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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Hébert-Blouin, Marie-Noëlle Scheithauer, Bernd W. Amrami, Kimberly K. Durham, Susan R. Spinner, Robert J. |
| Description | Country affiliation: United States Author Affiliation: Hébert-Blouin MN ( Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.) |
| Abstract | OBJECT: Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion in which mature skeletal muscle fibers lie within the nerve and its fascicles. Given limited follow-up, its natural history is poorly understood. The occurrence of aggressive fibromatosis in one of the authors' patients and its occurrence in reported cases suggests an etiological relationship between the 2 lesions. This study attempts to explain the association and its frequency. METHODS: All cases of NMCs seen in consultation or treated at the Mayo Clinic were identified. Demographic and clinical data were reviewed in cases with coexistent aggressive fibromatosis. Pathology and neuroimaging studies were reexamined. In addition, an extensive literature review was performed to explore the association of NMC with aggressive fibromatosis, with special attention given to pathological and imaging characteristics and the development of aggressive fibromatosis. RESULTS: The authors identified 10 patients with a diagnosis of NMC who were treated at the Mayo Clinic between 1992 and 2010. Four of 5 with adequate follow-up had developed a definite or suspected aggressive fibromatosis. A review of the initial pathological specimens in these cases revealed no evidence of fibromatosis, but all of the lesions exhibited accompanying hypocellular collagenous tissue. On MR images, all cases showed areas of low signal intensity, which significantly differed from muscle, nerve, and NMC components. On available serial MR imaging studies, aggressive fibromatosis seemed to originate in such lower-intensity regions. In the 18 previously reported cases of NMC, 5 patients developed recurrent masses diagnosed as either definite (2 cases) or possible (3 cases) fibromatosis. Review of the published imaging studies in these cases suggests the presence of lower intensity areas similar to those observed in the 10 patients treated at the Mayo Clinic. CONCLUSIONS: This study confirms that the development of aggressive fibromatosis in patients with NMC has been underreported. A direct relationship between the NMC and the development of aggressive fibromatosis is suggested by pathological and neuroimaging evidence. |
| File Format | HTM / HTML |
| ISSN | 00223085 |
| e-ISSN | 19330693 |
| Journal | Journal of Neurosurgery |
| Issue Number | 2 |
| Volume Number | 116 |
| Language | English |
| Publisher | American Association of Neurological Surgeons |
| Publisher Date | 2012-02-01 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Discipline Neurosurgery Choristoma Complications Fibroma Etiology Muscle, Skeletal Peripheral Nervous System Diseases Peripheral Nervous System Neoplasms Adolescent Child, Preschool Pathology Databases, Factual Magnetic Resonance Imaging Retrospective Studies |
| Content Type | Text |
| Resource Type | Article |
| Subject | Neurology (clinical) Surgery |
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