Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

Abstract

Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 revealed 333 nerve sheath tumors, of which 31 were MPNSTs. Four representative case reports are presented. Discussion. MNPSTs have rarely been reported from sub-Saharan Africa; in this study, they constituted 9.3% of all nerve sheath tumors. The trunk (42%) and limbs (45%) were the most frequently affected anatomical sites. Late presentation of malignant lesions in this environment is exemplified by the four case presentations patients. Conclusions. This report confirms observations from studies on MPNSTs from other environments. Anatomically centrally located MPNSTs may have a higher incidence in sub-Saharan Africa than in the West. Because NF1-associated MPNSTs are difficult to diagnose clinically, and because surgery is the only mode of therapy that offers a complete cure, a lifetime follow-up is important, as this would enable diagnosis of early lesions amenable to surgical extirpation.

A Case Report of Malignant Peripheral Nerve Sheath Tumour (Mpnst) Which Present as An Acute Traumatic Sciatic Neuropathy

“…In patients with neurofibromatosis, the disease will manifest earlier by at least one decade (Geller and Gebhardt, 2006). The mean age for the presentation of the disease in sporadic cases is 39.7 as compared to those with neurofibromatosis with mean age of 28.7 (Karr et al, 2006;Nthumba and Juma, 2011). MPNST can also occur in children with NF-1 mutation (Dimou et al, 2009;Geller and Gebhardt, 2006).…”

“…The common site of presentation is usually at the large nerve trunk in the peroneal nerve, sciatica nerve, radial plexus and sacral plexus (Nthumba and Juma, 2011;Geller and Gebhardt, 2006). In patients with neurofibromatosis, it usually develops from deep seated plexiform neurofibroma.…”

“…In patients with neurofibromatosis, it usually develops from deep seated plexiform neurofibroma. It presents with symptoms of nerve compression such as paraesthesia, radicular pain and weakness (Nthumba and Juma, 2011;Rodero et al, 2004). Hence, clinically it is very difficult to differentiate sciatica caused by spinal pathology from MPNST at the proximal sciatic nerve.…”

“…It is often underappreciated as a cause of peripheral nerve compression, especially the involvement of sciatic nerve where spinal pathology and traumatic sciatic neuropathy supersede the diagnosis. In the current published literatures, the first presentation of MPNST is usually a mass which follows nerve symptoms such as pain, paraesthesia and limb weakness (Nthumba and Juma, 2011). However, we are reporting an interesting case of MPNST at the proximal sciatic nerve which concealed as a traumatic cause of sciatic neuropathy and foot drop, where a subtle mass was only detected later.…”

“…Malignant Peripheral Nerve Sheath Tumour (MPNST) is an extremely rare medical condition (Karr et al, 2006;Nthumba and Juma, 2011). It is often underappreciated as a cause of peripheral nerve compression, especially the involvement of sciatic nerve where spinal pathology and traumatic sciatic neuropathy supersede the diagnosis.…”