Should paediatric tonsillar asymmetry be an indication for tonsillectomy? A single centre experience.

BACKGROUND: Paediatric tonsillar lymphoma (TL) is a rare diagnosis. Historically, the presence of clinical features such as tonsillar asymmetry, grossly abnormal tonsil appearance and cervical lymphadenopathy raise concern for this diagnosis. Tonsillar asymmetry is considered to be the most concerning clinical feature; however, asymmetry is often apparent due to differences in depth or shape of tonsillar fossa and tonsillar pillars, rather than a true difference in volume. There is debate whether a tonsillectomy is required in all cases of tonsil asymmetry to exclude lymphoma, and what clinical features should raise concern. The aim of this study was to establish whether the presence of clinical asymmetry can be deemed a reliable marker for genuine tonsil size discrepancies. We also sought to evaluate the clinical and examination characteristics that are concerning for lymphoma. METHODS: Retrospective review of clinical records for paediatric tonsil specimens sent for histological evaluation between 1 January 2012 and 1 January 2023 driven by a clinical suspicion of lymphoma at Starship Children's Hospital, New Zealand. Patient demographics and clinical data were recorded. A comparison was made between tonsil size asymmetry on clinical examination (Brodsky criteria) and tonsil volume difference based on dimensions given in pathology reports. RESULTS: One hundred and forty-three patients had tonsillectomies between 2012 and 2022 at Starship Children's Hospital due to concern for lymphoma. Of these, three were positive for lymphoma. Presence of pain and abnormal tonsil appearance were predictors for lymphoma (p<0.02). Interrater reliability agreement between clinical size difference and tonsil volume was poor, Kappa= -0.13 p<0.05. CONCLUSION: Clinical size difference is a poor predictor for true tonsil volume difference. We advise that assessment of tonsil size should be performed in conjunction with the examination of gross visual abnormalities and lymphadenopathy to guide clinical decision making.

Multinodal Cervical Angiomyomatous Hamartoma.

Angiomyomatous hamartoma (AMH) is a rare benign lesion of the lymph nodes. Angiomyomatous hamartoma tends to be found in inguinal lymph nodes, and usually in a single lymph node. We present a rare care case of a 53-year-old presenting with a neck lump, found to be AMH involving multiple lymph nodes in her neck. To our knowledge, this is the first case presenting with multiple nodes in this location. There are a limited number of case reports describing magnetic resonance imaging (MRI) features of AMH lesions located in inguinal and head and neck regions. Our MRI findings revealed the mass had intermediate T1 enhancement, high T2 signal enhancement, and high post-gadolinium enhancement and fat saturation of the lesion. Angiomyomatous hamartoma is a histological diagnosis, distinguished from other similar nodal vascular lesions by a number of key features: including the presence of central nodal distribution, muscular blood vessel walls, adipose tissue, and HMB45 negative staining. Early recognition of this benign lesion may have implications for a patient's clinical course and surgical requirements.

Key performance indicators for improving outcomes from retroperitoneal sarcoma surgery in New Zealand.

BACKGROUND: Retroperitoneal sarcomas are a rare and heterogenous group of tumours. There is increasing evidence that high volume sarcoma units achieve superior outcomes than low volume units. Due to the relatively small population, New Zealand is unlikely to generate a sufficient case volume to be considered high volume. To our knowledge, no well-established key performance indicators (KPIs) exist for retroperitoneal sarcoma surgery. In order to ensure quality standards we aim to propose KPIs which will act as a benchmark and a target for future quality improvement. METHODS: Potential KPIs were generated by internal discussion within the sarcoma team, taking into consideration available guidelines and evidence. Cases treated through the unit since 2015 were audited to determine historical performance against these KPIs. RESULTS: Eighty-six patients with primary retroperitoneal sarcomas were identified. 69% of patients were discussed at the sarcoma MDM prior to treatment, 62% underwent preoperative core biopsy. 82% of patients were seen and treated within target timeframes set by the Ministry of Health. 53% of patients underwent surgery with a sarcoma trained surgeon. R0/R1 rate was 88%. 90-day mortality was 4.6%. CONCLUSIONS: Surgical treatment of retroperitoneal sarcomas is complex and can be associated with significant morbidity. The proposed KPIs provide a measure of service performance and provide targets for quality improvement.

Racial and ethnic differences in abdominal soft tissue sarcoma incidence in New Zealand: a retrospective audit.

BACKGROUND: Soft tissue sarcomas are a rare and heterogenous group of tumours. Anecdotally there seems to be an over representation of patients of Maori ethnicity presented at the Auckland Regional Sarcoma Multidisciplinary Meeting (MDM). To date no study has reported on ethnicity demographics of abdominal and retroperitoneal sarcoma in New Zealand. The aim of this study is to characterize the ethnicity distribution of patients discussed at the regional MDM. METHODS: A retrospective audit was performed of patients presented at the Auckland Regional Sarcoma MDM between January 2015 and December 2020 with abdominal sarcoma. Ethnicity documentation for these patients was reviewed. RESULTS: One hundred and twenty-four patients with intra-abdominal and retroperitoneal tumours were discussed at MDM with 61 proceeding for resection, of those 43 (70.5%) were primary tumours, 10 (16.4%) were recurrent tumours and 8 (13.1%) had metastatic disease. Liposarcoma made up 56 (45.2%) cases, Leiomyosarcoma 33 (26.6%), Other 35 (28.2%). Ethnicities for this group specifically were European 64 (51.6%), Maori 31 (25.0%), Pacific Peoples 17 (13.7%), Asian 6 (4.8%) and Other/unknown 3(2.4%). This was found to be statistically significantly different to the expected ethnicity distribution based on 2018 census data from North Island DHBs (χ2  = 19.55, p = 0.00), with Maori and Pacific Peoples being over-represented and Asian patients being under-represented. Recommendation for surgery did not appear to be related to ethnicity. CONCLUSION: Our descriptive data shows a higher proportion of patients of Maori ethnicity discussed through the North Island regional sarcoma MDM than we would expect comparted to ethnicity distribution in the general population. It is unclear whether this represents a true difference in incidence based on ethnicity.

TIRADS Management Guidelines in the Investigation of Thyroid Nodules; Illustrating the Concerns, Costs, and Performance.

CONTEXT: Ultrasound (US) risk-stratification systems for investigation of thyroid nodules may not be as useful as anticipated. OBJECTIVE: We aimed to assess the performance and costs of the American College of Radiology Thyroid Image Reporting And Data System (ACR-TIRADS). DESIGN SETTINGS AND PARTICIPANTS: We examined the data set upon which ACR-TIRADS was developed, and applied TR1 or TR2 as a rule-out test, TR5 as a rule-in test, or applied ACR-TIRADS across all nodule categories. We assessed a hypothetical clinical comparator where 1 in 10 nodules are randomly selected for fine needle aspiration (FNA), assuming a pretest probability of clinically important thyroid cancer of 5%. RESULTS: The gender bias (92% female) and cancer prevalence (10%) of the data set suggests it may not accurately reflect the intended test population. Applying ACR-TIRADS across all nodule categories did not perform well, with sensitivity and specificity between 60% and 80% and overall accuracy worse than random selection (65% vs 85%). Test performance in the TR3 and TR4 categories had an accuracy of less than 60%. Using TR5 as a rule-in test was similar to random selection (specificity 89% vs 90%). Using TR1 and TR2 as a rule-out test had excellent sensitivity (97%), but for every additional person that ACR-TIRADS correctly reassures, this requires >100 ultrasound scans, resulting in 6 unnecessary operations and significant financial cost. CONCLUSIONS: Perhaps surprisingly, the performance ACR-TIRADS may often be no better than random selection. The management guidelines may be difficult to justify from a cost/benefit perspective. A prospective validation study that determines the true performance of TIRADS in the real-world is needed.

Antenatal dexamethasone before asphyxia promotes cystic neural injury in preterm fetal sheep by inducing hyperglycemia.

Antenatal glucocorticoid therapy significantly improves the short-term systemic outcomes of prematurely born infants, but there is limited information available on their impact on neurodevelopmental outcomes in at-risk preterm babies exposed to perinatal asphyxia. Preterm fetal sheep (0.7 of gestation) were exposed to a maternal injection of 12 mg dexamethasone or saline followed 4 h later by asphyxia induced by 25 min of complete umbilical cord occlusion. In a subsequent study, fetuses received titrated glucose infusions followed 4 h later by asphyxia to examine the hypothesis that hyperglycemia mediated the effects of dexamethasone. Post-mortems were performed 7 days after asphyxia for cerebral histology. Maternal dexamethasone before asphyxia was associated with severe, cystic brain injury compared to diffuse injury after saline injection, with increased numbers of seizures, worse recovery of brain activity, and increased arterial glucose levels before, during, and after asphyxia. Glucose infusions before asphyxia replicated these adverse outcomes, with a strong correlation between greater increases in glucose before asphyxia and greater neural injury. These findings strongly suggest that dexamethasone exposure and hyperglycemia can transform diffuse injury into cystic brain injury after asphyxia in preterm fetal sheep.