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Life-threatening Lymphatic Malformation With Somatic Activating NRAS Mutation Successfully Treated With Trametinib: A Case Study.
J Pediatr Hematol Oncol
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Kaposiform lymphangiomatosis (KLA) is a rare and aggressive subtype of complex lymphatic anomalies (CLA), characterized by abnormal lymphatic proliferation leading to distinct clinical manifestations. Despite the complexity of this condition, there is no established standard therapy, and treatment options such as sclerotherapy, laser therapy, and surgery remain variably effective and are limited to symptom management rather than curative. Sirolimus, an mTOR pathway inhibitor, has shown promise as a primary therapy, particularly in patients without a driver mutation.
View Article and Find Full Text PDFAchievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
Cureus
November 2024
Hematology and Medical Oncology, Kettering Health, Kettering, USA.
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
View Article and Find Full Text PDFSustained Ventricular Tachycardia as the first presentation of transthyretin amyloid cardiomyopathy.
Rev Fac Cien Med Univ Nac Cordoba
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Instituto Modelo de Cardiología. División Cardiología.
Transthyretin Amyloid Cardiomyopathy (ATTR-CM) was considered an uncommon disease until a few years ago, but advances in the epidemiology and non-invasive diagnostic tests have increased its timely detection. We report a 71 years-old man with history of hypertension and an incidental carcinoma of the left kidney detected 6 years ago, without heart failure who was performed cardiac magnetic resonance images (MRI) by suspicion of hypertrophic cardiomyopathy. Before his cardiologist be aware of the result, he suffered a severe sustained ventricular tachycardia (SVT) that required emergency cardioversion.
View Article and Find Full Text PDFCarotid endarterectomy in the setting of persistent hypoglossal artery.
J Vasc Surg Cases Innov Tech
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Division of Vascular Surgery, Department of Cardiothoracic and Vascular Surgery, McGovern Medical School at UTHealth Houston, Houston, TX.
Persistent hypoglossal artery (PHA) is a rare, anatomical variant in which the posterior cerebral circulation is primarily supplied by a branch of the carotid artery, rather than the vertebral arteries. This case report discusses carotid endarterectomy performed on a man, 67 years of age, with high-grade, asymptomatic carotid artery stenosis and ipsilateral PHA. Preoperative computed tomography angiography identified the PHA arising from the internal carotid artery, compensating for atretic bilateral vertebral arteries and providing primary perfusion to anterior spinal artery.
View Article and Find Full Text PDFHow to Manage a Large Undifferentiated Lung Cancer Mass: A Case Report.
Cureus
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Cardiothoracic Surgery, Hull University Teaching Hospitals NHS Trust, Cottingham, GBR.
Non-small cell lung cancer is the most common type of lung cancer globally. An important subtype to discuss is undifferentiated carcinomas, which are variants of large cell carcinoma, and these can typically present with evidence of neuroendocrine differentiation. The patient presented with a large mass in the right upper lobe extending into the middle lobe.
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