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Infantile myofibromatosis of the central nervous system
G Tamburrini1, M Gessi, C Colosimo
1Pediatric Neurosurgical Unit, Catholic University Medical School, Largo A. Gemelli, 8, 00168 Rome, Italy. gtamburrini@hotmail.com
Background:
Infantile myofibromatosis is the most common fibrous disorder of infancy and childhood. It may occur in two distinct forms: multicentric and solitary. In both cases involvement of the central nervous system (CNS) is unusual: brain myofibromas are usually intracranial in proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression. Spine myofibromas are exceptional and most of the cases reported in the literature represent secondary locations of visceral lesions. The natural history of myofibromas of the CNS in infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases. On these grounds surgical treatment is reserved for lesions that compress the brain or spinal cord and show an obvious progression. Small lesions or tumor residuals in asymptomatic children without visceral involvement should be closely observed as first choice.
Case Report:
We report the case of a 10-month-old child with a solitary intramedullary myofibroma, extended from C7 to the conus. The patient underwent partial surgical removal of this lesion. Control MRI has shown the spontaneous slow reduction of the tumor residual and a progressive improvement of preoperative neurological conditions has been observed. To our knowledge this is the first case in which a solitary myofibroma was localized within the spinal cord.
Insights
Infantile myofibromatosis can affect the central nervous system (CNS). This case report details the first solitary intramedullary spinal cord myofibroma, showing spontaneous regression after partial removal.
Area of Science:
- Pediatric Oncology
- Neuropathology
- Developmental Biology
Background:
- Infantile myofibromatosis is the most common pediatric fibrous tumor, typically presenting as multicentric or solitary lesions.
- Central nervous system (CNS) involvement is rare, usually manifesting as intracranial tumors near the dura mater.
- Spinal cord myofibromas are exceptional, often representing metastatic spread from visceral lesions.
Observation:
- A 10-month-old child presented with a solitary intramedullary myofibroma spanning from C7 to the conus.
- The patient underwent partial surgical resection of the spinal cord lesion.
- Post-operative MRI revealed spontaneous reduction of the residual tumor.
Findings:
- The case represents the first documented instance of a solitary myofibroma originating within the spinal cord.
- Following partial surgical removal, the residual tumor demonstrated slow, spontaneous regression.
- The patient exhibited progressive neurological improvement after the intervention.
Implications:
- This case expands the known spectrum of infantile myofibromatosis presentations.
- It highlights the potential for spontaneous regression of spinal cord myofibromas, even after partial resection.
- Conservative management with close observation may be a viable option for select pediatric spinal cord tumors.