Global frequency and distribution of head and neck sarcomas in adulthood: a systematic review.

PURPOSE: Head and neck sarcomas (HNS) constitute a rare and heterogeneous cancer entity. Management remains a challenge due their rarity and different biological behaviour among tens of subtypes. This systematic review aimed to describe HNS global frequency and distribution in adulthood. METHODS: A systematic review was performed using PICOTS search strategies for qualitative question and it was written in accordance with PRISMA 2020 Statement. 70,653 publications were identified, and 15 variables were evaluated for a total of 2428 patients. RESULTS: We identified 47 studies from 21 different countries from 5 different continents. Most of studies (83.3%) were performed in single institutions and America and Asia overruled for number of papers included (21 and 10, respectivelly). Osteosarcoma was more frequent, followed by chondrosarcoma, angiosarcoma and malignant fibrous histiocytoma. Early stage accounted for almost 80% of cases; advanced stage prevailed in developing countries. 1783 patients (90.1%) underwent surgery and 780 (39.4%) had adjuvant therapy. 50.8% of patients experienced tumour recurrence and the lowest mortality rate was reported in Europe (29.9%). CONCLUSIONS: HNS holds a relative poor prognosis possibly explained by the heterogeneity of the disease. Treatment of HNS has shown to be highly diverse among different countries, underlining the importance of uniformed treatment guidelines to achieve better patient management and to improve survival outcomes.

Calcitonin-secreting neuroendocrine tumor of the larynx, a diagnostic challenge of a rare neoplasm: a case report and literature review.

Background: Laryngeal neuroendocrine tumors (NETs) represent less than 1% of all malignancies originating from the larynx and available data are limited on case reports. Calcitonin secreting laryngeal NETs are extremely rare and serial dosing of calcitonin in these patients might reveal early relapse or persistence. Case Description: We report the case of a 71-year-old woman with persistent pharyngodynia who underwent surgery for an initial diagnosis of small cell undifferentiated neuroendocrine carcinoma (SCUNC) of the larynx (on the epiglottis extended to the left glosso-epiglottic vallecula). The immunohistochemical profile showed the presence of synaptophysin, neuron-specific enolase (NSE), chromogranin A, pan-cytokeratin, including cytokeratin AE1-AE2, and focally calcitonin. The circulating NSE was 13.4 microg/L (normal level <12.5 microg/L) and the basal serum level of calcitonin was 237 pg/mL (normal level <11.5 pg/mL). The patient was started on first-line carboplatin-etoposide chemotherapy because of early relapse to an axillary lymph node. After 4 cycles of treatment, a radiological stability and metabolic response were demonstrated together with a drastic decrease of circulating serum level of calcitonin (from 237 to 57.9 pg/mL). During the follow up, locoregional relapse of disease occurred, associated with an increase of serum calcitonin (89.3 pg/mL). Disease further progressed on and rechallenge with platinum-etoposide chemotherapy was administered, during which clinical progression was confirmed. Due to the lack of response, a revision of the histology was performed and concluded for a definitive diagnosis of moderately differentiated G2 NET, with a Ki-67 index of 22.6%. Conclusions: This is the eighth case report of laryngeal NET, highlighting the challenge in pathological differential diagnosis and therapeutic strategies. The association with elevated serum calcitonin and the trend of this parameter during clinical progression suggest a role of this marker in the diagnosis and early identification of recurrent laryngeal NETs.

SCIP flap in head and neck reconstruction after oncologic ablative surgery: a systematic review.

BACKGROUND: In the surgical treatment of head and neck locally advanced malignancies, microvascular free flaps represent the most valuable solution to reconstruct the tissue defect after resection of the primary neoplasm. In particular, microvascular free flaps allow to restore the functional and aesthetical features of the head and neck compartments. The superficial circumflex iliac perforator (SCIP) flap represents, as an evolution of the groin flap, a valid alternative to the radial fasciocutaneous free (RFFF) flap or the anterolateral thigh (ALT) flap. METHODS: This systematic review adhered to the recommendations of the Preferred Reporting Items of Systematic Reviews and Meta-analysis (PRISMA) 2009 guidelines. A computerized MEDLINE search was performed using the PubMed service of the U.S. National Library of Medicine ( www.pubmed.org ) and Scopus database ( www.scopus.com ). Two authors screened the articles, then selected and extracted data on malignancies characteristics, reconstructive techniques, outcomes, and complications. RESULTS: A total of 25 articles were selected and reviewed among the 39 identified through the search string. Six out of the selected 25 articles were case reports, while the remaining 19 articles were retrospective case series. The whole study population was represented by 174 oncologic patients undergoing ablation of a head and neck tumor and reconstruction with a SCIP flap. The site of reconstruction was the oral cavity in 125 (71.0%) patients, being the tongue the most common subsite in 73 (41.5%) patients, the pharynx in 10 (5.7%) cases, the larynx in 3 (1.7%) and head and neck skin in 36 (20.4%) patients. Only two cases of total flap loss were reported. Partial flap loss or shrinkage requiring minor surgical revisions was observed in 11 patients (6.32%). Primary closure of the donor site was achieved in the whole study population, according to the available data. CONCLUSIONS: In head and neck postoncological reconstruction, despite the caliber and the length of the pedicle, SCIP flap offers a pliable and thin skin paddle, allowing single-stage resurfacing, medium to large skin paddle, possibility of composite-fashion harvest and a well-concealed donor site.

Surgical anatomy of posterior tympanotomy: influence of the retrotympanum on round window exposure.

OBJECTIVES: To describe how the retrotympanic structures could influence the visibility of the round window niche and the round window membrane during cochlear implant surgery, and to investigate if a round window approach is possible even in cases with unfavourable anatomy. METHODS: Video recordings from 37 patients who underwent cochlear implantation were reviewed. The visibility of the round window niche and round window membrane at different timepoints was assessed according to a modified version of the Saint Thomas Hospital classification. The structures that concealed the round window niche and round window membrane were evaluated. RESULTS: After posterior tympanotomy, 54 per cent of cases had limited exposure (classes IIa, IIb and III) of the round window niche. After remodelling the retrotympanum, round window niche visibility significantly increased, with 100 per cent class I and IIa cases. Following remodelling of the round window niche, visibility of more than 50 per cent of the round window membrane surface was achieved in 100 per cent of cases. CONCLUSION: Remodelling the retrotympanum and the round window niche significantly increased exposure of the round window niche and round window membrane respectively, allowing round window insertion in all cases.

Radiologic evaluation and clinical assessment of facial sinus in adults and children - computed tomography study.

OBJECTIVE: The facial sinus is a recess of the lateral retrotympanum located between the chorda tympani (ChT) and facial nerve (FN). Chronic otitis media with cholesteatoma often spreads from the pars flaccida to the facial sinus (FS). In stapedotomy, if an unfavorable ChT type is encountered, there is a need for removal of bone between the ChT and FN. The aim of the study was to assess FSs in adults and children according to Alicandri-Ciufelli classification, to measure FS width and depth in computed tomography scans, evaluate the correlation between measurements and different types of facial sinuses, and provide a clinical context of these findings. METHODS: Cone Beam Computed Tomography (CBCT) of 130 adults and High Resolution Computed Tomography of 140 children were reviewed. The type of facial sinus was assessed according to Alicardi-Ciufelli's classification in different age groups. Width of entrance to facial sinus (FSW) and depth of FS (FSD) were evaluated among age groups. RESULTS: Type A of FS is dominant in both adult and children populations included in the study. The average depth of FS was 2.31±1.43 mm and 2.01±0.90 in children and adults respectively. The width of FS was 3.99±0.69 and 3.39±0.98 in children and adults respectively. The depth of FS (FSD) presented significant deviations (ANOVA, p<0.05) among all three types and age groups. In 116/540 (21.5%) cases the value of FSD was below 1 mm. CONCLUSION: The qualitative classification of facial sinuses into types A, B and C, introduced by Alicandri-Ciufelli and al. is justified by statistically significant differences of depth between individual types of tympanic sinuses. Type A sinuses may be extremely shallow (<1 mm - As) or normal (>1 mm - An). Preoperative assessment of CT scans of the temporal bones gives crucial information about type and size of facial sinus. It may increase the safety of surgeries in this area and play a role in choosing an optimal approach and surgical tools.

The transcervical-transparotid corridor for management of parapharyngeal space neoplasms: strengths and limits in a bi-institutional retrospective series.

BACKGROUND AND PURPOSE: Parapharyngeal space (PPS) neoplasms represent 1% of all head and neck tumors and are mostly benign. Surgery is the mainstay of treatment and the transcervical-transparotid (TC-TP) corridor still represents the workhorse for adequate PPS exposure. Our series investigates strengths and limits of this approach on a multi-institutional basis. METHODS: We reviewed consecutive patients submitted to PPS surgery via TC-TP route between 2010 and 2020. Hospital stay, early and long-term complications, and disease status were assessed. RESULTS: One hundred and twenty nine patients were enrolled. Most tumors were benign (79.8%) and involved the prestyloid space (83.7%); the median largest diameter was 4.0 cm. The TC-TP corridor was used in 70.5% of patients, while a pure TC route in about a quarter of cases. Early postoperative VII CN palsy was evident in 32.3% of patients, while X CN deficit in 9.4%. The long-term morbidity rate was 34.1%, with persistent CN impairment detectable in 26.4% of patients: carotid space location, lesion diameter and malignant histology were the main independent predictors of morbidity. A recurrence occurred in 12 patients (9.4%). CONCLUSIONS: The TC-TP corridor represents the benchmark for surgical management of most of PPS neoplasms, though substantial morbidity can still be expected.

A Retrospective Multicenter Italian Analysis of Epidemiological, Clinical and Histopathological Features in a Sample of Patients with Acinic Cell Carcinoma of the Parotid Gland.

BACKGROUND: The acinic cell carcinoma (AciCC) of the parotid gland is a rare tumor with an indolent behavior; however, a subgroup of this tumor presents an aggressive behavior with a tendency to recur. The aim of this multicenter study was to identify and stratify those patients with AciCC at high risk of tumor recurrence. METHODS: A retrospective study was carried out involving 77 patients treated with surgery between January 2000 and September 2022, in different Italian referral centers. Data about tumor characteristics and its recurrence were collected. The histological specimens and slides were independently reviewed by a senior pathologist coordinator (L.C.) and the institution's local head and neck pathologist. RESULTS: The patients' age average was 53.6 years, with a female prevalence in the group. The mean follow-up was 67.4 months (1-258, SD 59.39). The five-year overall survival (OS) was 83.2%. The 5-year disease-free survival (DFS) was 60% (95% CI 58.2-61.7). A high incidence of necrosis, extraglandular spread, lymphovascular invasion (LVI), atypical mitosis, and cellular pleomorphism was observed in the high-risk tumors compared to the low-risk ones. CONCLUSION: AciCC generally had an indolent behavior, optimal OS, DFS with few cervical node metastases, and rare distant relapses. This multicenter retrospective case series provides evidence of the need for clinical-epidemiological-histological stratification for patients at risk of poor outcomes. Our results suggest that the correct definition of high-risk AciCC should include tumor size, the presence of necrosis, extraglandular spread, LVI, atypical mitosis, and cellular pleomorphism.

Multilayer Anterior Skull Base Reconstruction with Cortical Rib Bone Graft: Preliminary Experience.

OBJECTIVE: During the past decades, different methods have been described for anterior skull base reconstruction. Regarding larger skull base defects, few investigators have described the use of bone grafts to foster support and prevent frontal lobe sagging, herniation, or falling. The aim of this study is to describe the use of a rib bone graft, which could be an option in these cases due to its rigidity and dimensions. METHODS: We retrospectively collected preoperative, intraoperative, and postoperative data at the last follow-up of 10 patients who underwent multilayer anterior skull base reconstruction, including rib bone graft, for large anterior cranial base defects at 2 tertiary care academic hospitals. RESULTS: Eight patients underwent endoscopic craniectomy for sinonasal malignancies, and the other two underwent transnasal endoscopic surgery for congenital meningoencephalocele. Anterior skull base defects measured on average 3.8 cm ± 0.9 SD antero-posteriorly (range 2.5-5 cm) and 2.3 ± 0.9 SD latero-laterally (range 0.9-4 cm). Multilayer reconstruction was performed in all cases, including a rib bone graft positioned as intracranial extradural layer. No patient experienced thoracic complications during the postoperative period. No side effects related to the bone graft or meningoencephalocele occurrence were reported after a mean follow-up of 8.0 ± 6.3 months. CONCLUSIONS: The use of a cortical rib bone graft could be a safe and effective option in skull base reconstruction when managing large defects after cancer removal.

Impact of lymph node ratio and number of lymph node metastases on survival and recurrence in laryngeal squamous cell carcinoma.

INTRODUCTION: The aim of this study is to assess the impact of lymph node ratio (LNR) and number of positive lymph nodes (NPLN) on mortality and recurrence rates in patients with laryngeal squamous cell carcinoma. MATERIALS AND METHODS: We conducted a retrospective multicenter international study involving 24 Otorhinolaryngology-Head and Neck Surgery divisions. Disease-specific survival (DSS) and disease-free survival (DFS) were evaluated as the main outcomes. The curves for DSS and DFS according to NPLN and LNR were analyzed to identify significant variations and establish specific cut-off values. RESULTS: 2507 patients met the inclusion criteria. DSS and DFS were significantly different in the groups of patients stratified according to LNR and NPLN. The 5-year DSS and DFS based on LNR and NPLN demonstrated an improved ability to stratify patients when compared to pN staging. CONCLUSION: Our data demonstrate the potential prognostic value of NPLN and LNR in laryngeal squamous cell carcinoma.

Paraglottic Space Invasion in Glottic Laryngeal Cancer: A Clinical-Pathological Study.

OBJECTIVE: This study aims to prospectively compare endoscopic, radiological, and pathological features of a cohort of patients with glottic laryngeal squamous cell carcinoma (LSCC) undergoing open partial horizontal laryngectomy (OPHL) type II/III or total laryngectomy to better understand the reliability of preoperative endoscopy and computed tomography (CT) to predict the inferior paraglottic space (iPGS) involvement. METHODS: We prospectively compared the endoscopic, radiological, and pathological findings in patients with glottic LSCC who underwent OPHL II/III, or total laryngectomy. RESULTS: Endoscopy achieved a diagnostic accuracy of 87.2% for the anterior iPGS (iPGSa) and 86.1% for the posterior iPGS (iPGSp). There was no statistically significant difference in terms of histopathologic iPGSa involvement between reduced (85%-17/20 pts) and absent (92%-24/26 pts) vocal cord mobility (p = 0.39). CT alone did not improve the diagnostic performance of the endoscopy, reaching a diagnostic accuracy of 62.9% and 73.7% for the iPGSa and iPGSp, respectively. When endoscopy and CT were combined, the diagnostic performance improved for the iPGSp, achieving a sensitivity (Se), specificity (Spe), positive predictive value (PPV), and negative predictive value (NPV) of 100%, 89.8%, 68.7%, and 100%, respectively. On the contrary, the combination of CT and endoscopy improved only the Se and NPV for the iPGSa with respect to the sole endoscopic assessment. CONCLUSIONS: Whenever motility impairment is present, a histopathologic invasion of the iPGS should be suspected. Endoscopic assessment of laryngeal motility achieved a satisfactory value of Se, Spe, PPV, and NPV in predicting the involvement of the iPGS. CT scan is still the mainstay imaging technique in the clinical staging of patients with LSCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:1184-1190, 2023.

Oropharyngeal squamous cell carcinoma: Prognostic factors for development of distant metastases and oncological outcomes.

BACKGROUND: Distant metastasis (DM) development in Oropharyngeal Squamous Cell Carcinoma (OPSCC) represents an important prognostic factor. The identification of a phenotype of metastatic patients may better define therapeutic and follow-up programs. METHODS: We included 408 patients with OPSCC, non-metastatic at the time of diagnosis, and treated with curative intent. The Overall Survival (OS) analyses were performed and the impact of developing DM on survival was analyzed through Cox proportional-hazard regression model. RESULTS: 57 (14%) patients develop DM. 302 (74%) were p16+ OPSCC and 35 of them experienced DM. Advanced clinical stage, smoking, p16-status, response to primary treatment, and loco-regional relapse influence the DM rate. Only in the p16+ group, DM onset results in a greater impact on OS (p < 0.0001). Lung metastases have a better OS compared to non-pulmonary ones (p = 0.049). CONCLUSION: This retrospective study shows a possible stratification of OPSCC patients based on the risk of the development of DMs.

Oncological and Functional Outcomes for Horizontal Glottectomy: A Systematic Review.

Horizontal glottectomy (HG) is a particular type of partial laryngectomy indicated for exclusive glottic tumor with anterior commissure involvement. The purpose of this study is to systematically review the literature about functional and oncological outcome of HG. This systematic review adhered to the recommendations of the PRISMA (Preferred Reporting Items of Systematic Reviews and Meta-analysis) 2009 guidelines. Articles mentioning patients undergoing HG for laryngeal squamous cell carcinoma were included. A total of 14 articles were selected and reviewed from 19 identified. The whole study population consisted of 420 patients who underwent HG. Three hundred and thirty-nine patients out of 359 were staged as T1. The range of post-operative follow-up was 5 months to 10 years. Fifty-five recurrences were experienced, being local, regional and distant in 35, 12 and 8 patients, respectively. Laryngeal preservation rate was 93.6%. Nasogastrict tube was removed on average after 10.1 days. The tracheostomy was maintained for 11.3 days. Mean hospitalization lasted for 11.7 days. According to the results of this systematic review, HG is an oncologically safe surgical option for T1a-T1b glottic tumors with oncological outcomes comparable to other treatment. HG could be a good therapeutical choice whenever poor laryngeal exposure and/or patient's refusal of radiotherapy are encountered, or when patient's medical history represents a contraindication for radiation therapy.

Prognostic role of EAONO/JOS, STAMCO, and ChOLE Staging for Exclusive Endoscopic and Endoscopic-Microscopic Tympanoplasty.

OBJECTIVE: The aim of the study is to evaluate cholesteatoma's surgical outcomes in patients treated with endoscopic ear surgery (EES) or a combined endoscopic-microscopic approach (cEMA) according to STAM, STAMCO, ChOLE, and EAONO/JOS system (EJS) classifications and staging. STUDY DESIGN: Retrospective study. SETTING: Monocentric study in a tertiary referral center. METHODS: One-hundred sixty-eight patients who underwent EES or cEMA for cholesteatoma between 2010 and 2018 were classified according to the abovementioned classification and staging. Data on cholesteatoma's recurrence and residual rates were collected. Inferential statistical analysis was performed to evaluate surgical outcomes and the prognostic value of classifications and staging. RESULTS: The recurrence rate was significantly lower in cholesteatomas classified in EJS stage 1 (2.6%) and STAM stage 1 (0%). A comparison of the different stages of the disease showed a significantly lower recurrence only for stage 1 versus the superior stages of both classifications. Involvement of mastoid bone was associated with a higher risk of recurrence (odds ratio [OR]: 4.12; p = .031). Attical involvement was associated with a higher risk of residual cholesteatoma (OR: 1.165; p = .046). CONCLUSION: EES or cEMA represents an effective treatment for middle ear cholesteatoma. The STAM classification and the EJS have shown a prognostic value, with STAM 1 and EAONO-JOS 1 stages associated with a better prognosis. Mastoid involvement represents a risk factor for recurrence. Attic localization is associated with residual disease. Localization at difficult access sites did not implicate a higher risk for recurrence or residual. ChOLE classification, Ossicular chain status, and complication status did not provide prognostic information regarding recurrence or residual cholesteatoma.

Diagnostic and Prognostic Value of microRNAs in Patients with Laryngeal Cancer: A Systematic Review.

Laryngeal squamous cell cancer (LSCC) is one of the most common malignant tumors of the head and neck region, with a poor survival rate (5-year overall survival 50-80%) as a consequence of an advanced-stage diagnosis and high recurrence rate. Tobacco smoking and alcohol abuse are the main risk factors of LSCC development. An early diagnosis of LSCC, a prompt detection of recurrence and a more precise monitoring of the efficacy of different treatment modalities are currently needed to reduce the mortality. Therefore, the identification of effective diagnostic and prognostic biomarkers for LSCC is crucial to guide disease management and improve clinical outcomes. In the past years, a dysregulated expression of small non-coding RNAs, including microRNAs (miRNAs), has been reported in many human cancers, including LSCC, and many miRNAs have been explored for their diagnostic and prognostic potential and proposed as biomarkers. We searched electronic databases for original papers that were focused on miRNAs and LSCC, using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) protocol. According to the outcome, 566 articles were initially screened, of which 177 studies were selected and included in the analysis. In this systematic review, we provide an overview of the current literature on the function and the potential diagnostic and prognostic role of tissue and circulating miRNAs in LSCC.

The role of adjuvant therapy in pT4N0 laryngectomized patients: Multicentric observational study.

BACKGROUND: To retrospectively evaluate oncological outcomes in two groups of patients with pT4aN0 glottic SCC treated with total laryngectomy (TL) and neck dissection (ND) who underwent postoperative radiotherapy or exclusive clinical and radiological follow-up. METHODS: It includes patients with pT4N0 glottic SCC who underwent TL and unilateral or bilateral ND with or without PORT. Divided in two comparison groups: the first group underwent adjuvant RT (TL-PORT); the second group referred to clinical and radiological follow-up (TL). RESULTS: PORT was associated with a better OS while no differences were found in terms of DSS. A better local control is achieved when PORT is administered while no differences in terms of regional and distant control rates were found. Bilateral ND positively impacts on the regional control while the PNI negatively impact the regional control. CONCLUSIONS: A tailored PORT protocol might be considered for pT4N0 glottic SCC treated with TL and ND, both considering the ND's extent and presence of PNI.

Expanded transcanal transpromontorial approach for acoustic neuroma removal.

Axial sections from preoperative magnetic resonance imaging without contrast, showing a cone-shaped lesion of the internal auditory canal, extending toward the most lateral part of the cerebello-pontine angle. (A) T1-weighted high-resolution isotropic volume excitation (THRIVE) sequence; (B) T1-weighted sequence; (C) Fluid attenuated inversion recovery (FLAIR) sequence. Laryngoscope, 133:282-286, 2023.

Microsurgical training using an ex-vivo model: microscope vs 3D exoscope.

Objective: The aim of this study is to evaluate the feasibility of the 3D exoscope in a microvascular anastomosis training setting and compare it with the gold-standard technique using the operating microscope (OM). Methods: Participants were recruited among otorhinolaryngology head and neck surgery (OHNS) residents of two tertiary care hospitals. Trainees were asked to complete 4 microvascular end-to-end anastomoses on chicken thighs with the OM and VITOM 3D exoscope. The performances were scored by experienced microvascular surgeons; an objective evaluation of the anastomosis and a subjective assessment of the workload were conducted. Results: 8 OHNS residents were recruited. Considering the amount of time needed to complete (TTC) the anastomosis, an improvement was shown by all the participants throughout the training program. The objective evaluation of the anastomosis did not show a significant difference. No significant differences were found by analyzing the subjective workload with the different tools. Conclusions: This article represents the first attempt to compare the use of the OM and the 3D exoscope during training for microsurgery. The results of our study demonstrate the noninferiority of microsurgical training obtained using the 3D exoscope compared to that offered by the OM.