Peri-operative prognostic factors for primary skull base chordomas: results from a single-center cohort.

BACKGROUND: Skull base chordomas (SBC) are rare malignant tumors and few factors have been found to be reliable markers for clinical decision making and survival prognostication. The aim of the present work was to identify specific prognostic factors potentially useful for the management of SBC patients. METHODS: A retrospective review of all the patients diagnosed and treated for SBC at the Fondazione IRCCS Istituto Neurologico "Carlo Besta" between January 1992 and December 2017 has been performed. Survival analysis was performed and a logistic regression model was used. Statistically significant predictors were rated based on their log odds in order to preliminarily build a personalized grading scale-the Peri-Operative Chordoma Scale (POCS). RESULTS: Fifty-nine primary chordoma patients were included. The average follow-up from the first treatment was 82.6 months (95% CI, 65.5-99.7). POCS was built over PFS and MR contrast enhancement (intense vs mild/no, value 4), preoperative motor deficit (yes vs no, value 3), and the development of any postoperative complications (yes vs no, value 2). POCS ranges between 0 and 9, with higher scores being associated with reduced likelihood of survival and progression-free state. CONCLUSIONS: Our results show that preoperative clinical symptoms (motor deficits), surgical features (extent of tumor resection and surgeon's experience), development of postoperative complications, and KPS decline represent significant prognostic factors. The degree of MR contrast enhancement significantly correlated to both OS and PFS. We also preliminarily developed the POCS as a prognostic grading scale which may help neurosurgeons in the personalized management of patients undergoing potential adjuvant therapies.

From Pregnancy to Lactation: When the Pathway is Complicated by Cancer.

BACKGROUND: Women with a cancer history report high distress during pregnancy and infant feeding. Despite the clear advantages of breastfeeding, little is known about factors influencing infant feeding behavior in women with cancer history. RESEARCH AIM: This three-time point longitudinal study aimed to explore the centrality of pregnancy and infant feeding experiences in 17 pregnant women with a cancer history (cases) compared to 17 pregnant women without cancer history (controls). METHODS: During pregnancy, participants filled out the Centrality of Events Scale and an ad hoc questionnaire about specific emotions, concerns, and expectations about infant feeding (T1), and their childbirth and infant feeding experiences during hospitalization (T2), and at 3-months postpartum (T3). RESULTS: Results at T1 demonstrated that participants with a history of cancer reported a higher perception of negative judgment and moral choice about breastfeeding than participants without a history of cancer. At T2 they reported a more positive childbirth experience than controls. From T2 to T3 participants with a history of cancer breastfed at a higher percentage than controls, and at T3 they reported higher levels of emotional and physical pleasure about the infant feeding experiences. CONCLUSIONS: Women with cancer history may experience a higher emotional and physical pleasure with infant feeding. Despite initial difficulties, a greater prevalence of breastfeeding was present for women with a history of cancer. Although this is a small sample, this research suggests that support and promotion of breastfeeding might be very effective after a serious medical diagnosis.

Evaluation of subclasses for T4-classified squamous cell carcinoma of the external auditory canal.

BACKGROUND: T4-classified squamous cell carcinoma (SCC) of external auditory canal (EAC) can potentially involve different anatomical structures, which could translate into different treatment strategies and survival outcomes within one classification. Our aim is to evaluate the clinical added value of T4-subclasses proposed by Lavieille and by Zanoletti. METHODS: Retrospective data, including patients with primary operated cT4-classified EAC SCC, was obtained from 12 international hospitals. We subclassified according to the T4-subclasses. The treatment strategies, disease-free survival (DFS) and overall survival per subclass were calculated. RESULTS: A total of 130 T4-classified EAC SCC were included. We found commonly used treatment strategies per subclass according to Lavieille and the DFS seems also to differ per subclass. Subclass according to Zanoletti showed comparable treatment strategies and survival outcomes per subclass. CONCLUSION: Our study suggests that the subclass according Lavieille might have added value in clinical practice to improve care of T4-classified EAC SCC.

Evidence-based surveillance protocol for vestibular schwannomas: a long-term analysis of tumor growth using conditional probability.

OBJECTIVE: The growth characteristics of vestibular schwannomas (VSs) under surveillance can be studied using a Bayesian method of growth risk stratification by time after surveillance onset, allowing dynamic evaluations of growth risks. There is no consensus on the optimum surveillance strategy in terms of frequency and duration, particularly for long-term growth risks. In this study, the long-term conditional probability of new VS growth was reported for patients after 5 years of demonstrated nongrowth. This allowed modeling of long-term VS growth risks, the creation of an evidence-based surveillance protocol, and the proposal of a cost-benefit analysis decision aid. METHODS: The authors performed an international multicenter retrospective analysis of prospectively collected databases from five tertiary care referral skull base units. Patients diagnosed with sporadic unilateral VS between 1990 and 2010 who had a minimum of 10 years of surveillance MRI showing VS nongrowth in the first 5 years of follow-up were included in the analysis. Conditional probabilities of growth were calculated according to Bayes' theorem, and nonlinear regression analyses allowed modeling of growth. A cost-benefit analysis was also performed. RESULTS: A total of 354 patients were included in the study. Across the surveillance period from 6 to 10 years postdiagnosis, a total of 12 tumors were seen to grow (3.4%). There was no significant difference in long-term growth risk for intracanalicular versus extracanalicular VSs (p = 0.41). At 6 years, the residual conditional probability of growth from this point onward was seen to be 2.28% (95% CI 0.70%-5.44%); at 7 years, 1.35% (95% CI 0.25%-4.10%); at 8 years, 0.80% (95% CI 0.07%-3.25%); at 9 years, 0.47% (95% CI 0.01%-2.71%); and at 10 years, 0.28% (95% CI 0.00%-2.37%). Modeling determined that the remaining lifetime risk of growth would be less than 1% at 7 years 7 months, less than 0.5% at 8 years 11 months, and less than 0.25% at 10 years 4 months. CONCLUSIONS: This multicenter study evaluates the conditional probability of VS growth in patients with long-term VS surveillance (6-10 years). On the basis of these growth risks, the authors posited a surveillance protocol with imaging at 6 months (t = 0.5), annually for 3 years (t = 1.5, 2.5, 3.5), twice at 2-year intervals (t = 5.5, 7.5), and a final scan after 3 years (t = 10.5). This can be used to better inform patients of their risk of growth at particular points along their surveillance timeline, balancing the risk of missing late growth with the costs of repeated imaging. A cost-benefit analysis decision aid was also proposed to allow units to make their own decisions regarding the cessation of surveillance.

Defining current practice patterns of vestibular schwannoma management in Italy: results of a nationwide survey.

OBJECTIVE: Despite the increasing incidence rate of vestibular schwannomas (VS), controversies in their management are still present. METHODS: A 35-item multiple-choice survey investigating the current practice patterns of VS care was sent to the members of the Italian Society of Otolaryngology, Head and Neck Surgery (SIO) and of the Italian Society of Neurosurgery (SINCH). RESULTS: Among 66 respondents, 37 (56.0%) claimed to be actively involved in VS management. Most interviewees (35.1%) declared > 20 years of experience and 59.5% claimed to work in an academic practice. The number of cases evaluated in each centre per year varied widely, with 54.0% evaluating > 25 cases/year and only 13.6% > 100 cases/year. Multidisciplinary care for VS evaluation was confirmed by 50.0% of respondents, and multidisciplinary surgical care by 62.2%. Observation and surgery were the most common management options proposed. Further details regarding VS care are presented. CONCLUSIONS: The present study provides the first overview on the current practice patterns of VS care in Italy. Although integrated in most centres, a multidisciplinary model of care needs to be encouraged. Wide heterogeneity in experience and practices is mostly influenced by the surgeon's different specialties and by the lack of shared guidelines.

Predictive Value of Preoperative and Intraoperative Neurophysiology in Evaluating Long-term Facial Function Outcome in Acoustic Neuroma Surgery.

OBJECTIVE: To determine the role of neurophysiological preoperative and intraoperative parameters in providing prognostic information regarding facial nerve (FN) function at 1 year after translabyrinthine acoustic neuroma (AN) resection surgery. STUDY DESIGN: Prospective observational study in a tertiary referral center. Patients treated via translabyrinthine surgical approach for sporadic AN microresection between December 2015 and 2018. METHODS: Patients underwent preoperative (electroneurography-ENG, electromyography-EMG, and Blink Reflex-BR) and intraoperative (FN motor action potential-MAP and continuous EMG traces) neurophysiological studies. FN function was graded postoperatively at 1 year using House-Brackmann Scoring System. RESULTS: Sixty-two patients were included in the analysis. Mean age was 53±10 years and average tumor diameter was 23 ±â€Š9 mm. At 1 year a normal facial function was observed in 68% of patients. In the univariate analysis a pathologic BR, low FN MAP values and ratios, and the presence of pathological neurotonic tracing (A-trains) on continuous EMG were associated with a poor facial nerve function outcome at 1 year postoperatively. Pathological preoperative BR testing and intraoperative A-trains showed a statistical significance also in the multivariable analysis, regardless of tumor size. CONCLUSIONS: Preoperative pathological BR testing and A-train activity on intraoperative EMG are correlated with poor FN outcomes at 1 year postoperative. This may provide important prognostic information to both patients and treating neuro-otologists. In the future this may guide preoperative and postoperative patient counselling and possibly optimize timing of facial nerve reanimation in selected patients.

Juvenile nasopharyngeal angiofibroma: evaluation and surgical management of advanced disease.

OBJECTIVE: Evaluate preoperative imaging in predicting operative stage. Describe the outcomes in surgically treated juvenile nasopharyngeal angiofibroma (JNA) with the influence of middle cranial fossa, carotid, or dural involvement on recurrence. STUDY DESIGN: Retrospective cohort of surgically treated patients with JNA. SUBJECTS AND METHODS: Eighty-five patients from a regional Italian referral center were assessed for recurrence, radiologic, and operative staging. High risk areas involved were recorded at surgery. RESULTS: Recurrence for advanced disease (IIIb+) was 18.2% (6 of 33) and 15.3% (13 of 85) overall. Preoperative staging poorly correlated with operative stage (P = 0.15). No single high risk area was predictive for recurrence, but the absence of any risk factor was associated with a favorable outcome (P < 0.01). CONCLUSION: Dural involvement by tumor is rare and imaging may overstage disease. Anterior access, endoscopic or open, is sufficient to address intracranial involvement. When an open approach is used, a midface degloving technique affords excellent exposure even for advanced disease. Lateral approaches with their associated morbidity can be reserved for selected recurrent disease.

Salvage surgery for recurrent nasopharyngeal carcinoma.

OBJECTIVES: To present our experience of salvage surgery for recurrent nasopharyngeal carcinoma after primary treatment by radiotherapy. PATIENTS AND METHODS: Eleven of 25 patient treated for nasopharyngeal carcinoma between 1990 and 2003 with radiotherapy had either residual or recurrent disease and underwent salvage surgery. The type C infratemporal fossa approach was used to access residual tumor. The patients' progress was followed by clinical examination and interval magnetic resonance scans. OUTCOME MEASURES AND RESULTS: The results were analyzed in terms of morbidity and oncological outcome; patients were recorded as NED (no existing disease), AWD (alive with disease), and DOD (died of disease). A disease-free survival rate of 72% was achieved in the salvage surgery group of patients and an overall disease-free survival rate of 56% applied to the initial cohort of 25 patients, following both the single mode and combined treatment. CONCLUSION: Salvage surgery is feasible for patients with recurrent nasopharyngeal carcinoma and may be achieved with minimal morbidity using the type C infratemporal fossa approach.

Lengthening Temporalis Myoplasty: Objective Outcomes and Site-Specific Quality-of-Life Assessment.

Objective Evaluate outcomes of the lengthening temporalis myoplasty in facial reanimations. Study Design Case series with planned data collection. Setting Ospedali Riuniti, Bergamo, and AOUC Careggi, Florence, Italy. Subjects and Methods From 2011 to 2016, 11 patients underwent lengthening temporalis myoplasty; demographic data were collected for each. Pre- and postoperative photographs and videos were recorded and used to measure the smile angle and the excursion of the oral commissure, according to the SMILE system (Scaled Measurements of Improvement in Lip Excursion). All patients were tested with the Facial Disability Index, and they also completed a questionnaire about the adherence to physiotherapy indications. Results All patients demonstrated a significant improvement in functional parameters and in quality of life. On the reanimated side, the mean z-line and a-value, measured when smiling, significantly improved in all patients: from 22.6 mm (95% CI, 20.23-25.05) before surgery to 30.9 mm (95% CI, 27.82-33.99) after surgery ( P < .001) and from 100.5° (95% CI, 93.96°-107.13°) to 111.6° (95% CI, 105.63°-117.64°; P < .001), respectively. The mean postoperative dynamic gain, passing from rest to a full smile at the reanimated side, was 3.1 mm (95% CI, 1.30-4.88) for the z-line and 3.3° (95% CI, 1.26°-5.29°) for the a-value. The Facial Disability Index score increased from a preoperative mean of 33.4 points (95% CI, 28.25-38.66) to 49.9 points (95% CI, 47.21-52.60) postoperatively ( P < .001). Conclusions The lengthening temporalis myoplasty can be successfully used for smile reanimation, with satisfying functional and quality-of-life outcomes.

Myringoplasty in simple chronic otitis media: critical analysis of long-term results in a 1,000-adult patient series.

OBJECTIVES: Investigate the recurrence of chronic otitis media after primary and revision myringoplasty, compare long-term anatomic and audiologic results of underlay and overlay myringoplasty, and examine the prognostic factors. STUDY DESIGN: Retrospective study. PATIENTS: Approximately 1,040 adult patients with chronic simple otitis media undergoing a myringoplasty (overlay and underlay) by different surgeons at a single institution (ENT Department of Bergamo Ospedali Riuniti) between May 1999 and March 2009. METHODS: The cumulative recurrence rate of chronic otitis media during 10-year follow-up period was calculated using a Kaplan-Meier survival analysis. A multivariate analysis was used to evaluate different prognostic factors based on long-term outcome in myringoplasty. RESULTS: The overall 10-year graft success rate was 78% in 1,040 patients. The 10-year recurrence rate of chronic otitis media was 15% in overlay myringoplasty and 26% using the underlay technique (p < 0.05). In revision myringoplasty, the overlay technique showed a better success rate than underlay (p < 0.05). Significant recovery was observed in the air conduction thresholds and air-bone gaps in both groups with no statistical difference between techniques (p = 0.1). Multivariate analysis demonstrated that the underlay myringoplasty technique, a pathologic contralateral ear and an anterior or subtotal perforation, using a perichondrial graft or age of surgery younger than 40 years were statistically significant (p < 0.01) factors that negatively influenced the myringoplasty outcomes. CONCLUSION: More successful outcomes in primary and revision surgery for chronic otitis media occurred using overlay myringoplasty, although there were more minor postoperative complications. Both clinical and technical variables affected the success rate of myringoplasty.

The problem of nodal disease in squamous cell carcinoma of the temporal bone.

CONCLUSION: The prognosis of the clinically positive neck in temporal bone squamous cell carcinoma is bad and failures never occur in the neck but at the site of the primary. A clinically positive neck at presentation is a sign of aggressiveness of the primary and, in such cases, therapeutic neck dissection is to be performed. The role of elective neck dissection is partially supported by our results, but as prognosis worsens and survival drops when a positive node appears in the neck, elective dissection is still advocated. In our material, no recurrence was recorded in the neck, but it was the resection of the primary tumor that proved to be oncologically inadequate. This led to the conclusion that in cases of clinically positive necks a more extended and adequate approach to the primary is thus advocated. OBJECTIVE: This work aimed to determine the prognostic role of positive neck nodes in squamous cell carcinoma of the temporal bone. METHODS: We studied the homogeneous case material of 47 surgical cases of temporal bone squamous cell carcinoma, operated on between the years 1983 and 2004. All the cases were reviewed retrospectively, staged according to the Pittsburgh classification, and the follow-up was updated at June 2008 with MRI. The minimum follow-up was set at 3 years. RESULTS: All the cases but two were treated with therapeutic or elective neck dissection. The neck was assessed both clinically and pathologically. The cases with clinically positive neck nodes at presentation had unfavorable prognosis. All the failures recurred at the site of the primary and never in the neck. The comparison of outcomes between the cases with clinically positive and negative necks showed a difference in survival. The rate of micrometastasis in the clinically negative neck was investigated as well. Our results indicate that though the necessity of elective neck dissection is still controversial, the bad prognostic value of the clinically positive neck is such to support the indication to treat the clinically negative neck.

A case of cervical metastases from temporal bone carcinoid.

BACKGROUND: Carcinoids are neuroendocrine tumors that typically occur in the gastrointestinal tract and lung and less frequently in the head and neck region. Whereas the metastatic potential of laryngeal carcinoid is well documented, only one case of metastasis of temporal bone carcinoid to the cervical lymph nodes has been reported. We present the case of a 55-year-old woman with cervical metastases almost 3 years after a primary diagnosis of temporal bone carcinoid. METHODS: The patient had undergone a subtotal petrosectomy for a temporal bone carcinoid and, 29 months later, revision surgery for a local recurrence. Four months later, multiple laterocervical swelling appeared, and the patient underwent modified radical neck dissection. RESULTS: Histologic examination after the neck dissection was suggestive of lymph node metastases from a carcinoid tumor. Immunohistochemical analysis of the lymph node metastases showed neoplastic cells positive for keratin, vimentin, chromogranin, and neuron-specific enolase and negative for thyroglobulin, calcitonin, S-100, and parathyroid hormone. The patient was treated with intensity-modulated radiation therapy and neck irradiation. CONCLUSIONS: We believe that temporal bone carcinoids have metastatic potential not predictable by histologic features. Surgical excision is the treatment of choice for patients with temporal bone carcinoids, with the approach and technique determined by the extent of the mass. Moreover, before planning surgery and during follow-up, neck node status must be carefully detected.