Midline brain hamartomatous lesions in fibrodysplasia ossificans progressiva with ACVR1 mutations.

Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by extensive heterotopic ossification of soft tissue structures leading to severe limitations in movement. FOP is caused by a germline mutation in the activating receptor type IA (ACVR1) gene. Worrisome is the fact that up to a third of diffuse intrinsic pontine gliomas (DIPG) also harbor the same point mutation in ACVR1.

Educational Case: Esophageal Carcinoma.

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What Every Neuropathologist Needs to Know: Neuropathology and the US Legal System.

The legal system of the United States is complex, with nuances that are particular to its many jurisdictions. The neuropathologist may professionally interact with the legal system in both criminal and civil proceedings as either a fact or expert witness, and in rare instances as a defendant. The nature of the legal issue at hand will define the pathologist's role and determine what actions are required or requested.

Neurofibromatosis: A Review of NF1, NF2, and Schwannomatosis.

The neurofibromatoses are a heterogeneous group of hereditary cancer syndromes that lead to tumors of the central and peripheral nervous systems, as well as other organ systems. By far the most common form is neurofibromatosis 1 (96%), followed by neurofibromatosis 2 (3%), and a more recently recognized, lesser known form, schwannomatosis. The diagnostic criteria, pathogenesis, molecular considerations, and clinical manifestations are discussed in this review article.

CNS intraoperative consultation: a survival guide for non-neuropathologists.

Intraoperative consultations for central nervous system disease may be challenging due to limitations of sample size, lack of familiarity with neurosurgical procedures, or poor access to neuroimaging studies. Despite these challenges, the surgical pathologist is charged with determining if the tissue sample is representative of the pathologic process while ensuring that enough diagnostic tissue has been retained for routine histology, immunohistochemistry, fluorescence in situ hybridization, molecular testing, and in some cases, tissue banking. Here, we present basic methods and a practical approach for CNS intraoperative consultation including critical pre-analytic considerations that promote optimal tissue management.

Primary Hodgkin lymphoma of the central nervous system: two case reports and review of the literature.

CNS involvement by systemic Hodgkin lymphoma (HL) is quite rare, but the disease limited to the CNS is an exceptionally rare entity. The incidence of CNS-HL has been estimated at 0.2-0.