Intraoperative Magnetic Resonance Imaging-Guided Biopsy in the Diagnosis of Suprasellar Langerhans Cell Histiocytosis.

BACKGROUND: Magnetic resonance imaging (MRI)-guided biopsy is an emerging diagnostic technique that holds great promise for otherwise difficult to access neuroanatomy. CASE DESCRIPTION: Here we describe MRI-guided biopsy of a suprasellar lesion located posterior and superior to the pituitary stalk. The approach was implemented successfully in a 38-year-old woman who had developed progressive visual deterioration. CONCLUSION: Intraoperative MRI revealed the need for trajectory adjustment due to an unintended, minor deviation in the burr hole entry point, demonstrating the benefit of an MRI-guided approach. Langerhans cell histiocytosis was diagnosed after biopsy, and the lesion regressed after cladribine treatment. Technical nuances of the case are reviewed in the context of the available literature.

Imaging correlates for the 2016 update on WHO classification of grade II/III gliomas: implications for IDH, 1p/19q and ATRX status.

The 2016 World Health Organization Classification of Tumors of the Central Nervous System incorporates the use of molecular information into the classification of brain tumors, including grade II and III gliomas, providing new prognostic information that cannot be delineated based on histopathology alone. We hypothesized that these genomic subgroups may also have distinct imaging features. A retrospective single institution study was performed on 40 patients with pathologically proven infiltrating WHO grade II/III gliomas with a pre-treatment MRI and molecular data on IDH, chromosomes 1p/19q and ATRX status. Two blinded Neuroradiologists qualitatively assessed MR features. The relationship between each parameter and molecular subgroup (IDH-wildtype; IDH-mutant-1p/19q codeleted-ATRX intact; IDH-mutant-1p/19q intact-ATRX loss) was evaluated with Fisher's exact test. Progression free survival (PFS) was also analyzed. A border that could not be defined on FLAIR was most characteristic of IDH-wildtype tumors, whereas IDH-mutant tumors demonstrated either well-defined or slightly ill-defined borders (p = 0.019). Degree of contrast enhancement and presence of restricted diffusion did not distinguish molecular subgroups. Frontal lobe predominance was associated with IDH-mutant tumors (p = 0.006). The IDH-wildtype subgroup had significantly shorter PFS than the IDH-mutant groups (p < 0.001). No differences in PFS were present when separating by tumor grade. FLAIR border patterns and tumor location were associated with distinct molecular subgroups of grade II/III gliomas. These imaging features may provide fundamental prognostic and predictive information at time of initial diagnostic imaging.

Erratum to: Imaging correlates for the 2016 update on WHO classification of grade II/III gliomas: implications for IDH, 1p/19q and ATRX status.

In the initial online publication, the values in the last two rows in Table 1 were in the wrong rows. The original article has been corrected.

Ruptured Dural Arteriovenous Fistula/Malformation.

When "common things are common," the discovery of a subdural hemorrhage in an adult is most likely to be due to trauma. When the subdural hemorrhage is associated with an intraparenchymal hematoma, statistically speaking, the subdural hemorrhage is likely the result of a hypertensive hemorrhage that has ruptured into the subdural space or trauma that resulted from a collapse to the ground following hypertensive intra-axial bleeding. However, "common things" do not always explain the source of a subdural hemorrhage or intraparenchymal hematoma. In this case, an adult woman presented to the hospital obtunded and was diagnosed with a subdural hemorrhage (with mass effect) and intraparenchymal hematoma as the result of a ruptured dural arteriovenous fistula/malformation. This case highlights an unusual source of intracranial bleeding that resulted in death.

Unusual and Rare Locations for Craniopharyngiomas: Clinical Significance and Review of the Literature.

OBJECTIVE: This study discusses rare and unusual locations of primary craniopharyngiomas. METHODS: We describe a case of a craniopharyngioma in the cerebellopontine angle. As a result of this unusual location, we performed a literature review of the ectopic occurrence of craniopharyngiomas using Pubmed, Cochrane Database, Trip, and Google Scholar to search for the terms "unusual," "uncommon," and "ectopic" in combination with "craniopharyngioma." The bibliographies of relevant articles were also searched. RESULTS: We found 28 reported cases of rare anatomic locations for primary craniopharyngiomas. The average age of the patients was 30.8 years. Several patients had Gardner syndrome. CONCLUSIONS: Craniopharyngiomas are most often located in the suprasellar region. Presentation in sites outside the parasellar region is rare. Among these ectopic sites, the cerebellopontine angle appears to be the most common location.

Large benign de novo conjunctival hemangioma in an 11-year-old boy: case report and literature review.

Benign hemangiomas are rare vascular tumors of the conjunctiva that typically present clinically in the first few weeks of life and resolve spontaneously. De novo presentation later in childhood has not been well documented. We present the unusual case of an 11-year-old boy with a rapidly growing benign de novo conjunctival hemangioma that was treated with surgical excision.

Histologic Evidence for Arteriovenous Malformation-Like Vasculature Occurring within an Intracerebral Schwannoma: A Case Report and Review of the Literature.

BACKGROUND: The phenomenon of intracerebral schwannoma is exceedingly rare, and its etiology still a matter of debate. No documented cases of intracerebral schwannoma containing vascular elements consistent with those of an arteriovenous malformation (AVM) have been reported. We describe such a case here. CASE DESCRIPTION: A left temporal intraparenchymal lesion was discovered incidentally in a 34-year-old man after he suffered a mild trauma. The lesion was resected and found on histologic examination to be an intracerebral schwannoma with AVM-like vasculature. The patient made a full recovery after resection. CONCLUSIONS: To our knowledge, this is the first case of an intracerebral schwannoma with AVM-like characteristics to be reported in the literature. We hypothesize that the co-occurrence of this rare pathologic entity is caused by an interrelated etiologic process, with the tumor microenvironment of the schwannoma inciting the development of the vascular malformation.

A Conceptual Overview of Axonopathy in Infants and Children with Allegedly Inflicted Head Trauma.

Fatal, allegedly inflicted pediatric head trauma remains a controversial topic in forensic pathology. Recommendations for systematic neuropathologic evaluation of the brains of supposedly injured infants and children usually include the assessment of long white matter tracts in search of axonopathy - specifically, diffuse axonal injury. The ability to recognize, document, and interpret injuries to axons has significant academic and medicolegal implications. For example, more than two decades of inconsistent nosology have resulted in confusion about the definition of diffuse axonal injury between various medical disciplines including radiology, neurosurgery, pediatrics, neuropathology, and forensic pathology. Furthermore, in the pediatric setting, acceptance that "pure" shaking can cause axonal shearing in infants and young children is not widespread. Additionally, controversy abounds whether or not axonal trauma can be identified within regions of white matter ischemia - a debate with very significant implications. Immunohistochemistry is often used not only to document axonal injury, but also to estimate the time since injury. As a result, the estimated post-injury interval may then be used by law enforcement officers and prosecutors to narrow "exclusive opportunity" and thus, identify potential suspects. Fundamental to these highly complicated and controversial topics is a philosophical understanding of the diffuse axonal injury spectrum disorders.

Clinicopathologic findings in (anti-FcepsilonR1alpha) autoimmune-related chronic urticaria.

BACKGROUND: One cause of chronic urticaria is autoreactivity which is diagnosed by detecting autoantibodies against the IgE receptor alpha subunit (anti-Fc R1alpha). OBJECTIVE: To compare the histopathologic features of chronic urticaria patients testing positive for anti-IgE receptor antibody (Ab) to those testing negative. METHODS: Totally, 438 patients with a clinical presentation of chronic urticaria (2011-2013) had anti-IgE receptor Ab tested and 37 of those patients had skin biopsy. We evaluated microscopic features including: spongiosis, dermal edema, presence of mast cells, density of lymphocytic infiltration, predomination of eosinophils/neutrophils; intravascular neutrophils and presence of vasculitis. The aforementioned features were compared between negative and positive anti-IgE receptor Ab groups. RESULTS: Of 37 patients , 69% were women and 31% were men. 49% had positive anti-IgE receptor Ab and 51% had negative anti-IgE receptor Ab. In the positive anti-IgE receptor Ab group, 83% showed intravascular neutrophils. Eosinophil predominance was identified in 72% and neutrophil predominance was identified in 28%. In the negative anti-IgE receptor Ab group, 89% showed intravascular neutrophils. Eosinophil predominance was identified in 53% and neutrophil predominance was identified in 47%. There was no evidence of vasculitis in either group. CONCLUSION: There were no significant histopathologic differences between the anti-IgE receptor Ab positive and negative cases. Therefore, serum testing for anti-IgE receptor Ab is required to identify this subgroup of chronic urticaria patients.