Reconstruction of the chest wall after excision of a giant malignant peripheral nerve sheath tumor

Abstract

Primary chest wall tumors are uncommon and constitute 0.2-2% of all tumors. Metastatic tumors and tumors of local extension are more common. Malignant peripheral nerve sheath tumor (MPNST) of the chest wall is even rarer and its incidence on the chest wall not stated in the literature. The incidence in the general population is 0.0001% while the
risk is approximately 4600 times higher in patients with type I  neurofibromatosis and 3-13% of them will finally develop into MPNST, usually after latent periods of 10-20 years. Clinically, these tumors are aggressive, locally invasive, and highly metastatic. Excision of giant chest wall tumor leaves a defect that is reconstructed using musculocutaneous flaps with or without a mesh. We report the case of a 24-year-old man who presented at the surgical outpatient clinic with 7 months history of persistent left sided chest pain minimally relieved by analgesics, 5 months of cough and worsening dyspnoea, and 3 months history of anterior chest swelling on the left side of the manubrium. Following evaluation and investigations, the tumor was excised and the residual defect closed with methylmetacrylate sandwiched between two prolene meshes and overlaid with both pectoralis major muscles. The histology of the excised mass revealed MPNST He made an uneventful postoperative recovery, but died barely 3 months later from widespread pulmonary metastases.
A review of the literature revealed that such tumors hardly ever reach such large-size as in our case.