Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is an emerging adult-onset systemic autoinflammatory disorder affecting multiple organ systems. While lung involvement is common in this syndrome, literature regarding specific patterns is sparse. In this report, we present a case description of a patient with VEXAS syndrome who presented at the emergency department on two separate occasions with acute interstitial pneumonia (AIP) and diffuse alveolar hemorrhage (DAH). A literature review with a comparison of our observed findings to the general findings of VEXAS syndrome, AIP, and DAH is provided. This report underscores the rarity of specific pulmonary manifestations associated with VEXAS syndrome, contributing valuable insight to the limited literature available on this topic.

VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is a newly described auto-inflammatory disease. Many cases feature pulmonary infiltrates or respiratory failure. This systematic review aimed to summarize respiratory manifestations in VEXAS syndrome described to date.
Databases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease. Information on basic demographics, type and prevalence of pulmonary manifestations, co-existing disease associations and author conclusions on pulmonary involvement were extracted. The protocol was registered on the PROSPERO register of systematic reviews.
Initially, 219 articles were retrieved with 36 ultimately included (all case reports or series). A total of 269 patients with VEXAS were included, 98.6% male, mean age 66.8 years at disease onset. The most frequently described pulmonary manifestation was infiltrates (43.1%; n = 116), followed by pleural effusion (7.4%; n = 20) and idiopathic interstitial pneumonia (3.3%; n = 9). Other pulmonary manifestations were: nonspecific interstitial pneumonia (n = 1), bronchiolitis obliterans (n = 3), pulmonary vasculitis (n = 6), bronchiectasis (n = 1), alveolar haemorrhage (n = 1), pulmonary embolism (n = 4), bronchial stenosis (n = 1), and alveolitis (n = 1). Several patients had one or more co-existing autoimmune/inflammatory condition. It was not reported which patients had particular pulmonary manifestations.
This is the first systematic review undertaken in VEXAS patients. Our results demonstrate that pulmonary involvement is common in this patient group. It is unclear if respiratory manifestations are part of the primary disease or a co-existing condition. Larger epidemiological analyses will aid further characterisation of pulmonary involvement and disease management.

Soybean is one of the most important sources of plant protein and is known for its wide range of agricultural, food, and industrial applications as well as health benefits. Interest in soybean proteins has been steadily growing as progressively more applications and benefits are discovered. This review article is focused on the major seed storage proteins of soybean, their three-dimensional structures, their nutritional importance and bioactive peptides, cellular synthesis, and accumulation in seeds. This will also summarize past efforts in the recombinant production of foreign proteins or bioactive peptides in soybean seed.

In recent years, increased molecular testing and improved immunohistochemical panels have facilitated more specific classification of salivary gland carcinomas, leading to recognition of several novel tumor types and unique histologic variants. Sclerosing microcystic adenocarcinoma, microsecretory adenocarcinoma, and secretory myoepithelial carcinoma are three such recently described entities that demonstrate low-grade cytology, production of prominent secretory material, and variable amounts of sclerotic stroma. This review provides a practical overview of these important and overlapping emerging entities in salivary gland pathology with a focus on distinctive histologic features and helpful ancillary studies that differentiate them from a wide range of familiar morphologic mimics.

Granulovacuolar degeneration (GVD) is a histopathological feature of Alzheimer\'s disease (AD) and some non-Alzheimer neurodegenerative diseases. It is also present in the brains of non-demented older adults. GVD is characterized by the presence of intracytoplasmic granule-containing vacuoles in most hippocampal neurons. It affects the neurons in sequential arrangement throughout the brain, which allows its characterization into different stages related to the severity of the disease. The mechanism of GVD formation is still poorly understood and its relationship with Tau structures remains unclear. Immunohistochemistry and ultrastructural examination suggest that GVD is mediated by cellular autophagic mechanisms. Other potential mechanisms related to GVD include protein accumulation caused by cellular defence mechanisms or impaired cellular functions. Several proteins are used as markers of GVD. Antibodies to cytoskeletal proteins and neurofilaments, both phosphorylated and non-phosphorylated forms, are used to stain GVD, the latter of which can be used to determine the nature of the cytoskeletal abnormalities in GVD formation. A link between GVD and microtubule-associated protein of tau was also reported but remains unclear. Previous studies reported neurons containing GVDs in the hippocampus of AD sections. Other neurodegenerative diseases also randomly showed GVDs in the brain. However, these quantitative studies have not demonstrated whether GVD is an essential component of AD or non-AD dementias. In this review, we discuss our previous quantitative results of a retrospective study from 2016 and compare them with the results of older published studies to examine whether GVD is an essential feature of AD dementia or additional neurodegenerative features. We also revisit the pathogenesis and histochemistry profile of this common pathology.

Peroxisome, a single-membrane organelle conserved in eukaryotic, is responsible for a series of oxidative reactions with its specific enzymatic components. A counterbalance between peroxisome biogenesis and degradation is crucial for the homeostasis of peroxisomes. One such degradation mechanism, termed pexophagy, is a type of selective autophagic process to deliver the excess/damaged peroxisomes into the vacuole. In plants, pexophagy is involved in the remodeling of seedlings and quality control of peroxisomes. Here, we describe the recent advance in plant pexophagy, with a focus to discuss the key regulators in plants in comparison with those in yeast and mammals, as well as future directions for pexophagy studies in plants.

BACKGROUND: Multinodular and vacuolating neuronal tumor has been recently described and included in the World Health Organization Classification of Tumors of The Central Nervous System, even though its consideration as a true tumor is controversial. Patients with these lesions usually present with refractory seizures and inconclusive imaging findings that may be confused with other more common diagnoses such as dysembryoplastic neuroepithelial tumors or low-grade gliomas. Therefore, surgical resection is warranted to reach a pathologic diagnosis and seizure control. To the best of our knowledge, only 16 cases have been published in the English literature.
METHODS: We present the case of a 52-year-old male who presented at our institution with a 2-year-history of absence of seizures. Brain MRI showed a T2-hyperintense lesion with no contrast enhancement affecting his temporal lobe. Temporal craniotomy and microsurgical resection was scheduled. The procedure was uneventful and a grayish, gluey mass was sent for pathologic analysis. The tumor was formed by immature neuronal cells organized in nodules with a vacuolated matrix. A thorough immunohistochemical analysis showed positivity for: Protein Gene Product 9.5. ATRX. OLIG2. SOX10. p16. Nestin. Synaptophysin. The findings were consistent with multinodular and vacuolating neuronal tumor. The patient has been seizure-free after surgery and with no signs of tumor progression.
CONCLUSIONS: We present a thorough review addressing this uncommon tumor along with a description of the 17th reported case of MVNT, a tumor that was described for the first time in 2013. Further studies and case studies are necessary to establish a well-defined morphological and immunohistochemical profile along with knowledge about its natural history.

Granulosa cell tumors (GCT) of the ovary are low grade tumor with a potential ability of late pelvic recurrences and distant metastases. However, there is sparse literature on the cytopathologic features of metastatic granulosa cell tumors (MGCT). Between 2000 and 2014, eight cases of MGCT were diagnosed by FNA. Clinical, cytologic, and histopathologic features were reviewed. The age ranged from 34 to 84 years. Metastases were found in abdominal wall (4 cases), pelvic mass (1 case), liver (2 cases), and lung (1 case). The time to metastasis ranged from 1 to 14 years. All cases were hypercellular, with both large and small overlapping cell clusters and individual cells. The cytologic features included: naked nuclei (8/8 cases), Call-Exner bodies (2/8 cases), and prominent metachromatic stroma (3/8 cases). Moderate cytoplasm (4/8 cases) to scant delicate cytoplasm (4/8 cases) was seen. Cytoplasmic vacuoles were also noted (6/8 cases). N/C ratios were high although lower than small round cell tumors like lymphoma. Prominent, central nucleoli were also present (6/8 cases) as well as nuclear grooves (2/8 cases). Cell block was available in 3/8 cases and all were positive for inhibin immunostain. Histologic examination of the metastatic tissue confirmed MGCT. The important cytologic features of GCT like uniformity of tumor cells, nuclear grooves and Call-Exner bodies are occasionally present in MGCT. Prominent nucleoli and many naked nuclei were frequent. Therefore, the accurate interpretation of cytologic findings of MGCT requires cytohistologic correlation and inhibin positivity to avoid unnecessary surgical interventions for diagnostic purposes. The possibility of late recurrence of GCT should be kept in mind. Diagn. Cytopathol. 2016;44:1058-1063. © 2016 Wiley Periodicals, Inc.

Malignant mesothelioma (MM) is a rare neoplasm, which is most commonly encountered in cytology through effusion specimens. Fine needle aspiration biopsy of MM, particularly the epithelioid subtype, can be a source of diagnostic difficulty and may mimic sampling of an adenocarcinoma. This is the first case report to demonstrate abundant extracellular myxoid material and numerous intracellular vacuoles, including signet ring cells, in a fine needle aspirate of metastatic MM. A review of the literature for myxoid change and vacuoles in fine needle aspiration biopsies of MM discloses that vacuoles are found in up to 35% of aspirates of MM, but myxoid change is very rare, reported in <5% of the cases. Cytologists should be aware of this rare morphologic pattern of metastatic epithelioid MM.

Chanarin-Dorfman syndrome, a \"neutral lipid storage disease with ichthyosis,\" is a multisystem inherited metabolic disorder associated with congenital ichthyosis and accumulation of lipid droplets in various types of cells.
METHODS: A 3-year-old male presented to the Pediatric Hepatology Unit, Cairo University Children\'s Hospital, Cairo, Egypt, with accidentally discovered hepatomegaly. He had generalised ichthyosis with dark skin pigmentation and bilateral ectropion. Abdominal examination revealed generalised abdominal distention with firm nontender hepatomegaly. His liver functions were deranged. Blood film showed many vacuolated neutrophils. Serum triglyceride and creatine kinase levels were elevated. Abdominal ultrasound showed a moderately enlarged liver with a bright echo pattern. Liver biopsy revealed marked diffuse macrovesicular fatty changes. The diagnosis of Chanarin-Dorfman Syndrome was made based on the dermatological, haematological, and liver biopsy findings.