Is Encapsulated Medullary Thyroid Carcinoma Associated With a Better Prognosis? A Case Series and a Review of the Literature.

Context: Medullary thyroid carcinoma (MTC) is a malignant neuroendocrine neoplasm that may spread to lymph nodes before the primary tumor is diagnosed; moreover, distant metastases are already present in about 10% of patients at diagnosis. Serum calcitonin (Ctn) usually reflects the spread of disease, thus orienting the extent of surgery and predicting the possibility of biochemical remission. Tumor size and vascular invasion are important prognostic factors, but little is known on the relationship between other histopathological features, such as the presence of a tumor capsule, and long term outcome of MTC. Purpose: To evaluate the prevalence of encapsulated tumors among MTCs and the association of tumor capsule with a favorable outcome after surgery. Methods: A retrospective observational single-center study was conducted together with a narrative review of the available literature. Results: Among 44 patients (27 female, 17 male; median age: 56 years) with MTC (6 hereditary, 37 sporadic) followed up at our center in the last four years (median follow-up: 29.2 months), seven (15.9%) showed an encapsulated tumor at histology and a clinical remission after surgery. None of them had nodal metastases and median preoperative Ctn (398 pg/mL, IQR 126.5-7336) did not differ significantly from that of the 14 patients (31.8%) with persistent disease after surgery (787 pg/mL, IQR 340.5-2905.5; p=0.633), although their tumor size was significantly higher (median 33 mm versus 16 mm respectively, p=0.036). Among patients with preoperative Ctn levels above 500 pg/mL (n=11), only two (18.2%) showed undetectable Ctn levels during follow-up, both having an encapsulated MTC (OR 0.000, p=0.02). Notably, they were two similar cases of large MTC (> 3 cm) with extensive hyalinization and calcification, associated with very high Ctn levels (> 13'500 and 1'100 pg/mL, respectively) but no nodal nor distant metastases, in complete remission after surgery although one of them carried the aggressive M918T somatic RET mutation. Conclusion: MTC rarely shows a tumor capsule, which seems to correlate with a better prognosis and absence of nodal metastases, regardless of RET or RAS mutational status. Among encapsulated MTCs (E-MTC), Ctn levels and tumor size are not predictive of persistence of disease after surgery.

Multidisciplinary management of congenital giant head and neck masses: Our experience and review of the literature.

BACKGROUND: Large fetal head and neck (HN) masses can be life-threatening at birth and postnatally owing to airway obstruction. The two most frequent congenital masses that may obstruct the airway are lymphatic malformation (LM) and teratoma. The aim of this paper was to evaluate the results of our experience in the management of giant congenital HN masses and to conduct a literature review. METHODS: The study involved a consecutive series of 13 newborns (7 females) affected by giant HN masses. Prenatal diagnosis was achieved by means of ultrasound (US) and fetal magnetic resonance imaging (MRI). Delivery was performed by means of EXIT procedure in case of radiological evidence of airway obstruction. In the postnatal period all feasible therapeutic options (surgery, sclerotherapy, medical therapy) were discussed and adopted by a multidisciplinary team. Twelve patients underwent surgery and one received Rapamycin for one month, with consequent surgical resection owing to increasing size of the mass. RESULTS: The histopathological diagnosis was LM in 11 cases and teratoma in 2 cases. Airway obstruction was solved in 11 cases; 2 LM patients required a tracheotomy because of persistent airway obstruction. Major complications were flap necrosis (one patient) and facial nerve palsy (2 cases). Recurrence occurred in 5 patients. CONCLUSIONS: The management of congenital HN masses is always challenging and necessarily requires an interdisciplinary approach. Current therapeutic options include surgery, sclerotherapy, medical therapy or a combination of them. When they are large enough to obstruct the airway, a patient-centered approach should guide timing and modality of treatment. LEVEL OF EVIDENCE: IV.

The 'full-blown' MRI of sudden hearing loss: 3D FLAIR in a patient with bilateral metastases in the internal auditory canals.

We report a case of a 57-year-old man with bilateral masses in the internal auditory canal. The peculiar findings at magnetic resonance imaging with tridimensional fluid-attenuated inversion recovery sequence combined with clinical data provided new insights into understanding the pathophysiology of the hearing loss.

Structural Fat Grafting to Improve Outcomes of Vocal Folds' Fat Augmentation: Long-term Results.

Objective Evaluating the long-term outcomes of vocal fold structural fat grafting. Study Design Case series with chart review. Setting University hospital. Subjects and Methods Seventy-nine dysphonic patients (16-82 years; 55 with unilateral laryngeal paralysis and 24 with vocal fold scarring) underwent vocal fold fat injection. Fat was harvested by low-pressure liposuction and then processed by centrifugation. Refined fat aliquots were placed in the vocal fold and paraglottic space in multiple tunnels to enhance graft neovascularization. All patients were followed for 12 months, 15 for 3 years, and 5 for 10 years with videolaryngostroboscopy, maximal phonation time (MPT) measurement, Voice Handicap Index (VHI) questionnaire, and GRBAS (grade, roughness, breathiness, asthenia, strain) perceptual evaluation. Laryngeal computed tomography (CT) and/or magnetic resonance imaging (MRI) studies were performed in 16 patients 3 to 28 months postoperatively; MRI was repeated in 5 cases 12 to 18 months after the first radiological study. Results The voice quality of all patients improved after surgery, and long-term stability was confirmed by MPT, GRBAS, and VHI ( P ranging between .004 and <.001). The results achieved 1 year postoperatively remained stable at 3 and 10 years. Videolaryn-gostroboscopy showed improved glottic closure in all patients despite a limited amount of fat resorption. CT and MRI demonstrated survival of the fat grafts in all of the 16 examined cases. Serial MRI scans showed no change in graft size over time. Conclusions The reported clinical and radiological data demonstrate that fat is an effective filler for permanent vocal fold augmentation if the refined micro-aliquots are placed in multiple tunnels.

Impact of adenotonsillectomy on pediatric quality of life: review of the literature.

Adenotonsillectomy (ADT) is one of the most widely used procedures in the treatment of paediatric recurrent acute tonsillitis (RAT) and obstructive sleep apnoea syndrome (OSAS), both of which have significant repercussions on the patients' quality of life (QoL). The purpose of our review of literature was to highlight the great variety of tools that are currently used to evaluate QoL in children, to examine data available on their efficacy and the feasibility of their use in daily clinical practice, and to determine possible limitations related to an indirect and subjective assessment of QoL in children.Although the use of different parameters makes it difficult to compare the published studies, an analysis of the evidence currently available in the literature suggests that ADT has a generally positive impact on the QoL (especially in case of OSAS). It also highlights the importance of combining tonsillectomy and adenoidectomy in the treatment of OSAS, and documents the comparability of tonsillectomy and tonsillotomy in improving obstructive symptoms. In conclusion, our findings suggest that literature supports that ADT is associated with positive changes in QOL; however further studies using comparable standardised criteria are necessary to confirm the size and duration of this benefit.

Ambulatory phonation monitoring in a sample of 92 call center operators.

OBJECTIVES: The voice is a primary work tool for call center operators, but the main risk factors for voice disorders in this category have not yet been clarified. This study aimed to analyze the vocal behavior in call center operators and search for correlations between the daily voice dose and the self-perceived voice-related handicap. STUDY DESIGN: Prospective. SUBJECTS AND METHODS: Ninety-two call center operators (aged 24-50 years) underwent ambulatory phonation monitoring during a working day and were administered the Voice Handicap Index (VHI) questionnaire and a questionnaire concerning smoking habits, symptoms, and extrawork activities requiring intensive voice use. RESULTS: Mean percentage phonation time (PT) during work was 14.74% and ranged from 4% to 31%. There was a significant difference between the percentage PT in working time and in extrawork time; however, subjects with high percentage PT in working time maintained a high value also in extrawork time. The mean PT was 87.5 ± 35.8 minutes and was not correlated with age, gender, number of work hours, symptoms, extraprofessional voice use, and VHI scores. The mean amplitude was significantly higher in subjects with longer PT and higher pitch (P < 0.001). VHI score (median = 9) was slightly higher than in the general population but not related to the number of work hours, indicating that work time was not a critical factor in causing the perception of voice problems. CONCLUSION: Our study provides data about the voice behavior of a large cohort of call center operators and demonstrates that the number of work hours and the percentage PT are not statistically related to the perception of voice disturbances in this working category.

Pharyngolaryngeal location of Kaposi's sarcoma with airway obstruction in an HIV-negative patient.

Kaposi's sarcoma (KS) is a human herpes virus-8 (HHV-8)-associated angioproliferative disorder, and its occurrence may be favored by human immunodeficiency virus (HIV) infection and iatrogenic immunosuppression. It has also been postulated that a chronic inflammatory disease of the skin can pave the way to its development. KS generally involves mucosal and cutaneous sites, including the head and neck. An oropharyngeal location is quite common, but laryngeal involvement with possible upper airway obstruction and respiratory distress requiring tracheotomy is rare, and no hypopharyngeal locations have yet been reported. We describe the case of a 68-year-old male patient who developed KS after immunosuppressive treatment for pemphigus vulgaris, an autoimmune bullous disease presenting with blisters and erosions on the skin and the oral mucosa. KS was initially localized to the oral cavity and oropharynx, but subsequent involvement of the laryngeal and hypopharyngeal tract led to acute airway obstruction and the need for tracheotomy. This unique case of pharyngolaryngeal KS suggests that clinicians faced with purple nodular lesions should consider a differential diagnosis of KS in immunocompromised patients, even if they are HIV negative, and should carefully manage the patency of the upper airways.

Head-to-head comparison of single-breath and tidal-breath exhaled nitric oxide measurements.

BACKGROUND: Exhaled nitric oxide (eNO) is an endogenous gas involved in airway pathophysiology and is determined in orally exhaled air by various techniques. However, traditional single-breath technique (eNO(SB)) requires active cooperation and is not always easily practicable (especially in young children); simpler techniques including tidal breathing measurements (eNO(TB)) are not standardized. The aim of this study was to evaluate the possible correlation and correspondence between eNO(SB) and eNO(TB) and the impact of potential confounders in children with chronic adenotonsillar disease. METHODS: Eighty-six children (mean age 8.7 ± 3.2 y) underwent eNO assessment by means of eNO(SB) and eNO(TB). The correlation among eNO(TB), eNO(SB), and other potential confounders (i.e., gender, age, weight, height, BMI, and passive smoking exposure) were studied. RESULTS: The analyses showed a poor correspondence between eNO(SB) and eNO(TB), with the latter underestimating (P < 0.001) mean eNO values: 6.4 parts per billion (ppb) (95% confidence interval (CI): 8.4-11.4 ppb) vs. 9.8 ppb (95% CI: 5.6-7.3 ppb). A greater correlation was found between eNO(SB) and eNO(TB) in children younger than 6 y. Only eNO(SB) and age predicted eNO(TB) (R2 = 43.6%). CONCLUSION: eNO(TB) is not a good predictor of eNO(SB) in children. Constant-flow eNO(SB) is the technique of choice for eNO assessment in young children.