Eosinophilic solid and cystic renal cell carcinoma: A case study with pathological correlates.

Introduction And Significance: Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) is a rare renal tumor primarily associated with female patients and those with tuberous sclerosis complex (TSC). Despite lacking distinct clinical or radiological features, its unique histological characteristics allow for differentiation from other renal neoplasms. While it often exhibits indolent growth, metastatic potential remains a concern.

Genetic syndromes associated with pancreatic neuroendocrine neoplasms and imaging diagnostic strategies.

Pancreatic neuroendocrine neoplasms (pNENs) are the second most common pancreatic malignancy. While most cases are sporadic, a small proportion is associated with genetic syndromes, such as Multiple Endocrine Neoplasia (MEN), Von Hippel-Lindau Syndrome (VHL), Neurofibromatosis Type 1 (NF1), and Tuberous Sclerosis Complex (TSC). This review aims to use pNENs as a clue to reveal the full spectrum of disease, providing a comprehensive understanding of diagnosis.

Imaging Findings and Management Strategies for Liver Masses in Children with Predisposition Disorders: A Review by the Pediatric LI-RADS Group.

Liver masses in children with underlying systemic disease or a predisposing syndrome can be benign or malignant, ranging from focal fat to hepatocellular carcinoma (HCC). Knowledge of the underlying condition, the pathophysiologic effect on the liver, and the development of liver disease and specific liver lesions allows radiologists to guide imaging with regard to modality and frequency and give recommendations for biopsy when appropriate. In some predisposition disorders, such as Beckwith Wiedemann spectrum, familial adenomatous polyposis syndrome, and tuberous sclerosis complex, established guidelines for imaging screening exist.

Pericardial Effusion as a Presentation of Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare, systemic neoplastic disease that primarily affects women of childbearing age. The disease can arise sporadically or in association with tuberous sclerosis. It is characterized by the proliferation of abnormal smooth muscle-like cells, leading to cystic lung destruction, accumulation of chylous fluid, and development of abdominal tumors.

Giant renal angiomyolipomas in tuberous sclerosis complex.

Angiomyolipomas are the benign tumours of the kidney which occur either sporadically or in association with tuberous sclerosis complex (TSC). Symptoms typically develop with an increase in the size of the lesion. We present a clinical image of a patient with giant renal angiomyolipomas who had other clinical features of TSC (facial angiofibromas, periungual fibroma, subependymal nodules in the brain, and lung cysts).

Epilepsy surgery in children with operculoinsular epilepsy: Results of a large unicentric cohort.

Objective: Epilepsy surgery in the operculoinsular cortex is challenging due to the difficult delineation of the epileptogenic zone and the high risk of postoperative deficits.

Methods: Pre- and postsurgical data from 30 pediatric patients who underwent operculoinsular cortex surgery at the Motol Epilepsy Center Prague from 2010 to 2022 were analyzed.

Results: Focal cortical dysplasia (FCD; n = 15, 50%) was the predominant cause of epilepsy, followed by epilepsy-associated tumors (n = 5, 17%) and tuberous sclerosis complex (n = 2, 7%).

The development for emerging biomarkers of lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare, slowly progressing, low-grade metastatic tumor primarily affecting women. Currently, vascular endothelial growth factor-D (VEGF-D) is the only validated diagnostic biomarker, enabling diagnosis of LAM without the need for lung biopsy in appropriate clinical settings. However, VEGF-D concentrations are normal in about 30% of patients, rendering it insufficient for diagnosing all cases of LAM.

Safety profile of abdominal magnetic resonance imaging (MRI) performed for renal disease surveillance in tuberous sclerosis complex patients with vagus nerve stimulation: Safety of MRI for TSC Patients with VNS.

Introduction: Individuals with tuberous sclerosis complex (TSC) often present with refractory epilepsy and may be undergoing treatment with vagus nerve stimulation (VNS) to control seizures. Surveillance magnetic resonance imaging (MRI) is necessary to monitor for the renal angiomyolipomas associated with TSC; however, MRI of the abdomen is not approved for patients withVNS therapy. We have many TSC patients with refractory epilelpsy who benefitted from VNS therapy, so we developed an MRI protocol that allows MRI of the abdomen to be performed in these patients to permit safe imaging of their kidneys.

Central nervous system manifestations of tuberous sclerosis complex: A single centre experience in Qatar.

Objectives: To review the clinical and radiological correlation of the central nervous system manifestations of tuberous sclerosis complex (TSC).

Methods: All patients under the age of 18 years with TSC seen at the Department of Pediatrics, Sidra Medicine, Doha, Qatar, between January 2003 and February 2021 were included in this retrospective study. Severity of epilepsy was determined using the early childhood epilepsy severity score (E-CHESS) tool.

Prediction of epileptogenicity in patients with tuberous sclerosis complex using multimodal cerebral MRI.

Objective: Epilepsy is the most common complication and cause of morbidity and mortality in tuberous sclerosis complex (TSC). Surgery is associated with an increased probability of achieving seizure-freedom. However, the preoperative noninvasive localisation of epileptogenic tubers remains challenging.

Multisystem diseases in the abdomen and pelvis: imaging manifestations and diagnostic roles of cross-sectional imaging.

Systemic diseases, such as IgG4-related disease, sarcoidosis, and amyloidosis, usually involve multiple systems or organs simultaneously or sequentially. The gastrointestinal tract, hepatobiliary system, and genitourinary tract are commonly involved in many multisystem diseases and can also be the first sites with disease involvement. Cross-sectional imaging, such as CT and MR, plays an important role in the diagnosis and management of multisystem diseases by aiding in the evaluation of multiorgan involvement.