A Case of IgG4-Related Disease Manifesting as Extensive Abdominal Periarteritis and Membranous Nephropathy, Successfully Controlled with Low-Dose Steroid Therapy without Relapse or Complications.

Introduction: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that can affect nearly every organ system, including blood vessels and the kidney. IgG4-related vascular lesions mainly involve the aorta, and the dominant renal manifestation is tubulointerstitial nephritis (TIN). Here, we report a case of IgG4-RD demonstrating extensive abdominal periarteritis and membranous nephropathy (MN).

ZBTB7A forms a heterodimer with ZBTB2 and inhibits ZBTB2 homodimerization required for full activation of HIF-1.

Hypoxia-inducible factor 1 (HIF-1), recognized as a master transcription factor for adaptation to hypoxia, is associated with malignant characteristics and therapy resistance in cancers. It has become clear that cofactors such as ZBTB2 are critical for the full activation of HIF-1; however, the mechanisms downregulating the ZBTB2-HIF-1 axis remain poorly understood. In this study, we identified ZBTB7A as a negative regulator of ZBTB2 by analyzing protein sequences and structures.

Re-evaluation and operative indications after induction therapy for thymic epithelial tumors.

Thymic epithelial tumors (TETs), encompassing thymoma and thymic carcinoma, represent a rare and heterogeneous group of thoracic malignancies with varying prognoses and treatment strategies. Surgical resection is the cornerstone of therapy for localized stages, but the management of locally advanced or unresectable TETs often involves induction therapy, including chemotherapy and/or radiation therapy, as a neoadjuvant approach aimed at downstaging the tumor to facilitate subsequent resection. This review synthesizes current knowledge on the re-evaluation process and operative indications following induction therapy for TETs, highlighting the pivotal role of accurate assessment in guiding surgical decisions and optimizing patient outcomes.

Imaging of thymic epithelial tumors-a clinical practice review.

This review article comprehensively examines the diagnostic approach to thymic epithelial tumors (TETs) and other mediastinal masses, focusing on imaging modalities and differential diagnosis. Beginning with a discussion on traditional and contemporary classification systems for mediastinal tumors, including the Japanese Association for Research on the Thymus (JART) and International Thymic Interest Group (ITMIG) classifications, it highlights the shift towards computed tomography (CT)-based categorizations. Emphasis is placed on the importance of distinguishing between solid and cystic lesions in the anterior mediastinum, with detailed insights into imaging characteristics and histological features of various TET subtypes such as thymomas, thymic carcinomas, and thymic neuroendocrine tumors (NETs).

SMARCA4-Deficient Poorly Differentiated Adenocarcinoma of the Gallbladder.

A 64-year-old woman presented with chest pain while eating and was referred to our hospital. Physical examination revealed abdominal distension, tenderness, and lower-extremity edema. Imaging revealed a large gallbladder tumor infiltrating the liver, with ascites and pleural effusion.

Association Between Pancreatic Cysts and Diabetes Mellitus in Von Hippel-Lindau Disease.

Introduction: Pancreatic cysts are frequently observed in patients with von Hippel-Lindau disease (VHL), and they are considered clinically not important. This study aimed to evaluate the association between pancreatic cysts and diabetes mellitus (DM) in patients with VHL.

Methods: Among patients who were on a patient list at the VHL Center at Kyoto University Hospital as of December 2022, those who had undergone an upper abdominal magnetic resonance imaging study after 2010 were retrospectively evaluated.

Hyalinizing Clear Cell Carcinoma in the Sphenoid Sinus.

A 39-year-old man presented with a 1-month history of headaches. Imaging revealed a mass with extensive destruction. T2-weighted imaging displayed mixture of low and sponge-like high intensities and also dark area, with FDG PET/CT showing uneven but intense accumulation.

DDX5 enhances HIF-1 activity by promoting the interaction of HIF-1α with HIF-1β and recruiting the resulting heterodimer to its target gene loci.

Background Information: Cancer cells acquire malignant characteristics and therapy resistance by employing the hypoxia-inducible factor 1 (HIF-1)-dependent adaptive response to hypoxic microenvironment in solid tumors. Since the underlying molecular mechanisms remain unclear, difficulties are associated with establishing effective therapeutic strategies.

Results: We herein identified DEAD-box helicase 5 (DDX5) as a novel activator of HIF-1 and found that it enhanced the heterodimer formation of HIF-1α and HIF-1β and facilitated the recruitment of the resulting HIF-1 to its recognition sequence, hypoxia-response element (HRE), leading to the expression of a subset of cancer-related genes under hypoxia.

Clinical and neuroimaging review of monogenic cerebral small vessel disease from the prenatal to adolescent developmental stage.

Cerebral small vessel disease (cSVD) refers to a group of pathological processes with various etiologies affecting the small vessels of the brain. Most cases are sporadic, with age-related and hypertension-related sSVD and cerebral amyloid angiopathy being the most prevalent forms. Monogenic cSVD accounts for up to 5% of causes of stroke.

Convolutional neural network-based program to predict lymph node metastasis of non-small cell lung cancer using F-FDG PET.

Purpose: To develop a convolutional neural network (CNN)-based program to analyze maximum intensity projection (MIP) images of 2-deoxy-2-[F-18]fluoro-D-glucose (FDG) positron emission tomography (PET) scans, aimed at predicting lymph node metastasis of non-small cell lung cancer (NSCLC), and to evaluate its effectiveness in providing diagnostic assistance to radiologists.

Methods: We obtained PET images of NSCLC from public datasets, including those of 435 patients with available N-stage information, which were divided into a training set (n = 304) and a test set (n = 131). We generated 36 maximum intensity projection (MIP) images for each patient.

Brg1 controls stemness and metastasis of pancreatic cancer through regulating hypoxia pathway.

Pancreatic ductal adenocarcinoma (PDAC) is a devastating disease. We previously reported that chromatin remodeler Brg1 is essential for acinar cell-derived PDAC formation in mice. However, the functional role of Brg1 in established PDAC and its metastasis remains unknown.

Novel Manifestation of Retinal Hemangioblastomas Detected by OCT Angiography in von Hippel-Lindau Disease.

Purpose: To elucidate the clinical characteristics of atypical retinal vascular proliferation in patients with von Hippel-Lindau (VHL) disease using OCT angiography (OCTA).

Design: Prospective, observational study.

Participants: Fifty-seven consecutive patients with a diagnosis of VHL disease who visited Kyoto University Hospital between January 2019 and March 2022.

Loss of ATF6α in a human carcinoma cell line is compensated not by its paralogue ATF6β but by sustained activation of the IRE1 and PERK arms for tumor growth in nude mice.

To survive poor nutritional conditions, tumor cells activate the unfolded protein response, which is composed of the IRE1, PERK, and ATF6 arms, to maintain the homeostasis of the endoplasmic reticulum, where secretory and transmembrane proteins destined for the secretory pathway gain their correct three-dimensional structure. The requirement of the IRE1 and PERK arms for tumor growth in nude mice is established. Here we investigated the requirement for the ATF6 arm, which consists of ubiquitously expressed ATF6α and ATF6β, by constructing ATF6α-knockout (KO), ATF6β-KO, and ATF6α/β-double KO (DKO) in HCT116 cells derived from human colorectal carcinoma.

Inverted FDG Uptake Pattern in Subcutaneous Panniculitis-Like T-Cell Lymphoma.

A 47-year-old woman presented with a 2-month history of subcutaneous nodules, erythema, and fever. 18F-FDG PET images demonstrated inverted FDG uptake pattern corresponding to the subcutaneous lesion against lymph nodes. The specimen of the inguinal lesion showed massive infiltration of small lymphocytes in the adipose tissue with rimming adipocytes, whereas very few tumor cells infiltrated the lymph nodes.

Unilateral Reduction of 18F-FDG Accumulation in Brown Adipose Tissue by Sympathectomy for Hyperhidrosis.

A 30-year-old woman with left breast cancer underwent 18F-FDG PET/CT for staging. Intense FDG uptake was observed in the primary lesion, as well as on the left side of the neck to the supraclavicular fossa and left paravertebral region. History taking revealed that she had undergone a right thoracic sympathectomy for hyperhidrosis, which resulted in attenuated FDG uptake in the right-sided brown adipose tissue (BAT).

ZBTB2 links p53 deficiency to HIF-1-mediated hypoxia signaling to promote cancer aggressiveness.

Aberrant activation of the hypoxia-inducible transcription factor HIF-1 and dysfunction of the tumor suppressor p53 have been reported to induce malignant phenotypes and therapy resistance of cancers. However, their mechanistic and functional relationship remains largely unknown. Here, we reveal a mechanism by which p53 deficiency triggers the activation of HIF-1-dependent hypoxia signaling and identify zinc finger and BTB domain-containing protein 2 (ZBTB2) as an important mediator.

A Von Hippel-Lindau Disease-Associated Microcystic Adenoma of the Ethmoid Sinus Mimicking Metastatic Clear Cell Renal Cell Carcinoma.

A 38-year-old man with von Hippel-Lindau (VHL) disease and a history of renal cell carcinoma presented with a 2-month history of recurrent epistaxis. MRI revealed a microcystic tumor in the left ethmoid sinus with strong contrast enhancement. 18 F-FDG PET/CT showed FDG uptake (SUV max , 4.

[68Ga-DOTATOC PET/CT for Diagnosing Neuroendocrine Tumors].

Lutathera is a peptide receptor radionuclide therapy (PRRT) for neuroendocrine tumors and was approved as the first PRRT drug in Japan in 2021. Although neuroendocrine tumors are often less aggressive than other highly malignant and invasive tumors, there have been few effective therapy options, so "Lutathera"is a long-awaited treatment. Lutathera is indicated for the treatment of "somatostatin receptor-positive neuroendocrine tumors".

Pancreatic enlargement in a patient receiving therapy with vasodilators for pulmonary arterial hypertension: a case report.

Epoprostenol is a prostacyclin (prostaglandin I2) analog that causes vasodilation and inhibits platelet aggregation and is used in the management of severe pulmonary arterial hypertension (PAH). We herein report a patient with PAH who developed pancreatic enlargement after the initiation of therapy including epoprostenol. Although it is well known that thyroid enlargement occurs in patients with PAH receiving epoprostenol therapy, the pancreatic findings associated with epoprostenol therapy have not been well described.

Preoperative Localization of Parathyroid Adenomas with Diffusion MR Imaging: Readout-segmented versus Single-shot Echo-planar Imaging.

Purpose: To evaluate whether readout-segmented echo-planar imaging (RS-EPI) diffusion-weighted imaging (DWI) can reduce image distortion and improve the lesion identification in parathyroid adenomas (PTAs) compared to single-shot EPI (SS-EPI) DWI, and to determine whether PTAs can be differentiated from other soft tissue structures of the head and neck region by using the apparent diffusion coefficient (ADC) value.

Methods: We retrospectively analyzed the preoperative MR images including DWI of 24 patients with surgically confirmed PTA. RS-EPI and SS-EPI DWI were evaluated by two independent readers for the identification of the lesions and distortion.

Association between diffuse renal uptake of F-FDG and acute kidney injury.

Objective: The aim of this study was to investigate the clinical characteristics of patients with diffuse renal uptake (DRU) of 2-deoxy-2-[F-18]fluoro-D-glucose (FDG), with particular focus on renal function.

Methods: We retrospectively analyzed 40 patients who showed DRU on FDG PET/CT and the same number of matched controls. The clinical features, imaging parameters (the mean SUVmax of the kidneys and the kidney size), and laboratory data including renal function parameters (Cr, the serum creatinine level; estimated glomerular filtration ratio (eGFR); Cr-ratio, Cr divided by the baseline; max-Cr-ratio, the maximum serum creatinine level within 30 days divided by the baseline) and C-reactive protein (CRP) were compared between the two groups.