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Metabolic Myopathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Muscle biopsy shows pure myopathic features with scattered atrophic, necrotic, and regenerated fibers. The most striking feature is the presence of central and subsarcolemmal vacuolations containing glycogens, stained pink-red with PAS stain (Figure 19.1a, c). The glycogen is digested by PAS-diastase. The enzyme is present in the lysosome and there is consequently abundance in acid phosphatase activity (Figure 19.1d). There is also MHC class-I upregulation in most muscle fibers, of undetermined significance.
Muscle Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Kourosh Rezania, Peter Pytel, Betty Soliven
Muscle biopsy may be morphologically normal or show accumulation of normal glycogen and, less likely, accumulation of a diastase-resistant, PAS-positive material. Enzyme histochemical staining can confirm the lack of enzyme activity. Frozen tissue can be used for biochemical testing.
Cysts and Tumours of the Bony Facial Skeleton
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Julia A. Woolgar, Gillian L. Hall
The rare clear cell odontogenic carcinoma (CCOC)95–97 predominantly affects the mandible of older females, presenting as jaw swelling with loosening of teeth and radiographically, as an ill-defined radiolucency with root resorption. Histologically, a biphasic pattern of sheets of clear cells and irregular cords of basaloid cells supported by fibrous septae is typical. Many of the tumour cells contain diastase-degradable PAS-positive granules. Differential diagnosis includes salivary gland neoplasms, clear cell variant of CEOT, metastatic renal cell carcinoma and malignant melanoma. Surgical resection may be followed by local recurrence, regional and distant (lungs, bone) metastases and prognosis is poor for large lesions. Post-operative radiotherapy should be considered especially if there is cortical erosion/perforation.
RNAi therapeutics for diseases involving protein aggregation: fazirsiran for alpha-1 antitrypsin deficiency-associated liver disease
Published in Expert Opinion on Investigational Drugs, 2023
Pavel Strnad, Javier San Martin
ERAD is the first line responsible for the clearance of misfolded Z-AAT monomers and targets abnormal proteins for ubiquitination and proteasomal degradation. However, as more Z-AAT proteins are produced, they are susceptible to interactions with other Z-AAT proteins to form polymers and their accumulation exceeds the ability of ERAD. This, in turn, activates ER stress response, which promotes protein folding as well as autophagy that is able to degrade the polymerized protein. When these protective responses are dysfunctional, overwhelmed, or otherwise unable to prevent further accumulation of the Z-AAT polymers, formation of globular inclusion bodies results. They can be visualized histologically with periodic acid – Schiff staining with diastase digestion (PAS+D). Over time, intracellular accumulation of mutant proteins leads to ER stress, hepatocellular injury, inflammation, and apoptosis, followed by compensatory hepatic regeneration. Repeated cycles of inflammation/apoptosis and chronic regeneration induce liver impairment, fibrosis, and cirrhosis, and may result in the development of end-stage liver disease [17,18]. The inability to secrete AAT protein from the liver results in a loss-of-function phenotype in the lung, where AAT is required to inhibit the activities of neutrophil elastase. Emphysema and chronic obstructive pulmonary disease are the well-known, potentially lethal pulmonary consequences of AATD.
Choroid Plexus Carcinoma with Hyaline Globules: An Uncommon Histological Finding
Published in Fetal and Pediatric Pathology, 2022
Shilpy Jha, Suvendu Purkait, Chinmaya Dash, Chinzah Lalsangzuala
Apart from the features that ascertain its malignant nature, various other morphological features are also described, including clear cell change, psammomatous calcification, hemorrhage, hyalinization, and oncocytic change [3]. To the best of our knowledge, there is only a single report of CPC with hyaline eosinophilic globules. In the reported case, the globules were PAS-positive and diastase resistant, similar to the present case. Most commonly, the hyaline globules are glycoprotein in nature. However, depending upon the associated malignancy, its character may vary. In a renal cell carcinoma and a cartilaginous neoplasm, the hyaline globules were comprised of secretory material, while in hepatocellular carcinoma and myofibrosarcoma these globules contain fine filaments [6–9]. Further, an ultrastructural examination of a case of anaplastic carcinoma of the pancreas revealed its lysosomal nature [10]. Shenoy and Desai thought the globules present in the CPC case were α1 antitrypsin in nature [4]. However, the authors did not assess the immunohistochemical nature of these globules. We found immunopositivity for AFP in the globules. AFP is one of the oncofoetal proteins and is produced by many germ cell and non-germ cell tumors apart from the yolk sac tumor. AFP immunopositive hyaline globules have been described in hepatoid variant of uterine carcinoma, carcinoid tumors, and embryonal carcinomas [11–13]. It is possible that during the process of tumorigenesis, some of the tumor cells acquired the phenotypic feature required for the production of this oncofoetal protein like other malignancies.
Eosinophilic globules in a classic ependymoma: evidence of a possible secretory role
Published in Ultrastructural Pathology, 2020
Janice S Ahn, Maureen Petersen, Allan H Friedman, Edward M López, Thomas J Cummings, Anne F Buckley, Giselle Y López
Accordingly, immunohistochemical, immunocytochemical, and ultrastructural studies show that the secretory product of the subcommissural organ in humans and other mammals is a PAS-positive, diastase-resistant, secretory substance composed of high molecular weight sialylated glycoproteins.7,10,11,16 In the ependymoma we describe, PAS and EMA both highlight glycoproteins in the eosinophilic globules, suggesting a similarity with the composition of Reissner fiber. Moreover, EMA highlights glycosylated glycoproteins typically covering apices of secretory epithelial cells. In our case, the luminal surfaces surrounding the globules were occasionally EMA-positive thereby suggesting a secretory epithelial function similar to the cells of the subcommissural organ.