Lipomatosis of nerve: a clinicopathologic analysis of 15 cases / 中华病理学杂志
Chinese Journal of Pathology
; (12): 165-168, 2011.
Article
en Zh
| WPRIM
| ID: wpr-261836
Biblioteca responsable:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To study the clinicopathologic features of lipomatosis of nerve (NLS).</p><p><b>METHODS</b>The clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.</p><p><b>RESULTS</b>There were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.</p><p><b>CONCLUSIONS</b>NLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.</p>
Texto completo:
1
Base de datos:
WPRIM
Asunto principal:
Patología
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Neoplasias del Sistema Nervioso Periférico
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Cirugía General
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Vimentina
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Deformidades Congénitas de la Mano
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Imagen por Resonancia Magnética
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Síndrome del Túnel Carpiano
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Proteínas S100
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Estudios Retrospectivos
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Proteínas de Neurofilamentos
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
Límite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
Zh
Revista:
Chinese Journal of Pathology
Año:
2011
Tipo del documento:
Article
Pais de publicación:
CHINA
/
CN
/
REPUBLIC OF CHINA