Visceral plexiform schwannoma: A case series

Abstract

Plexiform schwannoma (PS) is a benign tumour of the peripheral nerve sheath that is typically found in the skin. Fewer than 15 cases of visceral PS have been reported to date in both adults and children. We herein discuss a series of 3 patients (2 male and 1 female) with abdominal PS, aged 10-16 years (mean age, 12 years). All the patients had an acute presentation with abdominal pain, which was associated with rectal bleeding in 1 case and with walking difficulties in 1 case. Radiological investigations included abdominal magnetic resonance imaging (MRI) and computed tomography, along with neurofibromatosis screening (cerebral MRI and dermatological evaluation). Complete removal of the mass was possible in 2 of the patients (in 1 case by laparoscopically assisted surgery). Follow-up was uneventful. Abdominal PS is a rare occurrence. Due to its possible association with neurofibromatosis, the diagnosis of PS should prompt an investigation for other manifestations of this disorder.

A Rare Case of Plexiform Schwannoma of the Little Finger and Its Management: A Case Report

“…Plexiform neurofibroma is a hallmark clinical finding of neurofibromatosis and carries a potential risk of malignant transformation [ 11 ]. The histological presentation of plexiform schwannomas shows predominantly Antony A tissue consisting of compact and spindle-shaped Schwann cells in a palisading pattern or loose hypocellular area, which is the Antony B pattern and is positive for S100 expression [ 12 ]. In addition, sometimes MRI scans are often used to strengthen the diagnosis.…”

An Overview of Central Nervous System Tumours

“…PSs most commonly occur sporadically as solitary lesions, but have a greater incidence in patients with neurofibromatosis type 2 [NF2] and schwannomatosis [194]. They account for approximately 5% of schwannomas, with more than 23% occurring in the head and neck region, with another 15% occurring cutaneously with predilection for the head, neck, and flexor surface of the upper and lower limbs [195][196]. Clinically, the patients are usually asymptomatic, however, pain may occur due to compression of nearby nerves.…”