Visceral plexiform schwannoma: A case series.

Area of Science:

• Oncology
• Pediatric Surgery
• Neuropathology

Background:

• Plexiform schwannoma (PS) is a benign peripheral nerve sheath tumor, predominantly cutaneous.
• Visceral PS is exceptionally rare, with fewer than 15 reported cases in pediatric and adult populations.
• Abdominal PS presents a diagnostic challenge due to its rarity and potential for acute symptoms.

Purpose of the Study:

• To report a series of pediatric abdominal plexiform schwannoma cases.
• To describe the clinical presentation, diagnostic imaging, and surgical management of abdominal PS.
• To emphasize the association between abdominal PS and neurofibromatosis, necessitating further screening.

Main Methods:

• Retrospective case series of three pediatric patients diagnosed with abdominal PS.
• Clinical data collection including patient demographics, symptoms, and medical history.
• Review of radiological investigations: abdominal MRI, CT scans, and neurofibromatosis screening (cerebral MRI, dermatological evaluation).
• Surgical intervention and follow-up data analysis.

Main Results:

• Three pediatric patients (2 male, 1 female; age 10-16 years) presented with abdominal PS.
• Common symptoms included acute abdominal pain, with associated rectal bleeding in one and walking difficulties in another.
• Complete surgical resection was achieved in two patients; one underwent laparoscopically assisted surgery.
• Follow-up was uneventful for all patients.

Conclusions:

• Abdominal plexiform schwannoma is an exceedingly rare entity in pediatric patients.
• Acute abdominal pain is a significant presenting symptom, warranting thorough investigation.
• The potential association with neurofibromatosis mandates comprehensive screening for other manifestations of the disorder.