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Pediatric Plexiform Fibromyxoma: A Case Report
Patricia Bugeda Gómez1, Adrià Costa-Roig1, Carolina Montecino Romanini2
1Department of Pediatric Surgery.
Insights
Plexiform fibromyxoma, a rare stomach tumor, was exceptionally found in a 3-month-old infant’s jejunum. Surgical removal was curative, highlighting this tumor
Area of Science:
- Gastroenterology
- Pediatric Oncology
- Surgical Pathology
Background:
- Plexiform fibromyxoma is a rare mesenchymal tumor typically found in adult stomachs.
- The World Health Organization recognized it as a distinct entity in 2010.
- Pediatric cases are exceptionally rare, with limited documentation.
Observation:
- A 3-month-old infant presented with intestinal subocclusion due to a jejunal mass.
- Surgical intervention revealed an infiltrative tumor with spindle-shaped cells in a plexiform myxoid stroma.
- Immunohistochemical analysis confirmed the diagnosis of plexiform fibromyxoma.
Findings:
- The tumor exhibited an infiltrative growth pattern.
- Immunohistochemical markers were consistent with plexiform fibromyxoma.
- Differential diagnosis with other gastrointestinal tumors was discussed.
Implications:
- This case expands the known age range for plexiform fibromyxoma.
- It underscores the importance of considering rare tumors in pediatric intestinal obstructions.
- Surgical resection appears to be a curative treatment for this pediatric case.
Abstract:
The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed.
