Abstract

BackgroundIt is often challenging to completely resect multinodular/plexiform schwannomas involving important deep nerves using minimally invasive surgically techniques.Case descriptionA 32-year-old woman presented with a 5-year history of a slowly growing, painful mass in the medial aspect of the right ankle. Magnetic resonance imaging (MRI) demonstrated multiple nodular lesions with iso-signal intensity relative to skeletal muscle on T1-weighted sequences and heterogeneous high signal intensity on T2-weighted sequences. Mild to moderate enhancement was identified after gadolinium administration. All 58 tumors were completely enucleated using an intracapsular technique. Histological examination confirmed the diagnosis of schwannoma consisting mainly of Antoni A areas. The burning sensation was relieved immediately after surgery. The patient had no aggravated neurological deficit and was very satisfied with the outcome of the treatment at final follow-up.Discussion and evaluationWe experienced a very rare case of a large multinodular/plexiform schwannoma arising from the posterior tibial nerve and its larger terminal branch. Our case had the characteristic MRI features of this condition. It is extremely important to differentiate multinodular/plexiform schwannoma from plexiform neurofibroma and malignant peripheral nerve sheath tumor, with complete surgical enucleation being curative.ConclusionsMRI is a clinically useful modality in the evaluation and detection of deep-seated multinodular/plexiform schwannoma. Intracapsular enucleation seems to be an acceptable treatment for this peculiar tumor located in the foot and ankle.

Highlights

  • It is often challenging to completely resect multinodular/plexiform schwannomas involving important deep nerves using minimally invasive surgically techniques.Case description: A 32-year-old woman presented with a 5-year history of a slowly growing, painful mass in the medial aspect of the right ankle

  • We experienced a very rare case of a large multinodular/plexiform schwannoma arising from the posterior tibial nerve and its larger terminal branch

  • Intracapsular enucleation seems to be an acceptable treatment for this peculiar tumor located in the foot and ankle

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Summary

Discussion and evaluation

Multinodular/plexiform schwannoma is a distinctive subtype of schwannoma, accounting for only 5% of all schwannomas. It would be difficult to completely resect the lesions without causing significant neurological deficits in larger multinodular/plexiform schwannomas. Plexiform neurofibroma is one of the more commonly occurring plexiform neurogenic tumors and is essentially pathognomonic of neurofibromatosis type 1 (NF1) It can be present at birth or develop within the first year of life (Korf 1999). Complete resection is typically not possible without the risk of major neurological compromise for the lesions affecting multiple nerve fascicles. Previous studies have indicated that certain MRI features can be helpful for differentiating between these two lesions (Hébert-Blouin et al 2010; Li et al 2014). The imaging features were strongly in favor of multinodular/plexiform schwannoma and the definite diagnosis was confirmed by histological examination. Limitations of this case study are lack of electrophysiological information and short postoperative follow-up

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