Morphological Harbingers of ARMC5-Pathogenic Variant-Related Bilateral Macronodular Adrenocortical Disease
AbstractBilateral macronodular adrenocortical disease (BMAD) is a neoplastic disease associated with a high frequency of germline disease. Armadillo repeat containing 5 (ARMC5) pathogenic variants (PVs) have not been widely studied to determine the morphological and immunohistochemical characteristics of BMAD. We carried out a detailed morphologic review of 22 surgical specimens excised from patients with BMAD and compared them with PV ofARMC5 (PV  + ,n = 14) and those without (PV − ,n = 8), and further comparing them with a control group of adrenals excised from patients with renal cancer (n = 11). N...
Source: Endocrine Pathology - April 12, 2023 Category: Pathology Source Type: research

Preoperative Risk Stratification of Follicular-patterned Thyroid Lesions on Core Needle Biopsy by Histologic Subtyping and RAS Variant-specific Immunohistochemistry
AbstractFollicular-patterned lesions often have indeterminate results (diagnostic category III or IV) by core needle biopsy (CNB) and fine needle aspiration (FNA). However, CNB diagnoses follicular neoplasm (category IV) more frequently than FNA. Therefore, we aimed to develop a risk stratification system for CNB samples with category III/IV using immunohistochemistry (IHC). The specificity of the RAS Q61R antibody was validated on 58 thyroid nodules with six different types of  RAS genetic variants and 40 cases of RAS wild-type. We then applied IHC analysis of RAS Q61R to 207 CNB samples with category III/IV in which al...
Source: Endocrine Pathology - April 11, 2023 Category: Pathology Source Type: research

Prognostic Value of Microscopic Tumor Necrosis in Adrenal Cortical Carcinoma
AbstractAdrenal cortical carcinoma (ACC) is an uncommon neoplasm with variable prognosis. Several histologic criteria have been identified as predictors of malignancy in adrenal cortical tumors. The Weiss score is the system most widely employed for diagnostic purposes, but also possesses prognostic value. We aim to determine the relative impact of each Weiss parameter on ACC patient survival. A multicenter retrospective analysis was conducted on a series of 79 conventional ACCs surgically treated at the Florence and Utrecht centers of the European Network for the Study of Adrenal Tumors (ENSAT). Weiss classification was r...
Source: Endocrine Pathology - March 23, 2023 Category: Pathology Source Type: research

Insights Obtained from the Nontumorous Glandular Tissue in Patients with Endocrine Tumors
AbstractThe pathology of neoplasia tends to focus on the tumor that requires characterization, grading, and staging. However, nontumorous tissue surrounding the lesion can also provide information, particularly about pathogenetic mechanisms. In endocrine tissues, this takes the form of precursor lesions that characterize several genetic predisposition syndromes. In addition, because of the unique functional aspects of endocrine neoplasia, the nontumorous tissue provides evidence of hormone excess, with hyperplasia and/or atrophy and other involutional changes allowing the pathologist to confirm both hormone function by the...
Source: Endocrine Pathology - March 21, 2023 Category: Pathology Source Type: research

The Spectrum of Endocrine Pathology
AbstractEndocrine pathology comprises a spectrum of disorders originating in various sites throughout the body. Some disorders affect endocrine glands, and others arise from endocrine cells that are dispersed in non-endocrine tissues. Endocrine cells can broadly be classified as neuroendocrine, steroidogenic, or thyroid follicular cells; these three families have distinct embryologic origins, morphologic structure, and biochemical hormone synthetic pathways. Lesions affecting the endocrine system include developmental abnormalities, inflammatory processes that can be infectious or autoimmune, hypofunction with atrophy or h...
Source: Endocrine Pathology - March 18, 2023 Category: Pathology Source Type: research

Update on C-Cell Neuroendocrine Neoplasm: Prognostic and Predictive Histopathologic and Molecular Features of Medullary Thyroid Carcinoma
AbstractMedullary thyroid carcinoma (MTC) is a C-cell-derived epithelial neuroendocrine neoplasm. With the exception of rare examples, most are well-differentiated epithelial neuroendocrine neoplasms (also known as neuroendocrine tumors in the taxonomy of the International Agency for Research on Cancer [IARC] of the World Health Organization [WHO]). This review provides an overview and recent evidence-based data on the molecular genetics, disease risk stratification based on clinicopathologic variables including molecular profiling and histopathologic variables, and targeted molecular therapies in patients with advanced MT...
Source: Endocrine Pathology - March 8, 2023 Category: Pathology Source Type: research

Impact of Morphology in the Genotype and Phenotype Correlation of Bilateral Macronodular Adrenocortical Disease (BMAD): A Series of Clinicopathologically Well-Characterized 35 Cases
AbstractBilateral macronodular adrenocortical disease (BMAD) is characterized by the development of adrenal macronodules resulting in a pituitary-ACTH independent Cushing ’s syndrome. Although there are important similarities observed between the rare microscopic descriptions of this disease, the small series published are not representative of the molecular and genetic heterogenicity recently described in BMAD. We analyzed the pathological features in a series of BMAD and determined if there is correlation between these criteria and the characteristics of the patients. Two pathologists reviewed the slides of 35 patients...
Source: Endocrine Pathology - March 2, 2023 Category: Pathology Source Type: research

Sarcomatous Transformation of a Medically Treated Lactotroph Pituitary Neuroendocrine Tumor?
(Source: Endocrine Pathology)
Source: Endocrine Pathology - February 24, 2023 Category: Pathology Source Type: research

Diagnostic, Prognostic, and Predictive Role of Ki67 Proliferative Index in Neuroendocrine and Endocrine Neoplasms: Past, Present, and Future
AbstractThe introduction of Ki67 immunohistochemistry in the work-up of neuroendocrine neoplasms (NENs) has opened a new approach for their diagnosis and prognostic evaluation. Since the first demonstration of the prognostic role of Ki67 proliferative index in pancreatic NENs in 1996, several studies have been performed to explore its prognostic, diagnostic, and predictive role in other neuroendocrine and endocrine neoplasms. A large amount of information is now available and published results globally indicate that Ki67 proliferative index is useful to this scope, although some differences exist in relation to tumor site ...
Source: Endocrine Pathology - February 17, 2023 Category: Pathology Source Type: research

The Impact of the 2022 WHO Classification of Thyroid Neoplasms on Everyday Practice of Cytopathology
AbstractThis review outlines how the alterations in the 5th edition of the WHO Classification of Endocrine and Neuroendocrine Tumors of the thyroid gland are likely to impact thyroid cytopathology. It is important to note that WHO subclassifies thyroid tumors into several new categories based on increased comprehension of the cell of origin, pathologic features (including cytopathology), molecular classification, and biological behavior. The 3rd edition of theBethesda System for Reporting Thyroid Cytopathology (TBSRTC) will debut in the near future and will include changes in diagnostic category designations. The changes i...
Source: Endocrine Pathology - February 17, 2023 Category: Pathology Source Type: research

Thyroblastoma —a Primitive Multilineage Thyroid Neoplasm with Somatic DICER1 Alteration
(Source: Endocrine Pathology)
Source: Endocrine Pathology - February 15, 2023 Category: Pathology Source Type: research

Sclerosing Mucoepidermoid Carcinoma with Eosinophilia of the Thyroid and Mucoepidermoid Carcinoma of the Thyroid: What ’s in a Name?
(Source: Endocrine Pathology)
Source: Endocrine Pathology - February 9, 2023 Category: Pathology Source Type: research

The Thyroid Pathologist Meets Therapeutic Pharmacology
AbstractThe effects of many pharmacological agents on thyroid function are well known. Direct influences on measurements of thyroid function tests are also described. However, certain classes of drugs produce morphological changes in the gland. This review focuses on the significance of the following drug classes for the thyroid pathologist: iodine, antithyroid drugs, psychotropic drugs, antibiotics, cardiotropic drugs, antidiabetic drugs, and immunomodulatory agents. Radioactive iodine initially induces mild histologic changes; however, the long-term effects include marked follicular atrophy, fibrosis, and nuclear atypia ...
Source: Endocrine Pathology - February 1, 2023 Category: Pathology Source Type: research

The Presence of Typical “BRAFV600E-Like” Atypia in Papillary Thyroid Carcinoma is Highly Specific for the Presence of the BRAFV600E Mutation
AbstractPapillary thyroid carcinomas (PTCs) are driven by a variety of molecular abnormalities includingBRAF,RAS,ALK,RET, andNTRK alterations. PTCs driven by theBRAFV600E mutation, or tumours which demonstrate a similar gene expression profile to PTCs driven by this mutation, have been reported to demonstrate specific morphological features sometimes termed “BRAFV600E-like” atypia. BRAFV600E-like atypia is characterised by a well-developed papillary architecture, infiltrative growth, marked nuclear clearing, prominent intranuclear pseudoinclusions, abundant eosinophilic cytoplasm, and scattered psammoma bodies. We soug...
Source: Endocrine Pathology - January 28, 2023 Category: Pathology Source Type: research

FLCN-Driven Functional Adrenal Cortical Carcinoma with High Mitotic Tumor Grade: Extending the Endocrine Manifestations of Birt-Hogg-Dub é Syndrome
AbstractAdrenal cortical carcinoma is an aggressive and rare malignancy of steroidogenic cells of the adrenal gland. Most adult adrenal cortical carcinomas are sporadic, but a small fraction may be associated with inherited tumor syndromes, such as Li-Fraumeni, multiple endocrine neoplasia 1, Lynch syndrome, and Beckwith-Wiedemann syndrome, as well as isolated case reports of non-syndromic manifestations occurring in the context of other pathogenic germline variants. Birt-Hogg-Dub é (BHD) is a rare autosomal dominant syndrome caused by germline pathogenic variants in theFLCN gene. BHD syndrome causes a constellation of sy...
Source: Endocrine Pathology - January 26, 2023 Category: Pathology Source Type: research