays a major role in restenosis of frontal sinus. Vascularized nasoseptal flap helps in preventing closure of the newly formed ostium. Applying these flaps over the bare bone enhances the healing process and minimizes the crust formation.

Palatovaginal (pharyngeal) artery: clinical implication and surgical experience.

The palatovaginal or pharyngeal artery is a small branch of the internal maxillary artery supplying the nasopharynx. Bleeding from this artery is exceptional and only one case of traumatic epistaxis from this artery has been reported previously. We report a case of a 66-year-old male presenting with right recurrent posterior epistaxis. Endoscopic dissection of the pterygopalatine fossa and direct visualization of the palatosphenoidal canal permitted to identify the origin of bleeding, and coagulation of the pharyngeal artery solved the epistaxis. Although rare, intractable posterior epistaxis may arise from the pharyngeal artery. The anatomical knowledge of this artery and of the palatosphenoidal canal is of outmost importance in endoscopic transpterygoid and nasopharyngeal procedures, to identify the vidian canal, evaluate nasopharyngeal cancer spread in the pterygopalatine fossa, reduce bleeding during surgery of the nasopharynx, and harvest adequately the pedicle of the nasoseptal flap.

Isolated laryngeal tuberculosis complex infection: expect the unexpected.

Laryngeal tuberculosis (LT), a rare but possible manifestation of extrapulmonary tuberculosis (TBC) and the most frequent granulomatous disease of the larynx, is slowly resurfacing due to the worldwide recrudescence of TBC. We present the case of a 59 y-o Caucasian woman, non-smoker, with no history of recent travels in endemic areas, affected by pulmonary sarcoidosis, that presented with a symptomatic vegetating lesion involving the left free margin of the epiglottic and a small, ulcerated lesion over the right arytenoid mucosa. While the patient's profile would not lead to a primary suspect of laryngeal TBC, the diagnostic workup and histological examination confirmed the unusual finding, and the patient was started on standard antitubercular therapy, with a complete laryngeal response. Although isolated laryngeal tuberculosis is still a rare finding, it should be kept into consideration also in non-endemic areas, especially in patients with chronic disease requiring immunosuppressive drugs.

A systematic review of randomised controlled trials on topical nasal steroids.

Introduction: Intranasal corticosteroids (INCs) are the first line of therapy for chronic sinonasal conditions such as rhinitis and rhinosinusitis. Among these, one of the most frequently used is beclomethasone dipropionate (BDP). Over the years many studies have evaluated the efficacy of BDP as part of therapy for chronic rhinosinusitis (CRS) and allergic rhinitis (AR) along with nasal washes, which seems to be very well tolerated. Objective: To analyse the data in the literature regarding the various therapeutic regimens of BDP in different sinonasal disease and their efficacy and tolerability. Materials and methods: Using different search engines, the posology, efficacy, and tolerability of BDP were reviewed and a total of 64 full-length articles were examined for eligibility. After applying inclusion and exclusion criteria, 4 articles were reviewed. Results: BDP is among the group of INCs with significant improvement of nasal symptoms and has good efficacy and safety. Conclusions: BDP nasal spray is one of the most frequently prescribed INC for rhinitis and rhinosinusitis. Treatment with BDP resulted in significant and clinically meaningful improvements in nasal symptoms associated with AR and CRS. BDP is well tolerated, and the safety profile is similar to that of placebo in most patients. These results, in conjunction with the significant benefit reported in subjects with CRS and AR, provide convincing evidence of the overall effectiveness of BDP for the treatment of the full spectrum of sinonasal disease.

Surgical management of advanced sinonasal cancer: a 10-year mono-institutional experience.

Objective: Endoscopic endonasal surgery is effective in the treatment of sinonasal cancers. However, in cases of well-differentiated locally advanced neoplasms as well as recurrences, the most appropriate treatment is debated. The purpose of this study is to report a mono-institutional experience on craniofacial surgery performed in a tertiary-care referral centre. Methods: This was a retrospective analysis of 90 patients treated with transcranial and/or transfacial resection for sinonasal cancer between 2010 and 2020. Outcome measures included overall survival (OS), disease-specific survival (DSS), disease-free survival (DFS) and recurrence-free survival (RFS). Results: The 5-year OS, DSS and DFS were 48.2%, 60.6% and 28.7%, respectively. Factors correlated with prognosis were pT-classification (p = 0.002), histotype (p = 0.012) and dural involvement (p = 0.004). Independent prognostic factors were orbital apex infiltration (p = 0.03), age (p = 0.002) and adjuvant therapy (p = 0.03). Conclusions: When endoscopic endonasal surgery is contraindicated and chemoradiotherapy is not appropriate, craniofacial and transfacial approaches still represent an option to consider, despite the non-negligible morbidity.

Nasoseptal flap with extended pedicle dissection based on the maxillary artery: Clinical series of 55 cases.

KEY POINTS: Nasoseptal flap with extended pedicle dissection is a low morbidity and high success rate flap. It is a flap that can be applied to reconstruct a wide range of ipsilateral skull base defects.

Endovascular coils extrusion after internal carotid artery occlusion: From management to follow-up.

KEY POINTS: ICA coil extrusion (ICA-CE) occurs most frequently in the nasopharyngeal/sinonasal site. Evaluating the ICA coils stability, through an angiography, is of primary importance. ICA-CE management needs to be decided based on the patient's symptoms and general status.

Endoscopic-assisted transorbital extended orbital exenteration: A multi-institutional preclinical study.

BACKGROUND: Sinonasal malignancies with orbital invasion have dismal prognosis even when treated with orbital exenteration (OE). Sugawara et al. developed a surgical strategy called "extended-OE (EOE)," showing encouraging outcomes. We hypothesized that a similar resection is achievable under endoscopic guidance through the exenterated orbit (endoscopic-EOE). METHODS: The study was conducted in three institutions: University of Vienna; Mayo Clinic; University of Insubria; 48 orbital dissections were performed. A questionnaire was developed to evaluate feasibility and safety of each step, scoring from 1 to 10, ("impossible" to "easy," and "high risk" to "low risk," respectively), most likely complication(s) were hypothesized. RESULTS: The step-by-step technique is thoroughly described. The questionnaire was answered by 25 anterior skull base surgeons from six countries. Mean, median, range, and interquartile range of both feasibility and safety scores are reported. CONCLUSIONS: Endoscopic-EOE is a challenging but feasible procedure. Clinical validation is required to assess real-life outcomes.

Comprehensive access strategies to the frontal sinus.

PURPOSE OF REVIEW: Despite the impressive evolutions in endoscopic endonasal approaches and instrumentations, the frontal sinus remains a challenging area. Different surgical options have been described over the years, but the main criticism lies in choosing the most suitable approach for a given case, based on the anatomy of each patient and the disease to treat. The purpose of this study is to provide a comprehensive review of surgical access strategies currently available to address the frontal sinus, including both endonasal and traditional external procedures, analysing indications, contraindications, complications and outcomes. RECENT FINDINGS: Frontal sinus surgery includes minimally invasive endonasal approaches (balloon dilatation, Draf type I); extended endonasal approaches (Draf type IIA-IIB-IIC, Draf type III and their modifications via orbital transposition and contralateral pyriform aperture resection); external procedures (superior eyelid incision, frontal osteoplastic flap, Riedel procedure, Riedel-Mosher operation); and combined approaches. SUMMARY: Recent advances in endoscopic endonasal techniques have deeply reshaped the surgical options to manage frontal sinus diseases, in an attempt to minimize the invasiveness of the procedures and maximize their outcomes. Traditional external procedures should be used in selected cases nonamenable for endonasal surgery. The appropriate selection of cases appears to be of paramount importance to obtain successful outcomes.

Case of IV Stage Juvenile Nasopharyngeal Angiofibroma Presurgically Treated with a Single ECA Stop-Flow Embolization Technique Using Onyx 18.

Background: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor that mainly affects young boys. Its intervention may be complex due to its high vascularity, location, and extension. Preoperative embolization is used to prevent intrasurgical and postsurgical bleeding. Two main kinds of embolization are described in literature: intratumoral and transarterial, and numerous embolic materials are used. Case Presentation. We want to present a case of presurgical embolization of a stage IV JNA, performed using a single stop-flow balloon assisted technique with the balloon cuffed exclusively in the external carotid artery and using Onyx 18 as an embolic agent. Conclusions: The embolization with an exclusive external carotid artery single stop-flow technique using Onyx 18 is a safe, effective, and a definitive approach.

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.