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Paraganglioma-like dermal melanocytic tumor.

Thyvalappil A, Sudhamani B, Kizhakkethara G, Sridharan R, Kunjan R - Indian J Dermatol (2015 Jan-Feb)

Bottom Line: It has a benign course, although a lesion of low malignant potential cannot be excluded.We describe a case of 60-year-old female who presented with three PDMT lesions on her right leg.To the best of our knowledge this is the first case reported from India.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Pariyaram Medical College, Kannur, Kerala, India.

ABSTRACT
Paraganglioma-like dermal melanocytic tumor (PDMT) is a rare subtype of benign dermal melanocytic tumor, first described in 2004. Its histopathologic features resemble those of paraganglioma, showing presence of a distinctive partitioning of the tumor into small and large packets, nests, or short cords by delicate fibrous septa (zellballen pattern). But the immunostaining characteristics are those of melanocytic lesions, as PDMT express S-100, melan A, HMB 45 and lack pancytokeratin markers. It has a benign course, although a lesion of low malignant potential cannot be excluded. We describe a case of 60-year-old female who presented with three PDMT lesions on her right leg. To the best of our knowledge this is the first case reported from India.

No MeSH data available.


Related in: MedlinePlus

Immunohistochemistry showing (a) Positive S 100 (×10) and (b) Positive Melan-A (×40)
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Figure 4: Immunohistochemistry showing (a) Positive S 100 (×10) and (b) Positive Melan-A (×40)

Mentions: A differential diagnosis of sporotrichosis, chromoblastomycosis, B cell lymphoma and squamous cell carcinoma were considered and patient was investigated. Routine blood investigations, chest X-ray and ultrasound abdomen were within normal limits. Excision biopsy of a nodule was taken and sent for fungal culture and histopathological examination. Fungal culture showed no growth. Histopathology showed a subepithelial lesion extending up to subcutis composed of sheets and nests of cells in zellballen pattern [Figure 2] suggestive of paraganglioma. Cells had clear to eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli [Figure 3]. No epidermal involvement or pagetoid spread was seen. Atypia, increased mitotic activity or necrosis were absent, thus ruling out malignant tumors-like melanoma. Immunohistochemistry staining showed densely positive S100 and melan-A and faintly positive cytokeratin [Figure 4] confirming the diagnosis of PDMT.


Paraganglioma-like dermal melanocytic tumor.

Thyvalappil A, Sudhamani B, Kizhakkethara G, Sridharan R, Kunjan R - Indian J Dermatol (2015 Jan-Feb)

Immunohistochemistry showing (a) Positive S 100 (×10) and (b) Positive Melan-A (×40)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318069&req=5

Figure 4: Immunohistochemistry showing (a) Positive S 100 (×10) and (b) Positive Melan-A (×40)
Mentions: A differential diagnosis of sporotrichosis, chromoblastomycosis, B cell lymphoma and squamous cell carcinoma were considered and patient was investigated. Routine blood investigations, chest X-ray and ultrasound abdomen were within normal limits. Excision biopsy of a nodule was taken and sent for fungal culture and histopathological examination. Fungal culture showed no growth. Histopathology showed a subepithelial lesion extending up to subcutis composed of sheets and nests of cells in zellballen pattern [Figure 2] suggestive of paraganglioma. Cells had clear to eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli [Figure 3]. No epidermal involvement or pagetoid spread was seen. Atypia, increased mitotic activity or necrosis were absent, thus ruling out malignant tumors-like melanoma. Immunohistochemistry staining showed densely positive S100 and melan-A and faintly positive cytokeratin [Figure 4] confirming the diagnosis of PDMT.

Bottom Line: It has a benign course, although a lesion of low malignant potential cannot be excluded.We describe a case of 60-year-old female who presented with three PDMT lesions on her right leg.To the best of our knowledge this is the first case reported from India.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Pariyaram Medical College, Kannur, Kerala, India.

ABSTRACT
Paraganglioma-like dermal melanocytic tumor (PDMT) is a rare subtype of benign dermal melanocytic tumor, first described in 2004. Its histopathologic features resemble those of paraganglioma, showing presence of a distinctive partitioning of the tumor into small and large packets, nests, or short cords by delicate fibrous septa (zellballen pattern). But the immunostaining characteristics are those of melanocytic lesions, as PDMT express S-100, melan A, HMB 45 and lack pancytokeratin markers. It has a benign course, although a lesion of low malignant potential cannot be excluded. We describe a case of 60-year-old female who presented with three PDMT lesions on her right leg. To the best of our knowledge this is the first case reported from India.

No MeSH data available.


Related in: MedlinePlus