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Neurotized congenital melanocytic nevus resembling a pigmented neurofibroma.

Singh N, Chandrashekar L, Kar R, Sylvia MT, Thappa DM - Indian J Dermatol (2015 Jan-Feb)

Bottom Line: Neurotized congenital melanocytic nevus and pigmented neurofibroma (PNF) are close mimics and pose a clinicopathological challenge.We present a case of pigmented hypertrichotic plaque over lumbosacral region and discuss the differential diagnosis and its clinical, histopathological and immunohistochemistry features which may aid in differentiation.We highlight the difficulties faced in differentiating neurotized congenital melanocytic nevus from pigmented neurofibroma.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

ABSTRACT
Neurotized congenital melanocytic nevus and pigmented neurofibroma (PNF) are close mimics and pose a clinicopathological challenge. We present a case of pigmented hypertrichotic plaque over lumbosacral region and discuss the differential diagnosis and its clinical, histopathological and immunohistochemistry features which may aid in differentiation. We highlight the difficulties faced in differentiating neurotized congenital melanocytic nevus from pigmented neurofibroma.

No MeSH data available.


Related in: MedlinePlus

Neural differentiation in the spindle cell component with presence of oval structures, resembling tactile bodies (inset) (H and E, ×100)
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Figure 3: Neural differentiation in the spindle cell component with presence of oval structures, resembling tactile bodies (inset) (H and E, ×100)

Mentions: A 55-year-old male presented with a solitary growth over lumbosacral area present since birth and associated with radiating pain over lower back onto bilateral posterior thighs (right > left) for past 6 months. There was no family history of similar skin disorder. Cutaneous examination revealed a single, band like, nontender, hyperpigmented, verrucous soft plaque of size 7 × 5 cm over lumbosacral area in the midline [Figure 1]. Surface of the plaque showed hypertrichosis and a few soft nodules with size ranging from 0.5 × 0.5 cm to 2 × 2.5 cm. There was no bag of worms feel on palpation. There was a single café-au-lait macule of size 3 × 1 cm present to the left side of the plaque and a satellite nevus on the right side. Clinical examination revealed no other abnormality. Lisch nodules in iris were not seen on slit lamp examination. A skin biopsy was taken from the plaque and nodule and sent for histopathological examination. Magnetic resonance imaging study of pelvis and lumbosacral region did not reveal any abnormal findings. Histopathology of the skin biopsy specimen revealed an unremarkable epidermis with a zone of uninvolved dermis and a lesion extending mostly from mid-dermis up to the subcutaneous tissue. The upper portion of the lesion showed large nests of nevus cells in mid-dermis [Figure 2], some containing melanin pigment and an occasional small nest of nevus cells in upper dermis without any junctional activity. There was progressive maturation of these nests of cells into spindle-shaped cells with wavy nuclei resembling a neurofibroma in the lower part of the lesion extending from the dermis to subcutis. Interspersed and merging imperceptibly within these spindled cells were small nerve twigs, some of which had oval and lamellated appearance similar to tactile bodies [Figure 3] indicating neural differentiation. On immunohistochemistry, S100 [Figures 4 and 5] and HMB 45 [Figure 6] stained the nests of nevus cells, spindled cells and the neural component; Melan A [Figure 7] stained the nevus cells as well as the spindled cells but not the neural component; neuron-specific enolase [Figure 8] and CD57 [Figure 9] stained only the neural component; CD 34 highlighted the blood vessels and stromal cells only [Figure 10] and glial fibrillary acid protein (GFAP) was negative. Thus, we arrived at the diagnosis of congenital melanocytic nevus with extensive neurotization resembling a pigmented neurofibroma


Neurotized congenital melanocytic nevus resembling a pigmented neurofibroma.

Singh N, Chandrashekar L, Kar R, Sylvia MT, Thappa DM - Indian J Dermatol (2015 Jan-Feb)

Neural differentiation in the spindle cell component with presence of oval structures, resembling tactile bodies (inset) (H and E, ×100)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318062&req=5

Figure 3: Neural differentiation in the spindle cell component with presence of oval structures, resembling tactile bodies (inset) (H and E, ×100)
Mentions: A 55-year-old male presented with a solitary growth over lumbosacral area present since birth and associated with radiating pain over lower back onto bilateral posterior thighs (right > left) for past 6 months. There was no family history of similar skin disorder. Cutaneous examination revealed a single, band like, nontender, hyperpigmented, verrucous soft plaque of size 7 × 5 cm over lumbosacral area in the midline [Figure 1]. Surface of the plaque showed hypertrichosis and a few soft nodules with size ranging from 0.5 × 0.5 cm to 2 × 2.5 cm. There was no bag of worms feel on palpation. There was a single café-au-lait macule of size 3 × 1 cm present to the left side of the plaque and a satellite nevus on the right side. Clinical examination revealed no other abnormality. Lisch nodules in iris were not seen on slit lamp examination. A skin biopsy was taken from the plaque and nodule and sent for histopathological examination. Magnetic resonance imaging study of pelvis and lumbosacral region did not reveal any abnormal findings. Histopathology of the skin biopsy specimen revealed an unremarkable epidermis with a zone of uninvolved dermis and a lesion extending mostly from mid-dermis up to the subcutaneous tissue. The upper portion of the lesion showed large nests of nevus cells in mid-dermis [Figure 2], some containing melanin pigment and an occasional small nest of nevus cells in upper dermis without any junctional activity. There was progressive maturation of these nests of cells into spindle-shaped cells with wavy nuclei resembling a neurofibroma in the lower part of the lesion extending from the dermis to subcutis. Interspersed and merging imperceptibly within these spindled cells were small nerve twigs, some of which had oval and lamellated appearance similar to tactile bodies [Figure 3] indicating neural differentiation. On immunohistochemistry, S100 [Figures 4 and 5] and HMB 45 [Figure 6] stained the nests of nevus cells, spindled cells and the neural component; Melan A [Figure 7] stained the nevus cells as well as the spindled cells but not the neural component; neuron-specific enolase [Figure 8] and CD57 [Figure 9] stained only the neural component; CD 34 highlighted the blood vessels and stromal cells only [Figure 10] and glial fibrillary acid protein (GFAP) was negative. Thus, we arrived at the diagnosis of congenital melanocytic nevus with extensive neurotization resembling a pigmented neurofibroma

Bottom Line: Neurotized congenital melanocytic nevus and pigmented neurofibroma (PNF) are close mimics and pose a clinicopathological challenge.We present a case of pigmented hypertrichotic plaque over lumbosacral region and discuss the differential diagnosis and its clinical, histopathological and immunohistochemistry features which may aid in differentiation.We highlight the difficulties faced in differentiating neurotized congenital melanocytic nevus from pigmented neurofibroma.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

ABSTRACT
Neurotized congenital melanocytic nevus and pigmented neurofibroma (PNF) are close mimics and pose a clinicopathological challenge. We present a case of pigmented hypertrichotic plaque over lumbosacral region and discuss the differential diagnosis and its clinical, histopathological and immunohistochemistry features which may aid in differentiation. We highlight the difficulties faced in differentiating neurotized congenital melanocytic nevus from pigmented neurofibroma.

No MeSH data available.


Related in: MedlinePlus