Head and Neck Medicine by Alexandros G.Sfakianakis: 09/24/17

Mucosal melanoma is a rare aggressive cancer with a very poor prognosis. Clinical and pathological characteristics of mucosal melanoma differ from those of cutaneous melanoma and there are no established management guidelines for mucosal melanoma. Complete surgical excision is one of the most effective treatments for localized lesions, while targeted therapies and immunotherapies, such as monoclonal antibodies that target cytotoxic T-lymphocyte-associated molecule-4, and the programmed death (PD)-1/PD-ligand 1 pathway inhibitors, are treatment options for unresectable or metastatic lesions. Here, we describe the case of a patient with oral mucosal melanoma with multiple metastases. In our case, local injection of interferon (IFN)-β with dacarbazine–nimustine–vincristine therapy provided antitumor effects on an invasive tumor on the upper gingiva. Nivolumab therapy produced complete remission of lymph node and bone metastases. In contrast, the remaining in situ portion of oral mucosal melanoma on the hard palate was refractory to IFN-β monotherapy and nivolumab therapy. However, after administration of nivolumab, peritumoral injection of IFN-β showed rapid therapeutic effects. Our case suggested that nivolumab upregulated the antitumor effects of IFN-β, which induced the recruitment of CD8⁺ T cells into the tumor microenvironment contributing to the deletion of tumor cells. Combination therapy of IFN-β and nivolumab may be a potential treatment option for patients with oral mucosal melanoma.

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Immune reconstitution inflammatory syndrome in non-HIV immunosuppressed patients

Abstract

Immune reconstitution inflammatory syndrome (IRIS) represents a clinical phenomenon of immune-mediated inflammation against various antigens, including pathogenic microorganisms, drugs and unknown autoantigens, during recovery from immunosuppressed conditions. IRIS has become well recognized in HIV-infected populations. However, IRIS has seldom been recognized in HIV-negative immunocompromised patients. In the last 15 years, the immunopathogenesis of drug-induced hypersensitivity syndrome (DIHS) has been largely determined. Laboratory data and clinical observations support the idea that DIHS represents a prototype of non-HIV IRIS. Primary diseases in which non-HIV IRIS is secondary include severe cutaneous adverse drug reactions, such as DIHS, autoimmune diseases, collagen diseases, pregnancy and internal malignancies. Potential triggers of recovery from an immune deterioration state include a discontinuation or abrupt tapering of systemic steroids and/or immunosuppressants, withdrawal or reduced effects of anti-tumor necrosis factor-α antibodies, and the use of immune-checkpoint antagonists for the advanced stages of malignancies. Wide use of IRIS across large populations risks oversimplification but highlights a key unifying principle. Balanced sensitivity and specificity for its diagnostic criteria and classification are necessary for the establishment of clinical practice guidelines for non-HIV IRIS. Additionally, the development of a useful combination of biomarkers is currently an urgent issue.

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Bullous pemphigoid induced by ipilimumab in a patient with metastatic malignant melanoma after unsuccessful treatment with nivolumab

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Lymph node metastatic melanoma from ungual melanoma: Identification of somatic mutations in KIT and LZTR1

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Anaphylaxis due to ingestion of jellyfish with possible evidence of epicutaneous sensitization

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Adult Langerhans cell histiocytosis with pulmonary and colorectoanal involvement: a case report

Langerhans cell histiocytosis is a rare systemic disease characterized by the abnormal overproduction of histiocytes that tend to infiltrate single or multiple organ systems leading to significant tissue damag...

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Endoscopic enucleation of large jaw cysts: Promising outcomes

To describe the endoscopic approach for management of large jaw cysts and assess the feasibility of endoscopic enucleation as well as analyze its outcome on a wide scale at Mansoura University Hospital MUH.

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Synchronous Ganglioneuroma and Schwannoma Mistaken for Carotid Body Tumor

Ganglioneuromas are a very rare benign neural tumor, commonly derived from the ganglia of the sympathetic system, and are composed of mature Schwann cells, ganglion cells, and nerve fibres. They may arise anywhere from the base of the skull to the pelvis along the paravertebral sympathetic plexus. We report a rare case of synchronous ganglioneuroma and schwannoma, mistaken for carotid body tumor. The coexistence of these two entities in head and neck region is very rare.

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Endoscopic enucleation of large jaw cysts: Promising outcomes

Publication date: Available online 24 September 2017
Source:Auris Nasus Larynx
Author(s): Mohamed Abdelwahab, Ahmed Musaad Abd Elfattah, Yasser W. Khafagy, Ahmed El-Degwi
ObjectiveTo describe the endoscopic approach for management of large jaw cysts and assess the feasibility of endoscopic enucleation as well as analyze its outcome on a wide scale at Mansoura University Hospital MUH.MethodsThis prospective cohort study was done on 23 consecutive cases presenting with different types of large jaw cysts in the period from January 2013 to July 2016 at ENT Department, Mansoura University Hospital. All patients, (16 maxillary & 7 mandibular) cysts, were managed endoscopically. Follow up ranged from 6 to 48 months.ResultsAll patients showed complete resolution of their symptoms postoperatively except for 2 maxillary case who didn't undergo an antrostomy and 1 mandibular indicated a second look.ConclusionLarge jaw cysts lie at the border line of management options. Resection of the affected segment is a radical option but the most deforming. On the other hand, conservative procedures are prolonged and not definitive. To our knowledge this is the first case series of endoscopic enucleation of large maxillary and mandibular cysts, highlighting its advantages in variable types.

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Comparison of the accuracy of maxillary position between conventional model surgery and virtual surgical planning

Publication date: Available online 23 September 2017
Source:International Journal of Oral and Maxillofacial Surgery
Author(s): F.G. Ritto, A.R.M. Schimtt, T. Pimentel, J.V. Canellas, P.J. Medeiros
The aim of this study was to determine whether virtual surgical planning (VSP) is an accurate method for positioning the maxilla when compared to conventional articulator model surgery (CMS), through the superimposition of computed tomography (CT) images. This retrospective study included the records of 30 adult patients submitted to bimaxillary orthognathic surgery. Two groups were created according to the treatment planning performed: CMS and VSP. The treatment planning protocol was the same for all patients. Pre- and postoperative CT images were superimposed and the linear distances between upper jaw reference points were measured. Measurements were then compared to the treatment planning, and the difference in accuracy between CMS and VSP was determined using the t-test for independent samples. The success criterion adopted was a mean linear difference of <2mm. The mean linear difference between planned and obtained movements for CMS was 1.27±1.05mm, and for VSP was 1.20±1.08mm. With CMS, 80% of overlapping reference points had a difference of <2mm, while for VSP this value was 83.6%. There was no statistically significant difference between the two techniques regarding accuracy (P>0.05).

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Autoantibodies of IgM and IgG classes show differences in recognition of multiple autoantigens in chronic obstructive pulmonary disease

Publication date: Available online 23 September 2017
Source:Clinical Immunology
Author(s): Reham Shindi, Amna Almehairi, Ola H. Negm, Noor Kalsheker, Nichola S. Gale, Dennis J. Shale, Timothy W. Harrison, Charlotte E. Bolton, Michelle John, Ian Todd, Patrick J. Tighe, Lucy C. Fairclough
Autoimmunity occurs in chronic obstructive pulmonary disease (COPD). We describe an antigen microarray for detecting serum autoantibodies (AAbs) to determine how IgM, as well as IgG, AAbs distinguish patients with COPD from controls with a history of smoking without COPD. All COPD patients' sera contained elevated levels of AAbs to some of 30 autoantigens. There were significant differences in the autoantigenic specificities of IgM AAbs compared to IgG AAbs in the COPD sera: for example, AAbs to histone and scl-70 were mainly IgG, whereas AAbs to CENP-B and La/ssB were mainly IgM; by contrast, IgM and IgG AAbs to collagen-V were equally prevalent. Thus, a combination of IgM and IgG AAbs specific for multiple autoantigens are detected in all cases of COPD at a level at which all non-COPD controls are negative for AAbs. This highlights the importance of different classes of AAbs to a range of autoantigens in COPD.

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Circulating B cells in type 1 diabetics exhibit fewer maturation-associated phenotypes

Publication date: Available online 23 September 2017
Source:Clinical Immunology
Author(s): Patrick Hanley, Jennifer A. Sutter, Noah G. Goodman, Yangzhu Du, Debora R. Sekiguchi, Wenzhao Meng, Michael R. Rickels, Ali Naji, Eline T. Luning Prak
Although autoantibodies have been used for decades as diagnostic and prognostic markers in type 1 diabetes (T1D), further analysis of developmental abnormalities in B cells could reveal tolerance checkpoint defects that could improve individualized therapy. To evaluate B cell developmental progression in T1D, immunophenotyping was used to classify circulating B cells into transitional, mature naïve, mature activated, and resting memory subsets. Then each subset was analyzed for the expression of additional maturation-associated markers. While the frequencies of B cell subsets did not differ significantly between patients and controls, some T1D subjects exhibited reduced proportions of B cells that expressed transmembrane activator and CAML interactor (TACI) and Fas receptor (FasR). Furthermore, some T1D subjects had B cell subsets with lower frequencies of class switching. These results suggest circulating B cells exhibit variable maturation phenotypes in T1D. These phenotypic variations may correlate with differences in B cell selection in individual T1D patients.

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Laryngotracheal reconstruction and swallowing: A review

Publication date: November 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 102
Author(s): Jennifer F. Ha, Lynn Driver, David A. Zopf
ObjectivesSignificant advances in laryngotracheal reconstruction over the last few decades have revolutionised the management of paediatric patients with complex congenital or acquired airway stenosis. The primary aim of laryngotracheal reconstruction has focused primarily on airway and surgery specific outcomes, often at the expense of voice, as well as swallowing function, which are all intricately related. There is currently a paucity of data on swallowing outcome. The goal of this paper is to review and discuss the existing research on the impact of laryngotracheal on swallowing.MethodsNarrative review.ResultsSuccessful and safe oral feeding in children requires a highly complex and integrated sensorimotor system for proper timing and coordination, beginning with a well-coordinated suck-swallow-breathe sequence in infancy. Factors to consider include the normal laryngeal anatomy, nutrition as a stimulus and the development of feeding skills on swallowing, the underlying aetiology and other risk factors, LTR procedures and their adjuncts. All these impact on the children's growth. Swallow assessments and rehabilitation is therefore an important part of the post-operative care.ConclusionsAs airway reconstructive surgeries have improved in airway and surgery specific outcomes, swallowing function is an important secondary outcome that impacts on the children's and their families' life. Management in a multi-disciplinary manner will optimise the outcome and improve their quality of life.

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Evaluating the sinus and Nasal Quality of Life Survey in the pediatric cystic fibrosis patient population

Publication date: November 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 102
Author(s): Deborah X. Xie, Jeffanie Wu, Katherine Kelly, Rebekah F. Brown, Chevis Shannon, Frank W. Virgin
IntroductionThe Sinus and Nasal Quality of Life Survey (SN-5) is a validated quality of life (QOL) questionnaire for chronic rhinosinusitis in patients age 2–12. Its utility in the cystic fibrosis (CF) has been studied, but not yet validated. The purpose of this study is to determine the effectiveness of the SN-5 for evaluation of sinonasal symptoms in the pediatric CF population.MethodsThis retrospective study analyzed SN-5 surveys completed between 2012 and 2015 by pediatric CF patients and caregivers. Baseline and follow-up overall QOL scores and specific symptom scores were obtained from surveys completed in the three-year span. Non-parametric statistics were conducted to identify differences in survey data.ResultsA total of 165 patients completed baseline and follow-up surveys. The overall QOL of the patient cohort did not change over the duration of the study (p = 0.660). Thirty-seven patients indicated higher overall QOL, with all five symptom scores showing significant improvement. Analysis by age group showed that QOL was significantly correlated with all five symptoms for children ages 0–4. In patients 5–12 years, overall QOL was only correlated with sinus infection (r = −0.3090, p = 0.01). QOL was significantly correlated with sinus infection (r = −0.2903, p = 0.04) and allergy symptoms (r = −0.5644, p < 0.01) in patients >12 years of age.ConclusionThere remains a need for a validated CRS QOL tool for children with CF. Though the SN-5 has previously been described as a potential instrument, our data suggest that it may be more valuable in children ages 0–4.

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