RESUMO
Of the primary central nervous system (CNS) lymphomas, diffuse large B-cell lymphoma of the CNS (CNS-DLBCL) is an aggressive extranodal lymphoma that originates in the CNS. Lymphomatosis cerebri (LC) is an exceptionally rare subtype, posing diagnostic challenges due to the absence of abnormal enhancement and making the identification of suitable biopsy sites difficult. Arterial spin-labeling magnetic resonance imaging (ASL-MRI) is a non-invasive MRI technique that quantifies tumor blood flow. This report presents a case of CNS-DLBCL with LC, which was evaluated and biopsied using ASL-MRI of the brain. Herein, we present a case of a 32-year-old female who presented with abnormal involuntary movements and cognitive impairments. She underwent an MRI which showed a diffuse and infiltrative lesion in the bilateral basal ganglia, showing a high signal intensity area on fluid-attenuated inversion recovery (FLAIR) images with no contrast enhancement. Computed Tomography scans and Gallium-67 scintigraphy showed no abnormal uptake throughout the whole body. Although she received corticosteroid treatments, subsequent MRI showed an enlarged lesion, and she underwent a brain biopsy. The biopsy site was determined based on high perfusion demonstrated by ASL-MRI and the histological findings positive for B-cell markers led to diagnoses of CNS-DLBCL, specifically LC. Her symptoms improved following high-dose methotrexate and whole-brain irradiation. Subsequent MRI scans showed a dramatic improvement, and the high perfusion observed in the ASL-MRI disappeared. This report has emphasized the critical role of histopathology in diagnosing CNS-DLBCL presenting with LC, a highly aggressive lymphoma requiring prompt treatment. In this case, high ASL-MRI signal intensity indicated an increased area of tumor cell density suitable for biopsy. This is the first report to establish a relationship between cell density and ASL-MRI signal intensity in LC. The challenge in locating the optimal biopsy site due to the lack of contrast enhancement and the difference in tumor cell densities within high signal intensity areas on FLAIR imaging is presented. ASL-MRI provides information on tumor blood flow (TBF), which may be associated with higher tumor cell density, making it a valuable tool for identifying suitable biopsy sites. Thus, ASL-MRI is clinically beneficial for the biopsy of LC cases that show high signal intensity on FLAIR images without contrast enhancement.
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Aortic sarcomas are extremely rare. Sarcomas associated with aortic graft replacement are even rarer; only 17 cases have been examined through immunohistochemical staining to date, most of which were either angiosarcomas or intimal sarcomas. Here, we report the case of an 88-year-old man with an undifferentiated pleomorphic sarcoma (UPS) that developed after aortic graft replacement and was diagnosed through postmortem autopsy. To the best of our knowledge, this is the first case of graft-associated sarcoma diagnosed as an undifferentiated pleomorphic type following detailed immunohistochemical staining with sufficient antibodies and fluorescencein situ hybridization (FISH).
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Cardiac involvement of eosinophilic granulomatosis with polyangiitis is a rare but life-threatening complication. We present a case of eosinophilic granulomatosis with polyangiitis with moderately impaired ventricular function forming a ventricular thrombus. Pathological assessment of endomyocardial biopsy specimen revealed aggregated eosinophils in the subendocardium, suggesting ventricular endothelial damage leading to thrombus formation.
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The assessment of programmed death-ligand 1 (PD-L1) expression in esophageal squamous cell carcinoma (ESCC) has become increasingly important with the rise of immune checkpoint inhibitors (ICIs). However, challenges persist, including subjective interpretation and the unclear significance of staining intensity, as well as contrasting roles in tumoral and stromal regions. Our study enhances the understanding of PD-L1 in ESCCs by analyzing its expression in tumors and stroma with H-scores, highlighting its distinct clinicopathological impacts. In a retrospective cohort of 194 ESCC specimens from surgical resection, we quantified PD-L1 expression in tumoral and stromal compartments using H-scores, analyzing whole slide images with digital pathology analysis software. Kaplan-Meier analysis demonstrated that higher PD-L1 expression is significantly associated with improved postoperative overall survival (OS) and recurrence-free survival (RFS) in both tumoral and stromal areas. Multivariable analysis identified high tumoral PD-L1 expression as an independent prognostic factor for prolonged OS and RFS (HR = 0.47, p = 0.007; HR = 0.54, p = 0.022, respectively). In a separate analysis, high stromal PD-L1 expression was found to correlate with less advanced pathological stages and a prolonged response to cytotoxic chemotherapy, with no similar correlation found for ICI treatment response. This study reveals PD-L1's contrasting role in the ESCC tumor immune microenvironment, impacting prognosis, tumor stage, and treatment response.
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Nonbacterial thrombotic endocarditis (NBTE) is a manifestation of prothrombotic status observed in patients with malignancy. Most cases are discovered only in the advanced stages. However, cancer in early stages may also induce NBTE development. We herein report an 87-year-old man with NBTE with multiple thromboembolization coexisting with lung cancer in early clinical stage. Autopsy findings revealed platelet- and fibrin-rich vegetations in both the tricuspid and mitral valves without evidence of bacterial infection. NBTE should be considered in cases with occult thromboembolization. Not only the presence of typical vegetation but irregular leaflet thickening should be monitored with careful echocardiographic examinations.
Assuntos
Endocardite não Infecciosa , Endocardite , Neoplasias Pulmonares , Masculino , Humanos , Idoso de 80 Anos ou mais , Neoplasias Pulmonares/complicações , Endocardite/complicações , Endocardite/diagnóstico , Endocardite não Infecciosa/complicações , Endocardite não Infecciosa/diagnóstico por imagem , Valva Mitral/patologia , AutopsiaRESUMO
Key Clinical Message: Disseminated carcinomatosis of the bone marrow is rare. We present such a case, which is useful for raising awareness about the importance of early diagnosis and treatment of carcinomas complicated by disseminated carcinomatosis of the bone marrow. Abstract: This is the first autopsy report of disseminated carcinomatosis of the bone marrow (DCBM) in esophageal adenocarcinoma. Advanced poorly differentiated adenocarcinoma with signet ring cell carcinoma arising in Barrett's esophagus caused disseminated intravascular coagulation (DIC) with extensive bone marrow metastasis, resulting in death from cerebral hemorrhage. Although DCBM due to malignancy is rare with poor prognosis, it should be considered in malignancies associated with DIC, and prompt initiation of chemotherapy is the only way to improve the patient's prognosis.
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A 56-year-old male subject with bilateral eyelid swelling was diagnosed with an immunoglobulin G4-related disease. After whole-body surveillance, concomitant coronary arteritis with a mural thrombus and myocardial involvement were observed. In this case, multimodal diagnostic imaging assessment led to the diagnosis of both coronary arteritis and myocardial fibrosis associated with immunoglobulin G4-related disease. (Level of Difficulty: Advanced.).
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Distinguishing metastatic lung tumors from primary lung cancer is essential for planning the appropriate treatment strategy. Thyroid transcription factor-1 (TTF-1) is a reliable immunohistochemistry (IHC) marker for differentiating between primary lung adenocarcinomas and metastatic lung tumors originating from colorectal adenocarcinomas. Herein, we report a rare case of TTF-1 expression in both the metastatic lung tumor and primary rectal adenocarcinoma. Aside from the similar histological characteristics of both tumors when stained with hematoxylin-eosin, the IHC patterns, including negative results for alveolar epithelium markers (napsin A and CK7) and positive results for intestinal markers (CK20, CDX2, SATB2, and ß-catenin), of the lung tumor and the primary rectal adenocarcinoma strongly supported the final diagnosis. Considering the non-negligible frequency of TTF-1 positivity in colorectal adenocarcinomas, applying the IHC panel including multiple markers for alveolar epithelium and intestinal differentiation, would be helpful to support the diagnosis of metastatic lung tumor from a rectal adenocarcinoma.