From Macroscopic to Microscopic Insights. A “bag of worms” type in Neurofibromatosis.
Author: Carlos Francisco Vargas Lopez
Base Hospital / Institution: Terrassa Hospital
ePoster presentation
Abstract ID: 24-162
Purpose
To demonstrate the correlation between macroscopic and microscopic perspectives in orbital/periorbital plexiform neurofibroma, characterized by a ‘bag of worms’ appearance.
Methods
Our patient was followed up at Pediatric Center located in Barcelona. A 6-year-old patient exhibited a plexiform neurofibroma in the right upper eyelid. Surgery, anatomopathological testing, and immunohistochemistry were conducted.
Results
Debulking surgery was performed, during which we immediately noted the characteristic ‘bag of worms’ appearance upon commencement. Subsequently, anatomopathological testing was conducted. The histopathological analysis conducted in this study unveils the presence of neoplastic cells displaying an elongated morphology, enveloped within a myxoid stroma. Notably, these cells are organized in a distinctive plexiform architecture, delineating a unique histological pattern characteristic of the examined tumor. Furthermore, an immunohistochemical investigation was conducted, elucidating positive immunoreactivity for S100 protein within the neoplastic cells. This notable finding contributes crucial insights into the molecular profile and histogenesis of the observed tumor, enriching its diagnostic and pathological characterization.
Conclusion
This case offers a comprehensive exploration into the clinical, macroscopic, and microscopic dimensions of orbital/periorbital plexiform neurofibroma, elucidating their interrelations. The distinctive ‘bag of worms’ appearance noted during debulking surgery emerges as a diagnostic hallmark in neurofibromatosis. Histopathological findings unveiled neoplastic cells organized in a plexiform pattern surrounded by a myxoid stroma, consistent with neurofibromatosis. Furthermore, immunohistochemical assessment enhanced the diagnosis by revealing positive S100 protein staining within the neoplastic cells. These revelations deepen our understanding of the molecular and histological underpinnings of neurofibromatosis, thereby facilitating its accurate diagnosis and pathological delineation.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Hugo | Gonzales Valdivia | Terrassa Hospital |
| Ester | Casas Jimeno | Terrassa Hospital |
| Joan | Prat Bartomeu | Terrassa Hospital |
