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Two infant Paciniomas, distinct from neurofibromas, were surgically removed due to spinal deformities. These rare tumors, possibly malformations, were found connected to the spinal dura via sacral dimples and spina bifida.
Area of Science:
- Neuropathology
- Developmental biology
- Pediatric surgery
Background:
- Paciniomas are rare tumors originating from Pacinian corpuscles.
- Spinal deformities in infants can have various underlying causes.
- Understanding the nature of congenital spinal lesions is crucial for diagnosis and treatment.
Observation:
- Two cases of well-differentiated Paciniomas were resected from infants presenting with spinal deformities.
- The tumors appeared as thick cords extending from a sacral dimple through an occult spina bifida to the spinal dura.
- Histological examination of control sacrococcygeal regions from 15 infants revealed Pacinian corpuscles in 7, typically near the coccyx and not numerous.
Findings:
- The resected Paciniomas showed no resemblance to neurofibromas.
- The lesions were characterized as either a malformation or a hamartomatous overgrowth.
- Pacinian corpuscles are present in the sacrococcygeal region of infants, but typically sparse.
Implications:
- These findings suggest Paciniomas can present as congenital spinal malformations.
- Further research is needed to elucidate the developmental origin of Paciniomas.
- This study contributes to the differential diagnosis of pediatric spinal tumors and congenital anomalies.
Abstract:
Two well differentiated pure Paciniomas, bearing no resemblance to neurofibromas, were resected from infants with spinal deformities. Each was in the form of a thick cord running from a sacral dimple through a low occult spina bifida to the spinal dura. The lesions are considered to be a malformation or hamartomatous overgrowth. Control sacrococcygeal regions from necropsies on 15 infants were examined histologically, and Pacinian corpuscles were found in 7, usually near the tip of the coccyx, and never numerous.