Giant scalp plexiform neurofibroma associated with NF-1.

Herein we report an interesting image of a giant scalp plexiform neurofibroma associated with neurofibromatosis (NF)-1.

A 50-year-old male from a remote village in eastern Nepal, presented to our outpatient clinic with a complaint of a progressive lesion on his posterior scalp since last 4 years. There was no other significant family or any past medical or surgical illnesses. Local examination revealed a huge posterior scalp plexiform neurofibroma dangling down till his hip region (Fig. 1).There were multiple café au lait spots distributed throughout his body (Fig. 2). Computerized tomography of the head did not reveal any intracranial lesions (Fig. 3). Final diagnosis of a giant scalp plexiform neurofirbroma in neurofibromatosis type 1 was eventually made. The lesion was excised followed by primary closure of the wound. The histology report confirmed the benign pattern of the lesion (Fig. 4).

Figure 1:

Clinical image of a giant scalp neurofibroma.

Figure 2:

Presence of multiple cafe au lait spots.

Figure 3:

CT images showing no intracranial extension of the lesion or other associated pathologies.

Figure 4:

Histology revealing benign neurofibroma.

Giant plexiform neurofibroma of a scalp associated with neurofibromatosis type 1 is a very rare entity [1]. The main aim in its management is 2-fold. First is to obtain a multidisciplinary as well as stepwise approach to minimize the blood loss during its excision and the other is to differentiate it from its malignant counterpart—Neurofibrosarcoma [2, 3].