March 15, 2009
Fibrosing mediastinitis
I've talked about it for several posts now. Time for a definition and description:
Straight from RadioGraphics/ Radiology journal (Radiographics. 2001;21:737-757):
"An uncommon benign disorder characterized by proliferation of dense fibrous tissue within the mediastinum. This entity is also known as sclerosing mediastinitis and as mediastinal fibrosis. Affected patients are typically young and present with signs and symptoms related to obstruction of vital mediastinal structures, such as central systemic veins, the esophagus, airways, and pulmonary arteries or veins. The precise cause and pathogenesis of fibrosing mediastinitis in most cases is unknown, and links to infectious and noninfectious causes remain speculative. Recently, Flieder and colleagues (2) proposed the term idiopathic fibroinflammatory lesion of the mediastinum to replace the present term fibrosing mediastinitis. In doing so, they emphasized that, in most cases, a definite cause cannot be established with certainty and that there is substantial variability in the histopathologic appearance of the lesions. Whether this proposed term will eventually replace the present term is unclear."
My favorite word in the definition is "benign," which just means it isn't cancerous, but the word makes FM sound so harmless. In explaining what I have to other people, I am always a bit reluctant to say "benign." When I do explain that FM is not cancer, there is almost visible relief.
As if the words running through their minds "Ah benign. Not cancer: Cancer is the most dangerous and deadliest disease, not too bad then. She'll be ok. She won't die."
"No, no," I feel like saying. "This is horrible. You see, there's NO CURE. I have tissue growing around my trachea and arteries and veins strangling them..There are no protocols to follow, or any major clinical trials going on… anywhere. My doctor, a really really good oncologist, even wished I had Hodgkin's Lymphoma instead of this."
Not to say that cancer is an easy alternative. Cancer can be horrible, and the treatment is worse. But some forms, especially the above mentioned Hodgkin's has a superior remission rate, to the point of being called cured (80 to 90 for stage 1,2a in the first 5 years).
I almost feel like I have to apologize for not having something more easily explained.
Filed under: