Primary unilateral conjunctival small lymphocytic lymphoma in a child: Case report and literature review.

Ocular adnexal lymphomas (OALs) are extranodal lymphomas primarily involving the ocular adnexa, which includes the conjunctiva. OALs are considered primary if the involvement of lymphoma is confined to the ocular adnexa alone and secondary if there is lymphoma of the identical type present at another site. Conjunctival lymphomas are uncommon in children, with very few reported cases in published literature, none of which were small lymphocytic lymphomas (SLLs). We report a case of SLL confined to the conjunctiva, in a 15-year-old child, with full pediatric oncology examination, including full body positron emission tomography (PET) scan, revealing no other site involvement. To our knowledge, this is the first case to be reported of a child with primary unilateral conjunctival SLL.

Symptomatic Brain Radiation Necrosis in an Anaplastic Lymphoma Kinase (ALK)-Positive Non-small Cell Lung Cancer (NSCLC) Patient After Fractionated Stereotactic Radiotherapy While on Alectinib.

Anaplastic lymphoma kinase (ALK)-positive non-small cell lung cancer (NSCLC) has a higher incidence of brain metastasis. Despite having a favorable prognosis and relatively long survival with second-generation ALK tyrosine kinase inhibitors (TKI), patients can have substantial morbidity, negatively affecting functional progression-free and symptom-free survival. Studies have shown that ALK-rearranged NSCLC is a risk factor for developing radiation necrosis (RN). Recently, second-generation TKI, especially lorlatinib, alectinib, and brigatinib, have demonstrated good central nervous system (CNS) penetration and overall response rates in patients with brain metastasis. However, to improve overall outcomes in symptomatic or limited brain metastases, stereotactic radiosurgery (SRS) is increasingly preferred over whole brain radiotherapy (WBRT) prior to systemic therapy to avoid significant cognitive deterioration. To improve the therapeutic ratio, fractionated stereotactic radiotherapy (FSRT) has been explored for brain metastasis. Herein, we report on one ALK-rearranged NSCLC patient who developed RN despite FSRT, one year after the completion of radiotherapy while on alectinib.

Fonsecaea associated cerebral phaeohyphomycosis in a post-COVID-19 patient: A first case report.

Phaeohyphomycosis, previously known as chromoblastomycosis, is a chronic mycosis, usually affecting the skin. It is caused by dematiaceous fungi, which are a group of fungi that produce melanin in their cell walls. Cerebral phaeohyphomycosis occurs as a part of invasive presentation of the fungi, which usually affects immunocompromised patients, but may affect immunocompetent individuals as well. Cerebral infection in phaeohyphomycosis is associated with a poor prognosis regardless of the immune status of the patient. COVID-19 SARS-CoV-2 infection and/or medications used for its treatment may compromise the immune system, including in the post-COVID-19 period, resulting in invasive fungal infections, which have frequently been reported recently during the COVID-19 pandemic. We report a case of Fonsecaea associated cerebral phaeohyphomycosis in a recently diagnosed diabetic Omani lady, who presented to our hospital 6 weeks after recovery and discharge from hospitalization for moderate COVID-19 pneumonia.

High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication-a comprehensive clinical, radiographic, pathologic, and genomic analysis.

High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication (HGNET BCOR ex15 ITD) is a recently proposed tumor entity of the central nervous system (CNS) with a distinct methylation profile and characteristic genetic alteration. The complete spectrum of histologic features, accompanying genetic alterations, clinical outcomes, and optimal treatment for this new tumor entity are largely unknown. Here, we performed a comprehensive assessment of 10 new cases of HGNET BCOR ex15 ITD. The tumors mostly occurred in young children and were located in the cerebral or cerebellar hemispheres. On imaging all tumors were large, well-circumscribed, heterogeneous masses with variable enhancement and reduced diffusion. They were histologically characterized by predominantly solid growth, glioma-like fibrillarity, perivascular pseudorosettes, and palisading necrosis, but absence of microvascular proliferation. They demonstrated sparse to absent GFAP expression, no synaptophysin expression, variable OLIG2 and NeuN positivity, and diffuse strong BCOR nuclear positivity. While BCOR exon 15 internal tandem duplication was the solitary pathogenic alteration identified in six cases, four cases contained additional alterations including CDKN2A/B homozygous deletion, TERT amplification or promoter hotspot mutation, and damaging mutations in TP53, BCORL1, EP300, SMARCA2 and STAG2. While the limited clinical follow-up in prior reports had indicated a uniformly dismal prognosis for this tumor entity, this cohort includes multiple long-term survivors. Our study further supports inclusion of HGNET BCOR ex15 ITD as a distinct CNS tumor entity and expands the known clinicopathologic, radiographic, and genetic features.

A Cerebellar High-Grade Neuroepithelial Tumour with BCOR Alteration in a five-year-old Child: A case report.

New groups of high-grade neuroepithelial tumours (HGNET) have emerged from the reclassification of central nervous system (CNS) embryonal tumours that have recognised CNS HGNET with BCOR alteration (CNS HGNET-BCOR). We report a two-year, nine-month-old Omani boy who presented to the Royal Hospital, Muscat, Oman, in 2015 with subacute head tilting and neck pain. A well-defined cerebellar lesion was found and he was treated with standard chemoradiotherapy. After a relapse at the age of five years, molecular testing revealed a BCOR alteration. He was treated with further surgery and high-dose chemotherapy; unfortunately, he relapsed and died three years after he was diagnosed.

Brain damage in a large cohort of solvent abusers.

The neuropathology of solvent inhalation consists of patchy myelin loss with white matter macrophages that contain granular inclusions. It has been described only in a small number of cases. We sought to characterize the abnormalities in greater detail. In a retrospective study from 1995 to 2009, we encountered 88 autopsy cases with documented history of solvent abuse by inhalation and 1 with industrial exposure. Among these are 6 fetuses and infants with maternal exposure, 23 children (12-17 years), and 60 adults (18-66 years). Available brain samples from 75 cases were stained with solochrome cyanein (to demonstrate myelin) and periodic acid-Schiff (PAS) (to highlight the inclusions). Forty brains of ethanol and/or illicit drug exposed individuals and ten cases of multiple sclerosis were examined as controls. We found that 16 cases (age 23-49, median 37 years) had well-established leukoencephalopathy with multifocal myelin loss and abundant macrophages that stain with PAS and which contain birefringent inclusions. Six cases (age 15-55, median 27 years) had early leukoencephalopathy with scattered macrophages but no obvious myelin changes. Clusters of PAS-staining but non-birefringent macrophages were seen in 2/10 cases of (active) multiple sclerosis and in none of the ethanol/drug exposed brains. Ultrastructurally, inclusions from solvent cases differed from multiple sclerosis cases. Although exposure to solvents is impossible to quantify, there appears to be a duration-dependent effect. Brain damage related to solvent abuse can begin within only a few years of the onset. In the context of substance abuse, the changes are relatively specific for solvent inhalation and do not appear to result from demyelination alone. Interaction with ethanol cannot be excluded as a compounding risk factor.