A clinical pathologic review with analysis of prognostic factors was conducted in 56 patients who were seen at the University of Texas M. D. Anderson Hospital and Tumor Institute between 1969 and 1980 with the syndrome of superior vena cava (SVC) obstruction secondary to small cell bronchogenic carcinoma. Most patients were men (60%), nonambulatory (61%), and had demonstrable extrathoracic disease (70%). The most common symptoms and signs of SVC obstruction were jugular venous distention (100%), swollen face (88%), and dyspnea (50%). Swollen arms (34%) and engorgement of thoracic veins (32%) were also common. Initial treatment consisted of irradiation alone, 17 patients (30%); chemotherapy alone, 32 patients (57%); or both, 7 patients (13%). Patients receiving chemotherapy initially had poor prognoses, as evidenced by the greater proportion of nonambulatory patients (72%) in this group. All but two patients received chemotherapy at some point during their clinical courses. There were 12 (21%) early deaths; 2 (12%) in the radiation arm; 9 (28%) in the chemotherapy arm; and 1 (14%) in the combined modality group. All treatment modalities were rapidly effective in controlling the symptoms associated with SVC obstruction. Within 1 week from onset of treatment, 9 of 14 (64%) evaluable patients responded to irradiation, 23 of 23 (100%) to chemotherapy, and 5 of 6 (83%) to combined modality treatment. The type of initial treatment did not influence survival significantly, although patients who achieved complete clinical remissions survived longer (median, 62 weeks). Death was usually due to disease progression in distant sites. Multivariate analysis indicated that the most important prognosticators of patients' survival were pretreatment performance status, disease-extent score, and age.
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http://dx.doi.org/10.1002/1097-0142(19831201)52:11<2165::aid-cncr2820521132>3.0.co;2-z | DOI Listing |
Eur Heart J Case Rep
January 2025
Department of Interventional Cardiology, Hospital Nacional Edgardo Rebagliati Martins, EsSalud, 490, Jesús María, 15072 Lima, Perú.
Background: Pulmonary hypertension caused by extrinsic pulmonary vascular compression secondary to mediastinal neuroendocrine tumours is a very rare condition, posing a diagnostic challenge. There is no clear consensus regarding the best treatment strategy due to the lack of clinical data, leading to poor prognoses for these patients.
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J Pak Med Assoc
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Department of Nuclear Medicine, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore.
"Hot quadrate lobe sign" refers to visualization of caudate lobe of liver due to excess accumulation of radiotracer secondary to superior vena cava obstruction. Collateral channels are formed between thoracic and mediastinal vessels; internal mammary through the paraumbilical vessels which drain blood to the left portal vein and into the caudate lobe of liver. It was first described on Tc99-m sulfur colloid scan.
View Article and Find Full Text PDFJACC Case Rep
January 2025
Division of Cardiac Surgery, Department of Surgery, Johns Hopkins Hospital, Baltimore, Maryland, USA.
Fibrosing mediastinitis is a rare chronic inflammatory condition characterized by excessive fibrotic process in the mediastinum. Although various infectious processes are more common causes, it can also be related to several malignancies. We report a case of a 28-year-old woman with fibrosing mediastinitis related to an aggressive primary gray-zone lymphoma causing complete occlusion of the superior vena cava (SVC) and the innominate veins.
View Article and Find Full Text PDFIntern Emerg Med
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Department of Clinical and Experimental Medicine, Internal Medicine Unit, University of Catania, University Hospital G.Rodolico, Catania, Italy.
JNCI Cancer Spectr
January 2025
Section on Medical Neuroendocrinology National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, 20892, MD, USA.
Head and neck paragangliomas (HNPGLs) are typically slow-growing, hormonally inactive tumors of parasympathetic paraganglia. Inactivation of prolyl-hydroxylase domain-containing 2 protein causing indirect gain-of-function of hypoxia-inducible factor-2α (HIF-2α), encoded by EPAS1, was recently shown to cause carotid body hyperplasia. We previously described a syndrome with multiple sympathetic paragangliomas caused by direct gain-of-function variants in EPAS1 (Pacak-Zhuang syndrome, PZS) and developed a corresponding mouse model.
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