Imaging manifestations of IgG4-related disease.

IgG4-related disease is a multisystem immune-mediated disorder associated with lesions manifesting an IgG4-rich plasma cell infiltrate and often raised serum IgG4 concentrations. The disease can mimic neoplastic, infective, and inflammatory processes due to features such as development of masses or organ enlargement. Prompt consideration of this diagnosis is essential to avoid unnecessary investigations and offer appropriate treatments, which can include steroids and other immunosuppressive agents. Although histology is typically diagnostic, imaging is critical to assess disease burden, determine biopsy targets, and evaluate response to treatment. Characteristic imaging features can also point towards the diagnosis in the absence of biopsy. This review highlights these features, as well as more atypical findings, grouped by organ or system. Differential diagnoses are emphasised. The full spectrum of imaging methods is discussed. Whole-body imaging with integrated 2-[18F]-fluoro-2-deoxy-d-glucose (FDG) positron-emission tomography (PET)/computed tomography (CT) has an evolving role in the detection of multi-organ involvement and subsequent follow-up.

Diagnosis of Aortic Graft Infection: A Case Definition by the Management of Aortic Graft Infection Collaboration (MAGIC).

OBJECTIVE/BACKGROUND: The management of aortic graft infection (AGI) is highly complex and in the absence of a universally accepted case definition and evidence-based guidelines, clinical approaches and outcomes vary widely. The objective was to define precise criteria for diagnosing AGI. METHODS: A process of expert review and consensus, involving formal collaboration between vascular surgeons, infection specialists, and radiologists from several English National Health Service hospital Trusts with large vascular services (Management of Aortic Graft Infection Collaboration [MAGIC]), produced the definition. RESULTS: Diagnostic criteria from three categories were classified as major or minor. It is proposed that AGI should be suspected if a single major criterion or two or more minor criteria from different categories are present. AGI is diagnosed if there is one major plus any criterion (major or minor) from another category. (i) Clinical/surgical major criteria comprise intraoperative identification of pus around a graft and situations where direct communication between the prosthesis and a nonsterile site exists, including fistulae, exposed grafts in open wounds, and deployment of an endovascular stent-graft into an infected field (e.g., mycotic aneurysm); minor criteria are localized AGI features or fever ≥38°C, where AGI is the most likely cause. (ii) Radiological major criteria comprise increasing perigraft gas volume on serial computed tomography (CT) imaging or perigraft gas or fluid (≥7 weeks and ≥3 months, respectively) postimplantation; minor criteria include other CT features or evidence from alternative imaging techniques. (iii) Laboratory major criteria comprise isolation of microorganisms from percutaneous aspirates of perigraft fluid, explanted grafts, and other intraoperative specimens; minor criteria are positive blood cultures or elevated inflammatory indices with no alternative source. CONCLUSION: This AGI definition potentially offers a practical and consistent diagnostic standard, essential for comparing clinical management strategies, trial design, and developing evidence-based guidelines. It requires validation that is planned in a multicenter, clinical service database supported by the Vascular Society of Great Britain & Ireland.

The role of FDG-PET and bone marrow examination in lymphoma staging.

The introduction of positron emission tomography using [F-18]fluorodeoxyglucose (FDG-PET) has had a substantial impact on the management of patients with lymphoma. Increasing numbers of patients are having an FDG-PET study as part of their initial staging, despite FDG-PET cannot be considered yet a standard procedure for staging in many types of lymphoma. FDG-PET has demonstrated its superiority over conventional imaging to identify nodal and extra-nodal sites of disease and provides complementary information to that obtained with bone marrow biopsy. This can result in disparities in the staging and prognostication of patients based on the procedures used to assess the extension of the disease. The difficulty lies in how to use the information provided by FDG-PET to communicate effectively when using staging classifications and prognostic indices that were designed following conventional imaging.

The role of early ¹8F-FDG PET/CT in prediction of progression-free survival after 9°Y radioembolization: comparison with RECIST and tumour density criteria.

PURPOSE: This study evaluated the ability of (18)F-FDG PET/CT imaging to predict early response to (90)Y-radioembolization in comparison with contrast-enhanced CT (CECT) using RECIST and lesion density (Choi) criteria. Progression-free survival (PFS) in patients with liver metastases at 2 years and decline in tumour markers were the primary end-points of the study. METHODS: A total of 121 liver lesions were evaluated in 25 patients (14 men, 11 women) with liver-dominant metastatic colorectal cancer who underwent (18)F-FDG PET/CT and CECT before and 6-8 weeks after treatment. Changes in SUV(max), tumour density measured in terms of Hounsfield units and the sum of the longest diameters (LD) were calculated for the target liver lesions in each patient. The patient responses to treatment were categorized using EORTC PET criteria, tumour density criteria (Hounsfield units) and RECIST, and were correlated with the responses of tumour markers and 2-year PFS using Kaplan-Meier plots and the log-rank test for comparison. Multivariate proportional hazards (Cox) regression analysis was performed to assess the effect of relevant prognostic factors on PFS. RESULTS: Using (18)F-FDG PET/CT response criteria, 15 patients had a partial response (PR) and 10 patients had stable disease (SD), while using RECIST only 2 patients had a PR and 23 had SD. Two patients had a PR, 21 SD and 2 progressive disease using tumour density criteria. The mean changes in SUV(max), sum of the LDs and tumour density after treatment were 2.9 ± 2.6, 7.3 ± 14.4 mm and 1.9 ± 13.18 HU, respectively. Patients who had a PR on (18)F-FDG PET/CT had a mean decrease of 44.5 % in SUV(max) compared to those with SD who had a decrease of only 10.3 %. The decreases in SUV(max) and sum of the LDs were significant (p < 0.0001, p < 0.05, respectively) while the decrease in tumour density was not (p > 0.1065). The responses on the (18)F-FDG PET/CT studies were highly correlated with the responses of tumour markers (p < 0.0001 for LDH, p = 0.01 for CEA and p = 0.02 for Ca19-9), while the responses on the CECT studies using both RECIST and tumour density criteria were not significantly correlated with the responses of tumour markers. The responses on (18)F-FDG PET/CT studies also significantly predicted PFS (the median PFS in those with a PR was 12.0 months and in those with SD was 5 months, p < 0.0001), while RECIST and tumour density did not significantly predict PFS. Multivariate analysis demonstrated that responses on (18)F-FDG PET/CT studies and decreases in SUV(max) of ≤ 2.0 were the strongest predictors of PFS. CONCLUSION: Early response assessment to (90)Y-radioembolization using (18)F-FDG PET/CT is superior to RECIST and tumour density, demonstrating a correlation with tumour markers and significantly predicting PFS in patients with liver metastases. This could enable early response-adapted treatment strategies to be employed.

An international patient-centred study of retroperitoneal fibrosis.

BACKGROUND: The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. AIM: To understand the physical and psychosocial impact that RPF has upon peoples' lives. DESIGN: An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. METHODS: An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. RESULTS: A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. CONCLUSION: This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.

The changing face of reoperative parathyroidectomy: a single-centre comparison of 147 parathyroid reoperations.

INTRODUCTION: Reoperative parathyroidectomy for persistent and recurrent primary hyperparathyroidism is dependent on radiology. This study aimed to compare outcomes in reoperative parathyroidectomy at a single centre using a combination of traditional and newer imaging studies. MATERIALS AND METHODS: Retrospective case note review of all reoperative parathyroidectomies for persistent and recurrent primary hyperparathyroidism over five years (June 2014 to June 2019; group A). Imaging modalities used and their positive predictive value, complications and cure rates were compared with a published dataset spanning the preceding nine years (group B). RESULTS: From over 2000 parathyroidectomies, 147 were reoperations (101 in group A and 46 in group B). Age and sex ratios were similar (56 vs 62 years; 77% vs 72% female). Ultrasound use remains high and shows better positive predictive value (76% vs 57 %). 99mTc-sestamibi use has declined (79% vs 91%) but the positive predictive value has improved (74% vs 53%). 4DCT use has almost doubled (61% vs 37%) with better positive predictive value (88% vs 75%). 18F-fluorocholine positron emission tomography-computed tomography and ultrasound-guided fine-needle aspiration for parathyroid hormone are novel modalities only available for group A. Both carried a positive predictive value of 100%. Venous sampling with or without angiography use has decreased (35% vs 39%) but maintains a high positive predictive value (86% vs 91%). Cure rates were similar (96% vs 100%). Group A had 5% permanent hypoparathyroidism, 1% permanent vocal cord palsy and 1% haematoma requiring reoperation. No complications for group B. CONCLUSION: Optimal imaging is key to good cure rates in reoperative parathyroidectomy. High-quality, non-interventional imaging techniques have produced a shift in the preoperative algorithm without compromising outcomes.

Video-assisted thoracoscopic surgery for ectopic mediastinal parathyroid adenoma.

Background: Primary hyperparathyroidism (PHPT), caused by an ectopic mediastinal parathyroid adenoma, is uncommon. In the past, when the adenoma was not accessible from the neck, median sternotomy was advocated for safe and successful parathyroidectomy. Video-assisted thoracoscopic surgical (VATS) parathyroidectomy represents a modern alternative approach to this problem. Methods: Information on patients undergoing VATS was obtained from a specific database, including clinical presentation, biochemistry, preoperative imaging, surgical approach and patient outcomes. A comprehensive literature review was undertaken to draw comparisons with other publications. Results: Over a 2-year period, nine patients underwent VATS parathyroidectomy for sporadic PHPT. Five patients had persistent PHPT following previous unsuccessful parathyroidectomy via cervicotomy, and four had had no previous parathyroid surgery. The median duration of surgery was 90 (range 60-160) min. Eight patients were cured biochemically, with no major complications. One patient required conversion to a median sternotomy for removal of a thymoma that had resulted in false-positive preoperative imaging. Conclusion: With appropriate preoperative imaging, multidisciplinary input and expertise, VATS parathyroidectomy is an effective, safe and well tolerated approach to ectopic mediastinal parathyroid adenoma.

Antecedentes: El hiperparatiroidismo primario (primary hyperparathyroidism, pHPT) causado por un adenoma paratiroideo ectópico mediastínico es infrecuente. Hace años, cuando un adenoma no era accesible por vía cervical se propugnaba una esternotomía media para efectuar una paratiroidectomía segura y con éxito. La paratiroidectomía por cirugía toracoscópica asistida por video (video­assisted thoracoscopic surgical, VATS) es una alternativa moderna para el abordaje de esta patología. Métodos: La información de los pacientes tratados con VATS se obtuvo de una base de datos específica, incluyendo presentación clínica, bioquímica, radiología preoperatoria, abordaje quirúrgico y resultados de los pacientes. Se efectuó una revisión extensa de la literatura para efectuar comparaciones con otras publicaciones. Resultados: Durante un periodo de 2 años, 9 pacientes fueron tratados mediante paratiroidectomía por VATS debido a un pHPT esporádico, de los cuales 5 presentaban pHPT persistente después del fracaso de una paratiroidectomía por cervicotomía, mientras que los 4 restantes no habían sido operados previamente de cirugía paratiroidea. El tiempo medio operatorio fue de 101 minutos (rango 60­160). Ocho pacientes se curaron bioquímicamente, sin ninguna complicación mayor. Un paciente precisó conversión a una esternotomía media para extirpar un timoma que había sido un falso positivo en la radiología preoperatoria. Conclusión: La paratiroidectomía por VATS es una intervención efectiva, segura y bien tolerada para la extirpación de un adenoma ectópico mediastínico, siempre y cuando se disponga de radiología preoperatoria adecuada, equipo multidisciplinar y experiencia.

Computed tomography features of recurrent ovarian carcinoma according to time to relapse.

BACKGROUND: Although the sites of recurrent ovarian cancer are individually described in the literature, patterns of recurrent disease are poorly understood. PURPOSE: To describe CT patterns of disease in recurrent ovarian cancer. To emphasize common patterns, recognise subtle and unusual sites of recurrent disease. MATERIALS AND METHODS: We identified patients between 1981-2004 presenting with clinical recurrence or elevated CA 125 after complete primary clinical and radiological response. CT imaging at primary diagnosis, during and after treatment and at recurrence was retrospectively reviewed. Site, distribution, stage of disease and time to relapse was recorded. RESULTS: 400 patients were treated for ovarian cancer. 214(54%) achieved complete primary response. 161(75%) relapsed with complete imaging available in 67 patients. Of the 67 patients, 14 (21%) recurred within 1 year, 44 (66%) relapsed between 1-5 years. Therefore 87% of relapses occurred within 5 years following primary treatment. Five (8%) relapsed between 5-10 years and 4 (6%) relapsed after 10 years. Commonest pattern of relapse was pelvic mass in 35 (48%) patients, solitary in 15 (22%). 27 (45%) relapsed with peritoneal thickening, 27 (45%) had small or large bowel serosal disease, 22 (33%) had enlarged lymphadenopathy, 6 as sole manifestation of recurrence, 20 (30%) presented with unusual sites of recurrence: 6 splenic, 10 hepatic, 2 biliary, 3 brain and 2 muscle. CONCLUSION: Our study is the first to describe common patterns of recurrence in ovarian cancer. Most frequent site is pelvis, followed by peritoneum, serosal surfaces and nodal disease. 30% presented with disease at 'unusual' sites.

Evaluation of 18F-fluorothymidine positron emission tomography ([18F]FLT-PET/CT) methodology in assessing early response to chemotherapy in patients with gastro-oesophageal cancer.

BACKGROUND: 3'-Deoxy-3'-[18F]fluorothymidine ([18F]FLT) PET has limited utility in abdominal imaging due to high physiological hepatic uptake of a tracer. We evaluated [18F]FLT-PET/CT combined with a temporal-intensity information-based voxel-clustering approach termed kinetic spatial filtering (KSF) to improve tumour visualisation in patients with locally advanced and metastatic gastro-oesophageal cancer and as a marker of early response to chemotherapy. Dynamic [18F]FLT-PET/CT data were collected before and 3 weeks post first cycle of chemotherapy. Changes in tumour [18F]FLT-PET/CT variables were determined. Response was determined on contrast-enhanced CT after three cycles of therapy using RECIST 1.1. RESULTS: Ten patients were included. Following application of the KSF, visual distinction of all oesophageal and/or gastric tumours was observed in [18F]FLT-PET images. Among the nine patients available for response evaluation (RECIST 1.1), three patients had responded (partial response) and six patients were non-responders (stable disease). There was a significant association between Ki-67 and all baseline [18F]FLT-PET parameters. Area under the curve (AUC) from 0 to 1 min was associated with treatment response. CONCLUSIONS: The results of this study indicate that application of the KSF allowed accurate visualisation of both primary and metastatic lesions following imaging with the proliferation marker, [18F]FLT-PET/CT. However, [18F]FLT-PET uptake parameters did not correlate with response. Instead, we observe significant changes in tracer delivery following chemotherapy suggesting that further [18F]FLT-PET/CT studies in this tumour type should be undertaken with caution.

Postural change in ventilation and perfusion secondary to a thoracic scoliosis with complete resolution after surgery.

We present the case of a child with a thoracic scoliosis causing respiratory impairment in whom pre-surgical ventilation-perfusion lung scintigraphy in different postures was able to predict improvement in ventilation and perfusion after surgery.

Imaging of endometrial adenocarcinoma.

Endometrial cancer is the most common gynaecological malignancy and the incidence rising. Prognosis depends on age of patient, histological grade, depth of myometrial invasion and cervical invasion and lymph node metastases. Myometrial invasion and accurate cervical involvement cannot be predicted clinically. Pre-treatment knowledge of these criteria is advantageous in order to plan treatment. The clinical challenge is to effectively select patients at risk of relapse for more radical treatment whilst avoiding over treating low risk cases. This is important as endometrial cancer predominately occurs in postmenopausal women with co-morbidities. Modern imaging provides important tools in the accurate pre-treatment assessment of endometrial cancer and may optimize treatment planning. However, there is little consensus to date on imaging in the routine preoperative assessment of endometrial carcinoma and practice varies amongst many gynaecologists. Transvaginal ultrasound is often the initial imaging examination for women with uterine bleeding. However, once the diagnosis of endometrial cancer has been made, contrast-enhanced magnetic resonance imaging (MRI) provides the best assessment of the disease. The results of contrast-enhanced MRI may identify patients who need more aggressive therapy and referral to a cancer centre. In this article we review the role of imaging in the diagnosis and staging/preoperative assessment of endometrial carcinoma.

Embryology of the adrenal glands and its relevance to diagnostic imaging.

An understanding of the embryology of the adrenal glands is necessary to appreciate the location of adrenal ectopic, or rest, tissue which can occur anywhere along the course of gonadal descent. This tissue usually has no clinical significance, but may become hyperplastic in patients with primary or secondary adrenal pathology. In congenital adrenal hyperplasia, hyperplastic rest tissue may present as a soft-tissue mass, particularly in the gonads and retroperitoneum, and may be mistaken for tumour. The adrenal in the neonate is proportionately much larger than in the adult; in renal ectopy or agenesis the ipsilateral adrenal is normally sited and may be mistaken for a kidney because of its size. This review article illustrates the embryology of the adrenal with particular emphasis on the relevance of embryology to pathology.

The two week referral for colorectal cancer: a retrospective analysis.

OBJECTIVE: The two-week referral (TWR) system was introduced in July 2000 to address the delays in referral, diagnosis and treatment of colorectal cancer (CRC) and lessen the associated psychological morbidity of prolonged waiting. General practitioners complete a proforma outlining 'high-risk' criteria for CRC to ensure an urgent referral within 14 days. The aim of the study was to analyse the TWR process and the proforma criteria. PATIENTS AND METHODS: One hundred and forty-nine two-week referral proforma were retrospectively reviewed between January and August 2001. The waiting times and proforma data, together with investigations performed and diagnoses made were gathered for 144 patients. Three did not attend clinic and two sets of notes were missing. RESULTS: Ninety-six percent of patients (n = 144) were two week compliant and 14 CRC (10%) were diagnosed. The most common referral symptom was a recent change in bowel habit (36.6%) but specificity for all criteria was low. The highest diagnostic yield was a palpable abdominal or rectal mass where 16.7% had CRC and iron deficiency anaemia had high sensitivity (90%) for surgical pathology. Per rectum examination and haemoglobin analysis by general practitioners was infrequently performed. DISCUSSION: Our study has shown that CRC is difficult to diagnose by history and examination alone with a 10% detection rate. CRC incidence in TWR may be improved by primary care through routine rectal examinations, increased detection of iron deficiency anaemia and public education to reduce presentation via other referral routes. Further studies are needed to address these issues.