What is plexiform schwannoma?
Plexiform schwannoma is a rare, benign peripheral nerve sheath tumor composed of Schwann cells arranged in a plexiform pattern1,2. Plexiform schwannomas predominantly affect young adults and occur most commonly as a solitary, slow-growing, asymptomatic nodule on the head, neck, trunk, or upper extremities1-3.
What is plexiform pattern?
The strict definition of the term ‘plexiform’ is that. pattern which resembles a plexus, or network, or. assumes the configuration of a ‘bag of worms’.1 Plex- iform growth in pathology is generally associated. with complex multinodular lesions, the plexiform.
What are plexiform lesions?
Plexiform lesions are glomus-like structures that emerge at right angles from a muscular artery and bulge into a plexus of channels whose walls consist of fibrous tissue covered by endothelial cells.
Is plexiform neurofibroma curable?
How are plexiform neurofibromas treated? Until recently, the only known effective treatment for plexiform neurofibromas has been surgery. Approximately 75% of patients who undergo a complete removal of the tumor without causing significant neurologic impairment or dysfunction are cured.
What does a plexiform neurofibroma look like?
These tumors look like lumps under the skin. The skin over the lump may feel thicker and appear darker than the skin around it. The lump itself may feel like a bundle of thick cords or knots. Plexiform neurofibromas have a type of cell that releases histamine, a chemical in the body that can cause itching.
What is plexiform Arteriopathy?
Plexogenic arteriopathy has been a term used to describe a constellation of vascular changes occurring in those with pulmonary arterial hypertension. It is considered the histologic hallmark of idiopathic pulmonary arterial hypertension; it is seen in approximately 75% of cases 3.
Are plexiform neurofibromas painful?
Abstract. Plexiform neurofibromas (PNs) are common and potentially debilitating complications of neurofibromatosis 1 (NF1). These benign nerve-sheath tumors are associated with significant pain and morbidity because they compress vital structures.
How is plexiform neurofibroma diagnosed?
How are plexiform neurofibromas diagnosed? Although diagnosis of these lesions is usually possible by examination of the child, the full extent of the lesion is best seen by MRI scan. MRI scans may show these lesions to be much more extensive than previously thought.
How fast do plexiform neurofibromas grow?
The median rate of growth of the individual tumours expressed as a percentage of the volume measured on the first exam was 2.8% per year, with a range of −35.9% to 3667% per year.
What is pulmonary veno occlusive?
Collapse Section. Pulmonary veno-occlusive disease (PVOD) is characterized by the blockage (occlusion) of the blood vessels that carry oxygen-rich (oxygenated) blood from the lungs to the heart (the pulmonary veins).
What does plexiform neurofibroma look like?
What does a plexiform neurofibroma feel like?
Plexiform neurofibromas often feel like a bunch of cords or knots beneath the skin and may have a variation in texture or darker pigmentation on top of the skin.
Is pulmonary veno-occlusive disease fatal?
Pulmonary veno-occlusive disease (PVOD) is a rare and fatal cause of pulmonary hypertension (PH) that is difficult to diagnose and treat. First described in 1934 by Dr Julius Hora of the University of Munich,1 PVOD is characterized by widespread occlusion of the pulmonary venules by fibrous tissue.