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.

Chordoid meningiomas: incidence and clinicopathological features of a case series over 18 years.

Chordoid meningioma (CM) is a rare subtype of meningioma, classified as grade II, which exhibits a high rate of recurrence following subtotal resection. We retrospectively examined nine cases of chordoid meningioma over a case series of 1743 meningiomas (0.52%) operated upon at our institution from 1995 to 2013. All the reported clinicopathological findings were analyzed. Two hundred and twenty-one CM cases have been published to date worldwide and few single-center large case series have been issued. Seventy-five percent of the cases that underwent subtotal resection at our institution had recurrence within 1 year. Total resection of the tumor should be the major objective of surgery to reduce the possibility of tumor recurrence. The percentage of chordoid features within the tumor specimen could assist in predicting the pathogenesis of the lesion. The correlation of the index of proliferation to recurrence rate is still controversial. Much debate exists with regard to the role of adjuvant radiotherapy in CM cases. Immunohistochemical, cytological and ultrastructural studies should be used in combination to assure a correct diagnosis of CM. Owing to the rare occurrence of this meningioma subtype, larger case series are required to assist in providing a reference for diagnosis and to improve the therapeutic management of CM.

Abnormal neurons in teratomas in NMDAR encephalitis.

IMPORTANCE: Ovarian teratomas are frequently described in patients with N-methyl-d-aspartate receptor (NMDAR) encephalitis, yet NMDAR encephalitis is rarely described in patients with ovarian teratomas. Understanding why a minority of patients with teratomas are seen with autoimmune encephalitis may improve the management of NMDAR encephalitis and other teratoma-associated autoimmune diseases. OBJECTIVE: To characterize the unique organization of neuroglial elements within ovarian teratomas resected from patients with NMDAR encephalitis. DESIGN: Case-control study comparing the pathological features of ovarian teratomas resected from consecutively accrued cases with NMDAR encephalitis between January 1, 2009, and December 15, 2013, and ovarian teratomas resected from controls between June 1, 2012, and June 30, 2013. SETTING: Pathology tissue database at a tertiary academic care center. PARTICIPANTS: Five cases with teratoma-associated NMDAR encephalitis and serum or cerebrospinal fluid autoantibodies against central nervous system (CNS) NMDAR and 38 controls (39 ovarian teratomas) without neurological symptoms or signs. EXPOSURES: Formalin-fixed, paraffin-embedded ovarian teratomas were examined for the presence of CNS tissue and inflammatory infiltrates using direct microscopy, enhanced with standard histopathological and immunological stains. MAIN OUTCOMES AND MEASURES: Frequency of detection of atypical (dysplastic) CNS neuronal elements in ovarian teratomas resected from cases vs controls, as well as characterization of the relationship between atypical neurons and immune infiltrates. RESULTS: Central nervous system neuronal elements were detected in 4 of 5 teratomas resected from cases with NMDAR encephalitis and in 20 of 39 controls (P = .36). Atypical neurons were seen within teratomas resected from 4 of 5 cases but not in 39 controls, reliably distinguishing teratomas associated with NMDAR encephalitis (P < .001). If found within the CNS, these histological abnormalities would have received the diagnosis of gangliogliomas (n = 3) and ganglioneuroblastoma (n = 1). Reactive changes were present in teratomas from controls, including ferruginated neurons and Rosenthal fibers. Abnormal neuroglial elements were closely related to immune infiltrates in teratomas resected from 4 of 4 cases. Inflammatory infiltrates were not associated with neuroglial tissue in 20 controls, further differentiating these populations (P < .001). CONCLUSIONS AND RELEVANCE: Abnormal neurons within teratomas distinguish cases with NMDAR encephalitis from controls and may promote the development of autoimmunity.

Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: an illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt.

Abernethy malformation, an extrahepatic congenital portosystemic shunt, is more often diagnosed based on associated cardiac or pulmonary malformation. Although predominately a pediatric diagnosis, "late diagnoses" in adulthood have been reported especially in type II malformations that involve only a partial shunt of portal circulation directly into the inferior vena cava. Aside from the cardiac-related presentation, Abernethy malformation is also associated with multiple liver nodules, either benign or malignant, and pulmonary hypertension. In this report, we present immunoglobulin A glomerulonephritis with nephrotic syndrome as a hitherto unrecognized manifestation of this malformation outside the pediatric population, in a patient who also had pulmonary hypertension and multiple liver tumors. We also propose a pathogenetic basis for this multisystemic presentation that includes release into the systemic circulation of unfiltered bacteria, vasoactive substances, and immunoglobulin A-antigen complexes.