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Introduction  Clival tumors are rare and heterogeneous. Although some benign prototypical sellar lesions may present as clival tumors, the likelihood of malignant disease is higher. Here we define a novel algorithm for the workup and management of clival masses through an illustrative case of colorectal adenocarcinoma metastasis to the clivus. Methods  In this case report, the best practice guidelines for managing clival masses are described through a literature review and refined by senior author consensus. We conducted a focused systematic review to characterize the present case in the context of clival metastasis from gastrointestinal malignancy. Results  An 83-year-old woman presented with 4 weeks of headaches and blurry vision. Examination revealed partial right abducens and left oculomotor palsies. Magnetic resonance imaging (MRI) identified a large, weakly enhancing sellar and clival mass with sphenoid sinus extension. An aggressive subtotal endoscopic endonasal resection was performed with removal of all sphenoid, clival, and sellar disease without cavernous sinus wall resection. Pathology confirmed colorectal adenocarcinoma; computed tomography (CT) imaging identified an ascending colon mass with metastases to the liver and mesenteric nodes. Palliative oncologic therapies were recommended, but she elected hospice, and died 3 months after initial presentation. Gastrointestinal clival metastases are exceedingly rare among sellar and clival pathologies, with eight prior cases reported, most of which presented with diplopia from abducens nerve involvement. Conclusion  Clival masses are uncommon skull base lesions that are associated with more aggressive diseases. We present a consolidated framework for decision-making in these challenging patients, alongside an unusual case example that illustrates the importance of increased suspicion for malignant clinical entities in this setting., Competing Interests: Conflict of Interest CSG serves as Editor-in-Chief for the Journal of Neurological Surgery Reports ., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/ ).)

Background: Primary squamous cell carcinoma of the thyroid is a very rare malignancy with aggressive growth and poor prognosis. There is currently no consensus for treatment modality, however, most patients with primary squamous cell carcinoma of the thyroid are treated with surgery and adjuvant chemoradiation., Case Presentation: We report a rare case of primary squamous cell carcinoma of the thyroid in a 68-year-old White male who underwent chemoradiation and palliative immunotherapy after declining surgery. He was treated with intensity-modulated radiation therapy to 70 Gy in 35 fractions, with concurrent carboplatin-paclitaxel and palliative pembrolizumab. Local thyroid disease recurrence occurred at 6 months post-chemoradiation, and the patient died at 16 months post-chemoradiation., Conclusions: This is the first case report demonstrating the use of pembrolizumab as palliative therapy for primary squamous cell carcinoma of the thyroid. Our study also highlights the importance of chemoradiation in decreasing primary mass size and immunotherapy in preventing metastatic disease progression., (© 2022. The Author(s).)

Thoracic endometriosis is very rare. Usually, the thorax is the most frequent affected site outside the pelvis. Common symptoms include chest pain, dyspnea, and hemoptysis. Common manifestations include pneumothorax, hemothorax, and pulmonary or pleural nodules. In addition, symptoms and manifestations can be "catamenial" happening a few days after menstruation onset. This disease can be debilitating, causing a significant impact on the quality of life of young women. We present a case of a young female who was referred to our hospital with recurrent right-sided pleural effusions and pneumothoraces. Pleural fluid drainage was consistent with hemothorax. Transvaginal ultrasound showed mild intraperitoneal fluid in the Cul-de-Sac. Due to concerns for thoracic endometriosis, video-assisted thoracoscopic surgery was performed confirming the diagnosis by pathology. Therapeutic pleurectomy with diaphragmatic repair and pleurodesis was performed. The patient was started on medroxyprogesterone acetate injections two weeks after with great clinical response.

Context.—: Program requirements for Selective Pathology fellowships in the United States were established by the Accreditation Council for Graduate Medical Education (ACGME) in 2011 to govern fellowships providing advanced training in surgical pathology, focused anatomic pathology, or focused clinical pathology. Selective Pathology entered the ACGME's Next Accreditation System in 2015 with the introduction of the Selective Pathology Milestones 1.0, a set of benchmarks for evaluating fellow progress in each of the 6 ACGME core competencies. In 2019, the ACGME convened a work group for a planned periodic update to these milestones., Objective.—: To summarize changes to the Selective Pathology milestones., Design.—: The study design featured expert opinion and survey., Results.—: The Patient Care milestones for anatomic pathology-focused fellowships contain a renewed emphasis on both gross and microscopic examination, whereas for clinical pathology-focused fellowships, the emphasis is on interpretation of laboratory assays. The milestones for the non-Patient Care, non-Medical Knowledge competencies have been updated to a harmonized set of milestones designed to extend across all specialties and subspecialties. New to the milestones program is a supplemental guide that provides examples, suggested assessment tools, and references to aid in implementation. Public comments were supportive of the changes., Conclusions.—: The Milestones 2.0 are set for implementation in July 2021. Updates in the new milestones are aimed at facilitating training and harmonizing evaluation across subspecialties.

Introduction: Meningiomas are the most common primary central nervous system (CNS) tumor. They are most often benign, but a subset of these can behave aggressively. Current World Health Organization (WHO) guidelines classify meningiomas into three grades based on the histologic findings and presence or absence of brain invasion. These grades are intended to guide treatment, but meningiomas can behave inconsistently with regard to their assigned histopathological grade, influencing patient expectations and management. Advanced molecular profiling of meningiomas has led to the proposal of alternative molecular grading schemes that have shown superior predictive power. These include methylation patterns, copy number alterations, and mutually exclusive driver mutations affecting oncogenes, including BAP1, CDKN2A/B , and the TERT promoter, which are associated with particularly aggressive tumor biology. Despite the evident clinical value, advanced molecular profiling methods are not widely incorporated in routine clinical practice for meningiomas., Objective: To assess the degree of concordance between the molecular profile of meningiomas and the histopathologic WHO classification, the current method of predicting meningioma behavior., Methods: In a two-year single-institution experience, we used commercially available resources to determine molecular profiles of all resected meningiomas. Copy number aberrations and oncogenic driver mutations were identified and compared with the histopathologic grade., Results: One hundred fifty-one total meningioma cases were included for analysis (85.4% WHO grade 1, 13.3% WHO grade 2, and 1.3% grade 3). Chromosomal analysis of 124 of these samples showed that 29% of WHO grade 1 tumor featured copy number profiles consistent with higher grade meningioma, and 25% of WHO grade 2 meningiomas had copy number profiles consistent with less aggressive tumors. Furthermore, 8% harbored mutations in TERT, CDKN2A/B , or BAP1 of which 6% occurred in grade 1 meningiomas., Conclusions: Routine advanced molecular profiling of all resected meningiomas using commercially available resources allowed for identification of a significant number of meningiomas whose molecular profiles were inconsistent with WHO grade. Our work shows the clinical value of integrating routine molecular profiling with histopathologic grading to guide clinical decision making., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Roehrkasse, Peterson, Fung, Pelargos and Dunn.)

Atypical teratoid/rhabdoid tumor (ATRT) is a high-grade central nervous system tumor, with poor prognosis despite intensive multimodal therapy. Loss of nuclear immunostaining for INI1 due to inactivation of the hSNF5/INI1 tumor suppressor gene is pathognomonic of ATRT. We present a patient with congenital ATRT, who had spontaneous tumor regression without therapy, and is disease-free 4 years later. Tumor histopathology showed rhabdoid cells characteristic of ATRT, but immunohistochemistry revealed heterogeneous loss of nuclear INI1 staining. The populations of INI1-intact and INI1-deficient cells were separated by laser microdissection, for molecular analysis with DNA sequencing and fluorescence in situ hybridization. The INI1-negative cells were found to harbor a heterozygous deletion and truncating mutation of the hSNF5/INI1 locus, while the INI1-intact cells had 2 copies of the wild-type INI1 gene. To our knowledge, this is the first report of spontaneous regression of ATRT, with molecular heterogeneity for SMARCB1 inactivation, with no radiographic signs of recurrence at 4 years after diagnosis.

Target Population: Adults diagnosed with vestibular schwannomas., Question 1: What is the prognostic significance of Antoni A vs B histologic patterns in vestibular schwannomas?, Recommendation: No recommendations can be made due to a lack of adequate data., Question 2: What is the prognostic significance of mitotic figures seen in vestibular schwannoma specimens?, Recommendation: No recommendations can be made due to a lack of adequate data., Question 3: Are there other light microscopic features that predict clinical behavior of vestibular schwannomas?, Recommendation: No recommendations can be made due to a lack of adequate data., Question 4: Does the KI-67 labeling index predict clinical behavior of vestibular schwannomas?, Recommendation: No recommendations can be made due to a lack of adequate data., Question 5: Does the proliferating cell nuclear antigen labeling index predict clinical behavior of vestibular schwannomas?, Recommendation: No recommendations can be made due to a lack of adequate data., Question 6: Does degree of vascular endothelial growth factor expression predict clinical behavior of vestibular schwannomas?, Recommendation: No recommendations can be made due to a lack of adequate data.  The full guideline can be found at: https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter_6., (Copyright © 2017 by the Congress of Neurological Surgeons)

Competency-based medical education has evolved over the past decades to include the Accreditation Council for Graduate Medical Education Accreditation System of resident evaluation based on the Milestones project. Entrustable professional activities represent another means to determine learner proficiency and evaluate educational outcomes in the workplace and training environment. The objective of this project was to develop entrustable professional activities for pathology graduate medical education encompassing primary anatomic and clinical pathology residency training. The Graduate Medical Education Committee of the College of American Pathologists met over the course of 2 years to identify and define entrustable professional activities for pathology graduate medical education. Nineteen entrustable professional activities were developed, including 7 for anatomic pathology, 4 for clinical pathology, and 8 that apply to both disciplines with 5 of these concerning laboratory management. The content defined for each entrustable professional activity includes the entrustable professional activity title, a description of the knowledge and skills required for competent performance, mapping to relevant Accreditation Council for Graduate Medical Education Milestone subcompetencies, and general assessment methods. Many critical activities that define the practice of pathology fit well within the entrustable professional activity model. The entrustable professional activities outlined by the Graduate Medical Education Committee are meant to provide an initial framework for the development of entrustable professional activity-related assessment and curricular tools for pathology residency training., Competing Interests: Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.