Does the extent of neck surgery based on preoperative calcitonin level influence survival in medullary thyroid carcinoma: a retrospective tertiary centre experience.

INTRODUCTION: Medullary thyroid carcinoma (MTC) is a rare thyroid cancer arising from the calcitonin-secreting parafollicular cells. Previous studies suggested a preoperative calcitonin level >200ng/l is an indication for prophylactic lateral neck dissection (LND) to remove micrometastases and improve survival outcomes. METHODS: This retrospective single-centre study assessed the efficacy of preoperative calcitonin levels as a marker for determining need for prophylactic LND in MTC. Data were obtained on demographics, preoperative calcitonin levels, size and laterality of index tumour, type of neck dissection (central neck dissection (CND), LND), nodes removed, levels with involved nodes, number of nodes histologically involved, mortality, adjuvant therapy and locoregional recurrence. RESULTS: A total of 33 patients were identified from St George's University Hospitals NHS Foundation Trust between 1 January 2001 and 19 August 2021; 8 were excluded for data quality issues. Of the 18 classified with a high preoperative calcitonin level (>200ng/l), 10 (56%) had a LND and 8 (44%) had a CND. In the low-calcitonin group, three (43%) patients had a CND only and four (57%) had a LND. There was no difference in absolute or disease-free survival between the low and high groups (p=0.960, p=0.817), or between those who had a CND and LND in the high-calcitonin group (p=0.607, hazard ratio (HR) 0.55; p=0.129, HR 8.78). CONCLUSION: There was no statistically significant difference in outcomes between high and low calcitonin groups. A selective approach to performing LND in MTC patients based on clinical and imaging findings suggesting disease presence in the lateral neck should be explored further.

IgG4-related disease confined to the laryngopharynx: a case report highlighting the complexities in diagnosis for this rare cause of stridor.

This case represents only the 15th reported incidence of IgG4-related disease (IgG4-RD) affecting the laryngopharynx, adding diagnostic and therapeutic data for this rare condition and helping to inform the future management of these patients. A 66-year-old man presented with airway symptoms, and investigations by otolaryngology, cardiology and respiratory teams did not provide a clear diagnosis. Repeat biopsies of the laryngopharynx eventually confirmed a clinicopathological diagnosis of IgG4-RD. Treatment with prednisolone and methotrexate was successful. When infective and malignant causes of adult stridor have been excluded, inflammatory causes should be considered. The diagnosis of IgG4-RD isolated to the laryngopharynx may be delayed when using the widely accepted American College of Rheumatology classification criteria because it excludes upper aerodigestive tract features. IgG4-RD isolated to the laryngopharynx is extremely rare. This means a multidisciplinary approach is vital in ensuring timely diagnosis and treatment. Better diagnostic criteria are also needed.

Odontogenic keratocyst located in the retromolar trigone.

Odontogenic keratocysts (OKCs) are a commonly occurring, benign cystic intraosseous lesion thought to arise from the cell rests of the dental lamina. Most instances of OKC are commonly found in the posterior aspect of the mandible. In rare instances, there have been reported cases of OKCs arising in the peripheral gingiva, but these cases are few and far between. Rarer still are the few documented cases of OKCs arising in other soft tissues of the mouth. There are many divided opinions on the diagnosis of these lesions and whether they are truly odontogenic in origin, given their location when they are enucleated from a soft tissue area. To the best of our knowledge, we present the first reported case of a patient with a peripheral OKC located in the retromolar trigone. We discuss this unique case and review the current literature on peripheral OKCs.

Fingerprinting genomic instability in oral submucous fibrosis.

BACKGROUND: Oral submucous fibrosis (OSF) is a high-risk pre-cancerous condition where 7-13% of these patients develop head and neck squamous cell carcinoma (HNSCC). To date there is no cancer predictive markers for OSF patients. Genomic instability hallmarks early genetic events during malignant transformation causing loss of heterozygosity (LOH) and chromosomal copy number abnormality. However, to date there is no study on genomic instability in OSF. Although this condition is known as a high-risk pre-cancerous condition, there is no data regarding the genomic status of this disease in terms of genetic susceptibility to malignant transformation. METHODS: In this study, we investigated the existence of genetic signatures for carcinogenesis in OSF. We employed the high-resolution genome-wide Affymetrix Mapping single nucleotide polymorphism microarray technique to 'fingerprint' global genomic instability in the form of LOH in 15 patient-matched OSF-blood genomic DNA samples. RESULTS: This rapid high-resolution mapping technique has revealed for the first time that a small number of discrete hot-spot LOH loci appeared in 47-53% of the OSF tissues studied. Many of these LOH loci were previously identified regions of genomic instability associated with carcinogenesis of the HNSCC. CONCLUSION: To our knowledge, this is the first evidence that genomic instability in the form of LOH is present in OSF. We hypothesize that the genomic instability detected in OSF may play an important role in malignant transformation. Further functional association studies on these putative genes may reveal potential predictive oral cancer markers for OSF patients.

Upregulation of HIF-1alpha in malignant transformation of oral submucous fibrosis.

BACKGROUND: Oral submucous fibrosis (OSF) is a precancerous condition showing extensive fibrosis of the submucosa and affects most parts of the oral cavity, including pharynx and upper third of the oesophagus. The molecules involved in the biological pathways of the fibrotic process appeared to be either down- or upregulated at different stages of the disease. Despite the precancerous nature, malignant transformation of the epithelium in the background of fibrosis has not been studied in detail. HIF-1alpha is a known transcription factor that is induced by hypoxia. AIMS: To test the hypothesis that hypoxia plays a role in malignant transformation and progression of OSF. MATERIALS AND METHODS: We used both formalin-fixed and frozen samples of OSF and normal mucosa to investigate the relationship between HIF-1alpha and epithelial dysplasia using immunohistochemistry and RT-PCR. CONCLUSIONS: Our data indicate that HIF-1alpha is upregulated at both protein and mRNA levels in OSF and the correlation with epithelial dysplasia is statistically significant (P < 0.001). We propose that HIF-1alpha may play a role in malignant transformation of OSF. Further, over-expression of HIF-1alpha may contribute to the progression of fibrosis. It may be possible to use HIF-1alpha as a marker for malignant transformation of OSF.