Sinonasal angioleiomyoma with adipocyte differentiation: clinicopathologic study of 2 cases and review of the literature

Angioleiomyoma (ALM) is a benign tumor of the skin and soft tissues composed of well differentiated smooth muscle cells arranged around and among many vascular channels. Few cases have been reported in the sinonasal region, where ALM may show a variable amount of mature adipose tissue that may make hard the distinction from angiomyolipoma. We report here two cases that presented with a three- and six-month history of left nasal obstruction respectively. In both patients, clinical examination and imaging studies failed to reveal features of tuberous sclerosis complex and demonstrated a mass obliterating the nasal cavity arising from the inferior turbinate. Both masses were endoscopically excised. Pathologic analysis revealed highly vascularized tumors composed of well differentiated smooth muscle cells intermingled with a variable number of mature adipocytes. Immunostaining for melanocytic markers was negative. Based on these findings, both tumors were diagnosed as sinonasal-ALM with adipocytic differentiation. These two cases indicate that both clinical data (i.e. absence of features of tuberous sclerosis complex) and immunohistochemistry (i.e. absence of melanocytic markers) are mandatory for the recognition of sinonasal-ALM with adipocytic differentiation. The term angiomyolipoma to identify these lesions is confusing and should be abandoned

Letter to the editor: autoimmune pathogenic mechanisms in amyotrophic lateral sclerosis

The innate immune system may affect the function and survival of motor neurons in ALS by at least three mechanisms. First, there is evidence to suggest that aggregates of mutant SOD1—which is derived from microglial and astroglial cells—activate neighbouring microglia by binding to TLR2, TLR4, and CD14, and subsequently promote neuronal cell death [9]. Second, the release of pro- inflammatory cytokines may drive motor neuron damage. Third, although poorly understood, a mechanism has been suggested on the basis of the functional analysis of microglial cells that express mutant SOD1 [10]. These cells showed impaired overall motility and a reduced capacity to clear neuronal cell debris. Impairment of microglial cell phagocytosis may therefore contribute to the accumulation of further immunostimulatory proteins, including mutant SOD1, chromogranin A, and dsRNA, thereby resulting in disease progression

Mucoepidermoid carcinoma associated with osteosarcoma in a true malignant mixed tumor of the submandibular region

True malignant mixed tumor, also known as carcinosarcoma, is a rare tumor of the salivary gland composed of both malignant epithelial and malignant mesenchymal elements. Frequently carcinosarcoma arises in the background of a preexisting pleomorphic adenoma; however, if no evidence of benign mixed tumor is present, the lesion is known as carcinosarcoma "de novo." We reported the first case of true malignant mixed tumor of the submandibular gland composed of high grade mucoepidermoid carcinoma associated with osteosarcoma. Case Presentation. A 69-year-old Caucasian male came to our department complaining of the appearance of an asymptomatic left submandibular neoformation progressively increasing in size over 3 months. We opted for surgical treatment. Histological examination confirmed the diagnosis of carcinosarcoma with the coexistence of high grade mucoepidermoid carcinoma and osteosarcoma. Conclusion. To the best of our knowledge, in the true malignant mixed tumor of the submandibular gland, mucoepidermoid carcinoma associated with osteosarcoma has never been previously reported

Letter to the editor: autoimmune pathogenic mechanisms in Huntington’s disease

Letter to the Editor: Autoimmune pathogenic mechanisms in Huntington's disease

Social geography of rhinoscleroma and qualitatively and quantitatively abnormal cell-mediated immunity

Rhinoscleroma is a progressive chronic granulomatous disease of the upper respiratory tract that may extend to the tracheobronchial tract. It is common belief that the pathology is determined by Klebsiella Rhinoscleromatis. In the authors' opinion, the infection with Klebsiella Rhinoscleromatis may not represent the only etiopathogenic factor of the disease. Rhinoscleroma is reported in many countries, but has a peculiar social and geographic distribution, in that it assumes an endemic character only in some regions of the Middle East, West Russia, North Africa, Indonesia, Central and South America. In Europe, most of the cases are reported in Poland, Hungary and Romania. In Italy, Rhinoscleroma is almost exclusively located in the southern and island regions. Rhinoscleroma is predominantly reported in rural areas, in the presence of poor socio-economic conditions, which according to many authors would be a co-factor triggering the disease. In this article, the authors review some inconsistencies in etiology, histology and epidemiology of Rhinoscleroma. Based on the overall picture, they propose that intrinsic factors, possibly of genetic origin, may give rise to the disease, and suggest possible lines of research to distinguish between extrinsic and intrinsic factors as determinants for Rhinoscleroma

CoQ10 and vitamin A supplementation support voice rehabilitation. A double-blind, randomized, controlled, three-period cross-over pilot study

Objectives: To evaluate the effectiveness of an adjuvant therapy (CoQ10 in its watersoluble form and vitamin A) in supporting voice rehabilitation in a large group of patients with muscle tension dysphonia (MTD). Study Design: Twelve-week, double-blind, randomized, controlled, three-period crossover pilot study. The primary endpoint was the change in the Dysphonia Severity Index (DSI) over the 12-week study period. Secondary endpoints were the changes in the subcomponents of DSI, including MPT, F0-high, I-low, and jitter. Exploratory endpoints were the changes in the Shimmer and in Voice Handicap Index (VHI). Methods: Patients were randomly assigned in a 1:1 ratio to two counter-balanced arms. Group A (ADJ-PLA) patients were administered QTer 300 mg and Vit A acetate 500.000 Ul/g 1 mg twice daily for a 4-week intervention period, followed by a 4-week period of wash-out, and then were submitted to a last 4-week period of placebo. Patients in Group B (PLB-ADJ) were given the treatment period in reverse order. Both groups received a 45-min voice therapy in a group format once a day for 4 weeks during the first and the second active periods. The therapy was held during the wash-out period. Results: The analysis of main time effect indicated a trend toward recovery of vocal function regardless of group assignment. A significant time by group effect was found on DSI [F = 3.4 (2.5, 80.5), p = 0.03], F0-high [F = 4.5 (2.6, 82.9), p = 0.008] and Shimmer [F = 3.6 (1.5, 46.9), p = 0.048], under CoQ10 and Vit A treatment, with a small effect size. There was no significant time by group effect on the other study measures, namely MPT, I-low, VHI. Conclusions: A trend toward recovery of vocal function was observed in all the patients, likely due to voice rehabilitation. The improvement of DSI was greater under CoQ10 and Vitamin treatment, indicating a more pronounced improvement of vocal quality under adjuvant therapy. The study protocol was reviewed and approved by the Ethics Committee of Policlinico Umberto I Hospital, Rome, Italy Rif. 3069/13.02.2014

Olfactory evaluation in obstructive sleep apnoea patients

The sense of smell has a high impact on the quality of life. The aim of the present study was to investigate olfactory dysfunction in patients with obstructive sleep apnoea syndrome (OSAS) and correlate the severity of disease with olfactory dysfunction. The relationships between nasal obstruction, nasal mucociliary cleareance and olfactory tests were also evaluated. Sixty patients with a diagnosis of OSAS were enrolled and underwent olfactory function evaluation. In all patients olfactory performance was tested with the Sniffin’ Sticks method. Mucociliary transport times and anterior rhinomanometry were performed to identify eventual nasal obstruction and deficits in nasal mucociliary clearance. Olfactory dysfunction was present in 22 (36.6%) patients of the study group: of these, hyposmia was present in 19 (86.4%) and anosmia in 3 (13.6%). The mean TDI score in the study group was 30. A strong correlation between the olfactory dysfunction and severity of sleep apnoea measured using the AHI was found. Patients with OSA would seem to have a high incidence of olfactory dysfunction. The degree of olfactory dysfunction appears to be related to the severity of disease. However, other co-factors such as nasal obstruction and reduced mucociliary clearance might also play a role in of the aetiology of this condition

Advances in the management of HPV-related oropharyngeal cancer

Patients with human papillomavirus- (HPV-) related oropharyngeal squamous cell carcinoma (OPSCC) have a better prognosis than HPV-negative OPSCC when treated with standard high-dose cisplatin-based chemoradiotherapy. Consistent with this assertion and due to younger age at diagnosis, novel approaches tominimize treatment sequelaewhile preserving survival outcomes become of paramount importance. Here, we critically reviewed the evidence-based literature supporting the deintensification strategies in HPV-related OPSCC management, including radiotherapy dose and/or volume reduction, replacement of cisplatin radiosensitising chemotherapy, and the use of transoral surgery. Undoubtedly, further researches are needed before changing the standard of care in this setting of patients

Tracheocutaneous fistula in patients undergoing supracricoid partial laryngectomy: the role of chronic aspiration

The aim of the present retrospective controlled study was to analyse and compare risk factors for tracheocutaneous fistula in patients who received tracheostomy after supracricoid partial laryngectomy with those who received tracheostomy for other causes. We enrolled 39 patients with tracheocutaneous fistulas who were divided into two groups. The first received temporary tracheostomy for supracricoid partial laryngectomies (n = 21), while the control group consisted of patients who received temporary tracheostomy for other causes (n = 18). Risk factors believed to play a role in the pathogenesis of tracheocutaneous fistula were examined including advanced age, cardiopathy, local infections, radiotherapy, elevated body mass index, malnutrition, decannulation time and aspiration grade. The Leipzig and Pearson scale score was significantly higher in the supracricoid partial laryngectomy group (p = 0.006 and 0.031 for univariate and multivariate analyses, respectively). The penetration/aspiration scale score was significantly higher in the supracricoid partial laryngectomy group as determined by univariate analysis (p = 0.014). The decannulation time was significantly lower in the supracricoid partial laryngectomy group (p = 0.004 and 0.0004 for univariate and multivariate analyses, respectively). The number of surgical closures for tracheocutaneous fistula was significantly higher in the supracricoid partial laryngectomy group by univariate analysis (p = 0.027). These results suggest that chronic aspiration and related cough may be important pathogenic factors for tracheocutaneous fistula and could be responsible for the significantly higher rates of closure failure in patients after supracricoid partial laryngectomy

Redescription of Cardiosporidium cionae (Van Gaver and Stephan, 1907) (Apicomplexa: Piroplasmida), a plasmodial parassite of ascidian haemocytes

Cardiosporidium cionae (Apicomplexa), from the ascidian Ciona intestinalis L., is redescribed with novel ultrastructural, phylogenetic and prevalence data. Ultrastructural analysis of specimens of C. intestinalis collected from the Gulf of Naples showed sporonts and plasmodia of C. cionae within the host pericardial body. Several merogonic stages and free merozoites were found in the pericardial body, together with sexual stages. All stages showed typical apicomplexan cell organelles, i.e. apicoplasts, rhoptries and subpellicular microtubules. Merogonic stages of C. cionae were also produced inside haemocytes. A fragment of the rSSU gene of C. cionae was amplified by PCR using DNA extracted from the pericardial bodies. The amplified product showed closest affinity with other apicomplexan representatives and a 66 bp unique insertion, specific for C. cionae, at position 1644. Neighbour-joining phylogenetic analysis placed C. cionae in a clade with other piroplasm genera, including Cytauxzoon, Babesia and Theileria spp. The parasite was found in different populations of C. intestinalis with highest prevalence in October–November. Ultrastructural and DNA data showed that the organism, described in 1907 from the same host but not illustrated in detail, is a member of a novel marine apicomplexan radiation of tunicate parasites