Single Center Experience with Isolated Spinal Cord Neurosarcoidosis.

INTRODUCTION: Isolated spinal cord neurosarcoidosis is extremely rare. The potential implications of long-term immunosuppressant therapy make correct diagnosis imperative. However, there are challenges inherent in isolated spinal cord involvement that require a multidisciplinary approach. Here we present the largest series of definite and possible isolated spinal neurosarcoidosis and discuss our institutional experience in managing this rare but morbid condition. METHODS: A retrospective review was performed to identify all neurosarcoidosis cases starting from 2002 to 2020 at our institution. Patients were screened for cases of isolated spinal neurosarcoidosis. A descriptive analysis was performed for each case. RESULTS: A total of 64 cases of neurosarcoidosis were identified. The spine was involved in 26 (40.6%) patients. Only 4 (6.3%) cases had isolated spinal cord involvement. A full medical and imaging workup was performed in determining isolated spinal cord involvement. Three patients subsequently underwent surgical biopsy, and 1 did not undergo biopsy because of patient preference. One of the patients who underwent biopsy had an initial nondiagnostic biopsy and had a repeat biopsy. Corticosteroids were employed in all cases with additional immunosuppressive agents for maintenance therapy and refractory cases. All showed radiographic improvement and were clinically stable to improved. CONCLUSION: Isolated spinal cord involvement of neurosarcoidosis is rare and can present challenges in diagnosis. A biopsy can be performed when necessary. However, a biopsy of the spinal cord carries inherent risks and may not always be possible or result in a nondiagnostic sample. In the setting of high clinical suspicion, maximal medical therapy is still employed.

Teaching Pathology in an Integrated Preclinical Medical School Curriculum and Adaptations to COVID-19 Restrictions.

The coronavirus disease 2019 (COVID-19) pandemic has had a major impact on education at all age levels, including professional schools and health professions programs. We describe the experience of adapting preclinical medical school courses within an integrated curriculum to virtual instruction. A major feature of two of the courses were pathology small groups adapted from pathology courses in the previous medical school curriculum. These small groups were designed to use facilitated groups of 8 to 10 students. With a sudden change to virtual learning, these small groups were shifted to large group virtual sessions. In general, the conversion went well, with ongoing optimization of the format of the large group sessions mainly occurring over the first several sessions. End-of-course student evaluations were generally positive, but with a preference toward returning to live sessions in the future. Scores on 5 multiple choice examinations in the spring 2020 course were essentially identical in mean, standard deviation, and distribution to examinations in the previous 2 years of the course that had similar layout and topic organization. We discuss the challenges and successes of the switch to virtual instruction and of teaching pathology content within an integrated medical school curriculum.

Visual and Hormone Outcomes in Pituitary Apoplexy: Results of a Single Surgeon, Single Institution 15-Year Retrospective Review and Pooled Data Analysis.

Introduction Pituitary apoplexy commonly presents with visual and hormonal deficits. While traditionally regarded as an emergency, there have been increasing trends toward conservative management. Our institutional practice consists of early surgery; therefore, we reviewed our series evaluating vision outcomes, hormone function, and complications compared with the present literature. Methods We retrospectively reviewed our institution's medical records to identify pituitary apoplexy patients who were treated via the endoscopic endonasal approach by a single neurosurgeon (senior author). We recorded basic demographics, radiographic and operative features, and preoperative and postoperative vision and hormone status. Univariate and multivariate statistical analyses were performed. Pooled data analysis of visual outcomes in the current literature using Bayesian inference was performed. Results We identified 44 patients with histologically confirmed pituitary apoplexy treated by endoscopic transsphenoidal decompression; 77% were treated within 24 hours of presentation. Total 45% had cranial nerve (CN) palsy, 36% anopsia, and 20% had visual acuity deficits. Postoperatively, 100% of CN palsies improved, 81% of anopsias improved, and 66.7% of visual acuity deficits improved. Long-lasting panhypopituitarism (25%) and hypothyrodism (22%) were common. Cavernous sinus involvement predicted residual tumor ( p = 0.006). Pooled Bayesian inference showed 30% improvement in vision outcomes with surgical management compared with medical management with a number needed to treat of 3.3. Conclusion Early surgery for pituitary apoplexy was associated with excellent visual outcomes and the need for long-term hormone replacement is common. Cavernous sinus involvement is an independent predictor of residual tumor. Pooled statistical analysis favors aggressive surgical management of apoplexy for improved visual outcomes.

Brain metastases from adenocarcinoma of the lung with truly cystic magnetic resonance imaging appearance.

Metastatic disease represents over half of all malignancies in brain parenchyma, and carcinoma when metastatic will often spread to the brain, with lung and breast tumors being the most common culprits. The suggestive features of metastatic disease on magnetic resonance imaging include peritumoral, vasogenic edema and avid postcontrast enhancement. We present the case of a 50-year-old male with an established diagnosis of metastatic adenocarcinoma of the lung treated with erlotinib who developed multiple cystic brain lesions on surveillance MRI. These cysts demonstrated T2 prolongation, suppressed completely on FLAIR, lacked surrounding edema, and featured a complete lack of enhancement. Due to the ambiguous imaging findings, brain biopsy was performed to establish the diagnosis. The pathology revealed a single layer of malignant cells lining brain parenchyma and focal areas of glandular growth. The intracranial lesions responded well to total brain radiation. This case is unique for the imaging findings most characteristic of simple cysts in biopsy-proven metastatic disease and may relate to the effects of erlotinib on metastatic brain tumors.

Using Focused Laboratory Management and Quality Improvement Projects to Enhance Resident Training and Foster Scholarship.

Training in patient safety, quality, and management is widely recognized as an important element of graduate medical education. These concepts have been intertwined in pathology graduate medical education for many years, although training programs face challenges in creating explicit learning opportunities in these fields. Tangibly involving pathology residents in management and quality improvement projects has the potential to teach and reinforce key concepts and further fulfill Accreditation Council for Graduate Medical Education goals for pursuing projects related to patient safety and quality improvement. In this report, we present our experience at a pathology residency program (University of Iowa) in engaging pathology residents in projects related to practical issues of laboratory management, process improvement, and informatics. In this program, at least 1 management/quality improvement project, typically performed during a clinical chemistry/management rotation, was required and ideally resulted in a journal publication. The residency program also initiated a monthly management/informatics series for pathology externs, residents, and fellows that covers a wide range of topics. Since 2010, all pathology residents at the University of Iowa have completed at least 1 management/quality improvement project. Many of the projects involved aspects of laboratory test utilization, with some projects focused on other areas such as human resources, informatics, or process improvement. Since 2012, 31 peer-reviewed journal articles involving effort from 26 residents have been published. Multiple projects resulted in changes in ongoing practice, particularly within the hospital electronic health record. Focused management/quality improvement projects involving pathology residents can result in both meaningful quality improvement and scholarly output.

Pathology Milestones: Assessing Clinical Competency by Committee.

All Accreditation Council for Graduate Medical Education accredited pathology residency training programs are now required to evaluate residents using the new Pathology Milestones assessment tool. Similar to implementation of the 6 Accreditation Council for Graduate Medical Education competencies a decade ago, there have been challenges in implementation of the new milestones for many residency programs. The pathology department at the University of Iowa has implemented a process that divides the labor of the task in rating residents while also maintaining consistency in the process. The process is described in detail, and some initial trends in milestone evaluation are described and discussed. Our experience indicates that thoughtful implementation of the Pathology Milestones can provide programs with valuable information that can inform curricular changes.

Identification of a novel, recurrent SLC44A1-PRKCA fusion in papillary glioneuronal tumor.

Mixed neuronal-glial tumors are rare and challenging to subclassify. One recently recognized variant, papillary glioneuronal tumor (PGNT), is characterized by prominent pseudopapillary structures and glioneuronal elements. We identified a novel translocation, t(9;17)(q31;q24), as the sole karyotypic anomaly in two PGNTs. A fluorescence in situ hybridization (FISH)-based positional cloning strategy revealed SLC44A1, a member of the choline transporter-like protein family, and PRKCA, a protein kinase C family member of serine/threonine-specific protein kinases, as the 9q31 and 17q24 breakpoint candidate genes, respectively. Reverse transcription-polymerase chain reaction (RT-PCR) analysis using a forward primer from SLC44A1 exon 5 and a reverse primer from PRKCA exon 10 confirmed the presence of a SLC44A1-PRKCA fusion product in both tumors. Sequencing of each chimeric transcript uncovered an identical fusion cDNA junction occurring between SLC44A1 exon 15 and PRKCA exon 9. A dual-color breakpoint-spanning probe set custom-designed for interphase cell recognition of the translocation event identified the fusion in a third PGNT. These results suggest that the t(9;17)(q31;q24) with the resultant novel fusion oncogene SLC44A1-PRKCA is the defining molecular feature of PGNT that may be responsible for its pathogenesis. The FISH and RT-PCR assays developed in this study can serve as valuable diagnostic adjuncts for this rare disease entity.

Hypertrophic spinal pachymeningitis with thoracic myelopathy: the initial presentation of ANCA-related systemic vasculitis.

STUDY DESIGN: A retrospective case review combined with a review of current literature. OBJECTIVES: We describe a case of antineutrophil cytoplasmic antibodies (ANCA)-related systemic vasculitis, with nearly 4 years of clinical and radiographic follow-up, initially presenting as hypertrophic spinal pachymeningitis (HSP). The diagnosis, surgical, and medical treatment of HSP are discussed in the context of a literature review. SUMMARY OF BACKGROUND DATA: HSP is a rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. HSP has been associated with infectious, inflammatory, autoimmune, and traumatic conditions. Surgical decompression and/or corticosteroid therapy have been shown effective at managing this disorder, but identifying associated diseases can be essential to a favorable patient outcome. METHODS: The medical, pathologic, and radiographic records of this case were reviewed. A computer-based search of the PubMed database was used to perform a comprehensive literature review of HSP. RESULTS: We report a 66-year-old male with a history of renal cell carcinoma and bladder cancer who presented with upper abdominal pain, thoracic myelopathy (progressive weakness and numbness of the lower extremities, and gait disturbances), and weight loss. A diagnosis of HSP was subsequently shown to be the initial presentation of ANCA-related systemic vasculitis. CONCLUSIONS: The possibility of an ANCA-related systemic vasculitis presenting as HSP without systemic signs is a diagnostic and therapeutic challenge for the spinal surgeon. The diagnosis of HSP should initiate a search for an associated disease process and close follow-up after initial treatment. With interdisciplinary collaboration between medicine, radiology, pathology, and orthopedics, the diagnosis was made and a treatment initiated which halted disease progression and has maintained remission for more than 4 years.

Competency assessment of residents in surgical pathology using virtual microscopy.

Our goal was to develop an efficient and reliable performance-based virtual slide competency examination in general surgical pathology that objectively measures pathology resident's morphologic diagnostic skill. A Perl scripted MySQL database was used to develop the test editor and test interface. Virtual slides were created with the Aperio ScanScope. The examination consisted of 20 questions using 20 virtual slides. Slides were chosen to represent general surgical pathology specimens from a variety of organ systems. The examination was administered in a secure environment and was completed in 1 to 1 1/2 hours. Examination reliability, as an indicator of the test's ability to discriminate between trainee ability levels, was excellent (r = 0.84). The linear correlation coefficient of virtual slide competency examination score versus months of surgical pathology training was 0.83 (P = .0001). The learning curve was much steeper early in training. Correlation of virtual slide competency examination performance with resident's performance on the 64 item Resident In-Service Examination surgical pathology subsection was 0.70. Correlation of virtual slide competency examination performance with global end of rotation ratings was 0.28. This pilot implementation demonstrates that it is possible to create a short, reliable performance-based assessment tool for measuring morphologic diagnostic skill using a virtual slide competency examination. Furthermore, the examination as implemented in our program will be a valid measure of an individual resident's progress in morphologic competency. Virtual slide technology and computer accessibility have advanced to the point that the virtual slide competency examination model implemented in our program could have applicability across multiple residency programs.

Accreditation council for graduate medical education (ACGME) competencies in neuropathology training.

The Accreditation Council for Graduate Medical Education (ACGME) has defined 6 core competencies for all physicians: patient care; medical knowledge; practice-based learning and improvement; interpersonal and communication skills; professionalism; and systems-based practice. However, the specific wording of the descriptions often assumes that the physician is a clinician rather than a pathologist. Therefore, the American Association of Neuropathologists, Inc. asked its Professional Affairs Committee to examine the core competencies and determine how they relate to training in neuropathology. The Committee's report is presented here in 6 sections, corresponding to the 6 competencies. In each section, the ACGME definition of that particular competency is either quoted directly or, more often, modified slightly to clarify how the competency applies to neuropathology. Each of the defined competencies is then followed by possible assessment tools, selected from those recommended in the ACGME's "toolbox." Specific suggestions are given for designing tools that apply to neuropathology. Many of the suggested activities and documentation methods can be combined into efficient, carefully formulated training/evaluation exercises. Different tools may be more applicable in some training programs.