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Pakistani teenager left bedridden by 44lbs tumour

Plexiform Neurofibromas are, in most cases, benign tumours of the peripheral nerves, also called a peripheral nerve sheath tumour (PNST).

They are caused by genetic mutations.

The tumour may arise from the nerves underneath the skin surface, or from nerves deep within the body. They can occur anywhere in the body and grow to fairly large sizes.

The frequent sites include the head and neck region, arms, legs, chest, and abdomen.

Study reports indicate that Plexiform Neurofibroma is unique to those with neurofibromatosis type 1 (NF-1). Neurofibromas generally begin to appear during teenage and young adulthood (age range 15-20 years). But, they may be seen in individuals of any age group.

A treatment of Plexiform Neurofibroma may be undertaken, if they present cosmetic concern to the individual or if the symptoms are severe. In such cases, a surgical excision is sufficient treatment

In most cases, the prognosis is excellent on removal of Plexiform Neurofibroma, since it is a benign tumour in a majority. However, some tumours are known to undergo malignant transformation, in which case the prognosis may be guarded

A diagnosis of Plexiform Neurofibroma may involve a complete physical examination with imaging studies, such as X-ray, CT, or MRI scans of the affected region.

In about 5-10 per cent of cases the tumours may undergo malignant transformation. In such cases, when it becomes malignant, it is called a malignant peripheral nerve sheath tumour (MPNST).

These malignant transformations may be indicated by a sudden growth in size of tumor, the overlying skin gets ulcerated or pain.

In general, the prognosis of Plexiform Neurofibroma is excellent on its complete excision and removal, as long as they are benign.

If the tumour becomes malignant, then the 5-year survival rate is about 16 per cent in individuals with NF-1 (syndromic Plexiform Neurofibroma) and 53 per cent in those without NF-1 (non-syndromic Plexiform Neurofibroma).

Source: DoveMed



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