Cardiac Malignant Peripheral Nerve Sheath Tumor Accessed By 18F-FDG PET/CT.

This is a case report of a patient presenting an extremely rare cardiac malignancy: malignant peripheral nerve sheath tumour. The 18F-fluorodeoxyglucosis positron emission tomography associated to computed tomography (18F-FDG PET/CT) accesses the tumour anatomy and metabolic activity, thereby making it possible to characterize a malignant neoplasm noninvasively. The diagnostic approach with 18F-FDG PET/CT spared the heart from a likely futile invasive procedure when detecting a distant metastasis and changed the biopsy site and therapeutic planning.

[The nerve root syndrome in small animals - A review focussing on pathophysiology and therapy in the dog]. / Das Nervenwurzelsyndrom beim Kleintier.

A large variety of etiologies is considered to be the cause of nerve root syndrome in dogs. Lateralized disc herniation, foraminal stenosis and malignant as well as benign nerve sheath tumors are some of the most important triggers described. The clinical signs of a nerve root syndrome are characterized by monoparesis in combination with progressive lameness, which may be accompanied by an elevation of the affected limb. Although the problem is well known among clinicians, there is no review article in the veterinary literature that specifically covers the subject of "nerve root syndrome in small animals". Mostly, this is merely mentioned as a symptom of its potential etiologies, as the so-called "nerve root sign" or "nerve root signature". In the pathophysiology of nerve root compression or irritation, a number of biomechanical and biochemical factors play a role. These occur individually or in combination and may lead to the same changes. The pathophysiology of the syndrome seems to focus around changes in microcirculation. These microcirculation disorders not only lead to pathomorphological changes such as edema formation, demyelination and axon death, but also initiate a cascade of reactions at the site of damage as well as in the central nervous tissue. This leads to the release of various neuropeptides, modulation of nerve excitability and impulse transmission. Different pathomechanisms therefore often lead to a uniform damage pattern, which makes it difficult to point out the original triggering factors. The body's response to these factors determines whether a nerve root syndrome actually develops or not. The treatment of the cause, if found, and an individual and multimodal pain therapy seem to be the most successful therapeutic approaches for nerve root syndrome in dogs.

Could an osteoinductor result in degeneration of a neurofibroma in NF1?

We report a case of fatal evolution of neurofibromatosis in a young boy. A laminectomy was performed when he was 9 years old. A secondary hyperkyphosis led to many surgeries resulting in recurrent malunions. When he was 23 years old, a breakage of his rods was treated by a new instrumentation and a T12-L1 interbody cage fitted with rh-BMP. Five months later, he developed a huge posterior tumour on his back. The biopsy diagnosed a neurofibrosarcoma. The growth of the tumour was extremely rapid. He died after several months from a septic shock. NF1 is characterised by neurofibromas that have a possibility of malign degeneration and conversion to a sarcoma. However, the chronology, rapidity of evolution and the exceptional volume of the tumour made us wonder whether the BMP had a part of responsibility as osteoinductor in the malignant degeneration, in this particular case, of neurofibromatosis. It seemed important to point out this case to the medical community.

Malignant change in cutaneous neurofibromas--case reports.

Neurofibromatosis is the most common single gene disorder to affect the nervous system, with an estimated incidence of 1 in 3000 live births. Neurofibromatosis (NF) may be classified into von Recklinghausen NF (NFI) and bilateral acoustic NF (NFII) based on the distribution of lesions. The most common lesion associated with the von Recklinghausen type is the neurofibroma. Various complications are associated with neurofibromatosis, the most feared of which is malignant change in the neurofibroma. This article describes the study of 7 cases of proven malignant change in neurofibromas with regards to presentation, clinical progress and treatment followed by a review of the present literature.