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Figure 1: Lateral aspect of the tumor Figure 3: Dissection of the tumor showing limitation to the epidermis

Figure 4: Final aspect of the mass after removal with over 5 kg total
weight

and plexiform. Cutaneous lesions and subcutaneous lesions
are circumscribed, however none of them have compatible
features with NF-1. [4]

During physical examination, neurofibromas may present a
pathognomonic buttonhole invagination when pressed with
a finger. However, their morphology is not always consistent,
resulting in lesions either soft or firm to the touch. Plexiform
neurofibromas are noncircumscribed, thick and irregular. This
specific subtype for NF-1 can be associated with aesthetic
deformation by entwining important supportive structures. [5]
Figure 2: Posterior view of the tumor demonstrating much narrower
base that allowed direct excision One of the main physical signs encountered along with
neurofibromatosis diagnosis is the presence of café au lait
discharged 48 h later after an uneventful postoperative spots. These rather unfamiliar pigmentary patterns are brown
recovery. Histopathological analysis confirmed diagnosis of macules irregularly shaped and evenly pigmented. Usually,
benign neurofibroma. when neurofibromatosis is diagnosed, subjects have 6 or
more spots that are 1.5 cm or greater in diameter. However,
DISCUSSION in young children, 5 or more café au lait macules greater than
0.5 cm in diameter are indicative of neurofibromatosis and
Neurofibromas are tumors composed of schwann cells, for which investigation is required. Less than 1% of healthy
fibroblasts, mast cells, and vascular components. They can children have 3 or more of such spots, although 1 or 2
be found in nerves, independently of the location in its café au lait macules are commonly encountered in healthy
surface. Moreover, many authors consider neurofibromas the individuals without disease. Due to the large volume of the
commonest benign tumors of the NF-1. Neurofibromas can mass presented in this case, it was difficult to determine if
[3]
be presented in three subtypes: cutaneous, subcutaneous, the alterations on the skin colour were café au lait spots or
280 Plast Aesthet Res || Volume 3 || August 12, 2016