Glandular odontogenic cyst: an updated analysis of 169 cases reported in the literature

Abstract

Objective

To integrate the available data published on glandular odontogenic cyst (GOC) into a comprehensive analysis of its clinical/radiologic and histopathological features.

Methods

An electronic search was undertaken in May/2017. Eligibility criteria included publications having enough clinical/radiological/histological information to confirm the diagnosis.

Results

58 publications (169 GOCs) were included. The lesion was slightly more prevalent in men than in women. There was a high prevalence in the fifty/sixth decades of life, in the anterior regions, and in mandibles. Lesions were commonly associated with bone expansion (73%) and unilocular radiological appearance (61.5%). GOC was found to be associated with tooth displacement or an unerupted tooth (30.9%), cortical bone perforation (26%), presence of clinical symptoms (24.3%), root resorption (13.9%). Microscopic parameters most commonly observed in GOCs - in at least 95% of the lesions: presence of hobnail cells, intraepithelial microcysts, epithelial lining with variable thickness. The presence of apocrine snouting was the microscopic parameter less often found (40.4%).

Conclusions

Although the recurrence rate of GOCs is not as high as previously believed, it is a relevant phenomenon (21.6%). Adjunctive procedures after enucleation should be considered. None of the clinical/radiologic and histopathological features evaluated had a statistically significant effect on the recurrence rate.

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Idiopathic sudden sensorineural hearing loss and Ménière syndrome: The role of cerebral venous drainage

Abstract

Aim

to evaluate the influence of cerebral venous drainage on the pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) and Ménière syndrome (MD).

Methods

we enrolled 59 consecutive patients (32 males, mean age 53.05+15.37 years): 40 ISSHL and 19 MD. All underwent physical examination, biochemical evaluation (glycemic and lipid profile, viral serology, C reactive protein, etc), audiometric (tonal, vocal, vestibular evoked myogenic potentials and auditory brainstem response test) and impedentiometric examination. The pure tone average (PTA) was calculated for the following frequencies: 250, 500, 1000, 2000, 3000, 4000, 8000. An echo-color Doppler evaluation of the venous cerebral veins, internal jugular (IJV) and vertebral veins (VV) at supine and 90° position was performed.

Results

no morphological alterations were found both in patients and controls. There were no signs of stenosis, blocked flow, membranes, etc. We found lower minimum, mean and maximum velocities in distal IJVs (p=0.019; p=0.013; p=0.022; respectively) and left VVs (p=0.027; p=0.008; p=0.001; respectively) in supine (0°) position in both MD and ISSHL patients as compared to controls. The same was for orthostatic position (90°). We found negative correlations between the velocities in extracranial veins and PTA values: therefore, the worst the audiometric performance of the subjects, the lower the velocities in the venous cerebral drainage.

Conclusions

ISSHL and MD patients showed altered venous flow in IJVs and VVs as compared to controls, independently from posture. This different behavior of venous tone control can influence the ear performance and may have a role in the pathogenesis of both diseases.

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Lichen amyloidosis involving the scalp

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Measurement Error of a Simplified Protocol for Quantitative Sensory Tests in Chronic Pain Patients.

Background and Objectives: Large-scale application of Quantitative Sensory Tests (QST) is impaired by lacking standardized testing protocols. One unclear methodological aspect is the number of records needed to minimize measurement error. Traditionally, measurements are repeated 3 to 5 times, and their mean value is considered. When transferring QST to a clinical setting, reducing the number of records would be desirable to meet the time constraints encountered in a routine clinical environment and to reduce the testing burden to chronic pain patients. However, there might be a trade-off between measurement error and number of records. We determined the measurement error of a single versus the mean of 3 records of pressure pain detection threshold (PPDT), electrical pain detection threshold (EPDT), and nociceptive withdrawal reflex threshold (NWRT) in 429 chronic pain patients recruited in a routine clinical setting. Methods: We calculated intraclass correlation coefficients and performed a Bland-Altman analysis. Results: Intraclass correlation coefficients were all clearly greater than 0.75, and Bland-Altman analysis showed minute systematic errors with small point estimates and narrow 95% confidence intervals. Reducing the number of records from traditionally 3 to only 1 did not lead to relevant measurement error in PPDT, EPDT, or NWRT. Conclusions: This study contributes to a standardized QST protocol, and based on the minimal measurement error of 1 single record of PPDT, EPDT, and NWRT, we submit to reduce the testing burden. This would allow saving time, resources, and patient discomfort. Copyright (C) 2017 by American Society of Regional Anesthesia and Pain Medicine.

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Desmoplastic small round cell tumor: evaluation of reverse transcription-polymerase chain reaction and fluorescence in situ hybridization as ancillary molecular diagnostic techniques

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare, biologically aggressive soft tissue neoplasm of uncertain differentiation, most often arising in the abdominal and pelvic cavities of adolescents and young adults with a striking male predominance. Histologically, it is characterized by islands of uniform small round cells in prominent desmoplastic stroma, and it has a polyimmunophenotypic profile, typically expressing WT1 and cytokeratin, desmin, and neural/neuroendocrine differentiation markers to varying degrees. Tumors at other sites and with variant morphology are more rarely described. DSRCT is associated with a recurrent t(11;22)(p13;q12) translocation, leading to the characteristic EWSR1-WT1 gene fusion. Fluorescence in situ hybridization (FISH), to detect EWSR1 rearrangement, and reverse transcription-polymerase chain reaction (RT-PCR) to assess for EWSR1-WT1 fusion transcripts are routine diagnostic ancillary tools. We present a large institutional comparative series of FISH and RT-PCR for DSRCT diagnosis. Twenty-six specimens (from 25 patients) histologically diagnosed as DSRCT were assessed for EWSR1 rearrangement and EWSR1-WT1 fusion transcripts. Of these 26 specimens, 24 yielded positive results with either FISH or RT-PCR or both. FISH was performed in 23 samples, with EWSR1 rearrangement seen in 21 (91.3%). RT-PCR was performed in 18 samples, of which 13 (72.2%) harbored EWSR1-WT1 fusion transcripts. The sensitivity of FISH in detecting DSRCT was 91.3%, and that of RT-PCR was 92.8% following omission of four technical failures. Therefore, both methods are comparable in terms of sensitivity. FISH is more sensitive if technical failures for RT-PCR are taken into account, and RT-PCR is more specific in confirming DSRCT. Both methods complement each other by confirming cases that the other method may not. In isolation, FISH is a relatively non-specific diagnostic adjunct due to the number of different neoplasms that can harbor EWSR1 rearrangement, such as Ewing sarcoma. However, in cases with appropriate morphology and a typical pattern of immunostaining, FISH is confirmatory of the diagnosis.

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Pediatric primary Sjögren syndrome presenting with bilateral ranulas: A case report and systematic review of the literature

Publication date: October 2017
Source:International Journal of Pediatric Otorhinolaryngology, Volume 101
Author(s): Casey Means, Mark A. Aldape, Ericka King
ContextPrimary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome.ObjectiveThis study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children.Data sourcesOvid MEDLINE.Study selectionA MEDLINE literature search was performed using the following search terms: primary, Sjögren, disease, and children. Results were limited to human subjects and articles written in English between 1981 and 2014. Applicable articles were reviewed and qualitatively summarized.Data extractionPreferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRIMA).ResultsInitial MEDLINE search yielded 146 articles, 80 of which were excluded as not clinically pertaining to Sjögren syndrome. An additional 25 were excluded due to lack of pediatric-specific data. Systematic review of the literature revealed no reports of ranula in association with Sjögren syndrome. 6 papers were manually included from review of reference lists of included articles. Our review indicated that recurrent parotitis is the most commonly reported presenting symptom in children, followed by ocular and oral symptoms, musculoskeletal, and renal symptoms. Compared to adults, children are less likely to present with dry eyes and mouth.LimitationsAll studies were retrospective chart reviews, case series or case reports.ConclusionThis is the first report of a child presenting with floor of mouth ranulas in association with Sjögren syndrome. While recurrent parotitis is the most common presentation in children, other salivary gland and extra-salivary manifestations may be seen, and the clinician must maintain a high index of suspicion for underlying Sjögren syndrome.



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Endocrine problems in the critically ill 2: endocrine emergencies

Key points

• Most endocrine emergencies are triggered by a secondary insult, which should be sought and managed appropriately.
• Clinical features may be non-specific, mimicking other disease processes. Maintain a high index of suspicion.
• Derangement of thyroid/adrenal function is common in the critically ill; correction of these derangements in the absence of underlying adrenal or thyroid disease is not required.
• Confirmation of diagnosis should not delay treatment in suspected acute adrenal insufficiency.
• Vitamin D deficiency is associated with increased all-cause mortality, yet there is no current evidence to support its routine replacement in critical illness.

Endocrine emergencies account for approximately 1.3% of UK critical care admissions. Although relatively uncommon, many of these emergencies are potentially life-threatening. Most endocrine crises are triggered by a secondary insult, commonly infection, and clinical features are often vague and potentially attributable to other causes, such as the precipitating illness. Consequently, the underlying endocrine disorder may easily be overlooked. This article will outline the presentation, investigation, and acute management of adult endocrine emergencies. Diabetic emergencies and glycaemic control in critical illness are discussed in the accompanying article, 'Endocrine problems in the critically ill 1: diabetes and glycaemic control'.¹

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