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ACase Report of Malignant Peripheral Nerve Sheath Tumour (Mpnst)
3
of the tumour in the visceral organs or nerve palsy
could occur if biopsies were attempted (Wasa et al.,
2010). Although there are certain features on MRI
which could be useful in differentiating MPNST from
its benign counterpart such as schwannoma or
neurofibroma, they are often unreliable (Geller and
Gebhardt, 2006). Radiological Gross histopatholgy
and immunostaining is still the mainstay of diagnosis.
It is also essential prior to the surgery (Geller and
Gebhardt, 2006).
The mainstay of treatment is surgical resection (Katz
et al., 2009). There are two surgical approaches; limb
sparing surgery (en bloc or radical resection) or limb
amputation (Nthumba and Juma, 2011). Limb sparing
surgery is favoured over limb amputation. The main
aim is to achieve clear margins of resection and to
prevent systemic spread with adjuvant chemotherapy
(Lin et al., 2007). However, residual prognosis of the
limb can be a devastating consequence of limb sparing
surgery (Dorsi et al., 2011). Reconstruction of the
nerves is not recommended as it does not restore the
full functionality of the limb (Dorsi et al., 2011). In
some cases, amputation of the limb is inevitable if the
tumour has progressed around the vital structures
(Dorsi et al., 2011).
Chemotherapy has low sensitivity to the sarcoma.
Radiotherapy on another hand has shown to control
local recurrences (Karr et al., 2006; Dimou et al.,
2009). However, it has no effect on long term survival.
It was suggested that radiotherapy should commence
as early as possible for intermediate to high grade
tumour or low grade tumour after marginal excision
(Lin et al., 2007).
3 Conclusion
Extraspinal causes of sciatica alone are extremely rare
and it is often underappreciated even though in some
cases the disease might prove fatal to the patient. The
patient in the case study has a delayed diagnosis for
15 months. Even though surgery has achieved a
complete resection with clear margins of resection, the
patient developed a secondary metastasis in the lung
months after the surgery despite her receiving ongoing
chemotherapy. The clinical diagnosis of sciatic
MPNST remains difficult especially if the clinical
presentation suggest of other common diseases. An
extraspinal cause should take into considering in the
management of sciatica pain and footdrop. A late
detection of malignant tumour could prove to have
poor prognosis.
References
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