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Extensive plexiform neurofibroma in a premature neonate
Aditya Joshi1, Moira Lancelot2, Nandita R Bhattacharjee3
1Department of Pediatrics, Marshfield Clinic/Ministry Saint Joseph's Children Hospital, Marshfield, Wisconsin, USA.
Clinical Medicine & Research
|November 9, 2014
Summary
This study reports an extremely rare case of extensive plexiform neurofibroma in a premature infant. The tumor
Area of Science:
- Pediatric Oncology
- Medical Genetics
- Radiology
Background:
- Plexiform neurofibromas are benign tumors arising from nerve sheath cells.
- While typically associated with neurofibromatosis type 1, extensive presentations in neonates are rare.
Observation:
- A premature neonate presented with a large left neck mass detected postnatally.
- Magnetic resonance imaging (MRI) revealed extensive tumor involvement of the head, neck, chest, abdomen, pelvis, and spinal canal, causing spinal cord compression.
Findings:
- Biopsy confirmed the mass as a plexiform neurofibroma.
- This represents the most extensive neonatal plexiform neurofibroma reported in the literature.
Implications:
- Surgical decompression was not feasible due to the tumor's widespread nature.
- The non-responsiveness of neurofibromas to conventional therapies necessitates exploring alternative treatment strategies for improved outcomes in such severe cases.
