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Multiple hairy pacinian neurofibromas (nerve-sheath myxomas)
K McCormack1, D Kaplan, J C Murray
1Department of Pathology, Duke University Medical Center, Durham, NC 27710.
Journal of the American Academy of Dermatology
|February 1, 1988
Summary
Pacinian neurofibromas are rare tumors resembling Vater-Pacini corpuscles. Unlike previous cases, this patient
Area of Science:
- Dermatopathology
- Oncology
- Skeletal Radiology
Background:
- Pacinian neurofibromas, a potential variant of nerve-sheath myxomas, are characterized by structures resembling Vater-Pacini corpuscles.
- Previous reports documented solitary, congenital pacinian neurofibromas on the buttocks, with some cases associated with skeletal anomalies.
Observation:
- A patient presented with multiple, hairy pacinian neurofibromas on the buttocks.
- Radiographic evaluation revealed no evidence of underlying skeletal anomalies in this patient.
Findings:
- The observed multiple pacinian neurofibromas (nerve-sheath myxomas) differed from previously reported solitary lesions.
- The absence of skeletal anomalies in this case contrasts with findings in some prior reports of similar tumors.
Implications:
- This case suggests that multiple pacinian neurofibromas may occur without associated skeletal abnormalities.
- Distinguishing between true pacinian neurofibromas and other entities like 'sacrococcygeal paciniomas' is crucial for accurate diagnosis and prognosis.
- Further research is needed to clarify the spectrum and associated conditions of pacinian neurofibromas.