Identification of Predictors of Metastatic Potential in Paragangliomas to Develop a Prognostic Score (PSPGL).

Context: Paragangliomas (PGLs) are rare tumors in adrenal and extra-adrenal locations. Metastasis are found in approximately 5% to 35% of PGLs, and there are no reliable predictors of metastatic disease. Objective: This work aimed to develop a prognostic score of metastatic potential in PGLs. Methods: A retrospective analysis was conducted of clinical data from a cohort with PGLs and tumor histological assessment. Patients were divided into metastatic PGL (presence of metastasis) and nonmetastatic PGL (absence of metastasis ≥96 months of follow-up) groups. Univariate and multivariable analysis were performed to identify predictors of metastatic potential. A prognostic score was developed based on coefficients of multivariable analysis. Kaplan-Meier curves were generated to estimate disease-specific survival (DSS). Results: Out of 263 patients, 35 patients had metastatic PGL and 110 patients had nonmetastatic PGL. In multivariable analysis, 4 features were independently related to metastatic disease and composed the Prognostic Score of Paragangliomas (PSPGL): presence of central or confluent necrosis (33 points), more than 3 mitosis/10 high-power field (HPF) (28 points), extension into adipose tissue (20 points), and extra-adrenal location (19 points). A PSPGL of 24 or greater showed similar sensitivity with higher specificity than the Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP). PSPGL less than or equal to 20 was associated with a risk of metastasis of approximately 10%, whereas a PSPGL of 40 or greater was associated with approximately 80%. The presence of metastasis and Ki-67 of 3% or greater were related to lower DSS. Conclusion: The PSPGL, composed of 4 easy-to-assess parameters, demonstrated good performance in predicting metastatic potential and good ability in estimating metastasis risk.

Capgras syndrome associated with limbic encephalitis in a patient with diffuse large B-cell lymphoma / Síndrome de Capgras associada a Encefalite Límbica em um Paciente com Linfoma Difuso de Grandes Células B

We report the case of a patient with insidious onset and slowly progressive cognitive impairment, behavioral symptoms, temporal lobe seizures and delusional thoughts typical of delusional misidentification syndromes. Clinical presentation along with extensive diagnostic work-up revealed limbic encephalitis secondary to diffuse large B-cell lymphoma. The patient underwent immunotherapy with high-dose corticosteroid but no significant improvement was observed. No specific treatment for lymphoma was performed because the patient died of septic shock following a nosocomial respiratory infection. Delusional misidentification syndromes are an unusual and unique form of cognitive impairment in which a patient consistently misidentifies persons, places, objects, or events. Capgras syndrome is the most common subtype of this disorder, being defined by the recurrent and transient belief that someone close has been substituted by an imposter. These entities are generally associated with neurodegenerative diseases and psychiatric disturbances. Rare reports of associations between misidentification syndromes and autoimmune diseases such as multiple sclerosis have been published, but no papers address a correlation with limbic encephalitis or lymphoma.

Nós relatamos o caso de um paciente com início insidioso e lentamente progressivo de declínio cognitivo, sintomas comportamentais, crises convulsivas do lobo temporal e pensamentos delirantes típicos dos delírios monotemáticos de identificação. A apresentação clínica associada aos exames complementares levaram ao diagnóstico de encefalite límbica relacionada a linfoma difuso de grandes células B. O paciente foi submetido a imunoterapia com altas doses de corticoide sem melhora clínica. Não foi realizado tratamento específico para o linfoma em função do paciente ter evoluído para óbito por choque séptico secundário a infecção respiratória nosocomial. Os delírios monotemáticos de identificação representam uma forma única e incomum de comprometimento cognitivo nas quais o paciente consistentemente identifica de forma errônea pessoas, lugares, objetos ou eventos. A síndrome de Capgras é o subtipo mais comum dessas desordens, sendo definida como a crença recorrente e transitória que um impostor substituiu uma pessoa familiar. Essas entidades são geralmente relacionadas com doenças neurodegenerativas e distúrbios psiquiátricos. Raros relatos da associação entre os delírios monotemáticos de identificação e doenças autoimunes como esclerose múltipla foram publicados, porém não há artigos abordando a correlação com encefalite límbica ou linfoma.

Hemangioma de mama simulando metástase no PET-CT / Breast hemangioma mimicking metastasis at PET-CT

Hemangioma de mama é um tumor benigno raro que apresenta pouca ou nenhuma captação de 18F-flúor-2-deoxi-Dglicose (FDG) na tomografia por emissão de pósitrons (PET). Relatamos um nódulo mamário compatível, patologicamente, com hemangioma, em uma mulher cuja PET scan demonstrou captação elevada de FDG (simulando tumor maligno). Também fizemos breve revisão das causas que levam a resultados falso-positivos e falso-negativos pela PET.

Breast hemangioma is a rare benign tumor that presents either absent or low 18F-fluoro-2-deoxy-D-glucose (FDG) uptake at positron emission tomography (PET). The authors report the case of a breast nodule pathologically compatible with hemangioma in a woman whose PET-scan has demonstrated increased FDG uptake (simulating a malignant tumor). A brief review of factors leading to false positive and false negative PET results is also undertaken.