Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1087  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 80-81
Paraganglioma-like dermal melanocytic tumor


1 Department of Dermatology, Pariyaram Medical College, Kannur, Kerala, India
2 Department of Pathology, Pariyaram Medical College, Kannur, Kerala, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Bindurani Sudhamani
Raniraj House, Peoples Road, Westhill P O, Kozhikode - 5, Kerala - 673 005
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147804

Rights and Permissions

   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.


Keywords: Dermal melanocytic tumor, paraganglioma, paraganglioma-like dermal melanocytic tumor


How to cite this article:
Thyvalappil A, Sudhamani B, Kizhakkethara G, Sridharan R, Kunjan R. Paraganglioma-like dermal melanocytic tumor. Indian J Dermatol 2015;60:80-1

How to cite this URL:
Thyvalappil A, Sudhamani B, Kizhakkethara G, Sridharan R, Kunjan R. Paraganglioma-like dermal melanocytic tumor. Indian J Dermatol [serial online] 2015 [cited 2020 Feb 24];60:80-1. Available from: http://www.e-ijd.org/text.asp?2015/60/1/80/147804

What was known?
Paraganglioma like dermal melanocytic tumour is a recently described subtype of primary dermal melanocytic tumor of which only 10 cases have been reported so far and to the best of our knowledge none in dermatology journals



   Introduction Top


Paraganglioma-like dermal melanocytic tumor (PDMT) is a clinically and pathologically unique subtype of primary dermal melanocytic tumor. The term was based on their nested growth pattern in histology, which shows nests of clear to amphophilic oval cells separated by delicate fibrous strands. [1],[2] Only 10 cases have been reported so far and to the best of our knowledge none in dermatology journals.


   Case Report Top


A 60-year-old female presented with one year history of three asymptomatic slowly growing lesions on her right lower leg. It started as a single raised lesion near medial aspect of right ankle, which slowly increased in size. Three months later patient developed two more similar slow growing lesions nearby. On examination, patient had three linearly arranged erythematous polypoidal fleshy nodules of size ranging from 1 × 1 to 3 × 2 cm, with the larger lesion showing ulcerations and crusting on the surface [Figure 1]. Lesions were firm and nonindurated. There was no pallor or lymph node enlargement. Systemic examination did not show any abnormality.
Figure 1: Erythematous polypoidal fleshy nodules over the leg (taken after excision biopsy of one lesion)

Click here to view


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.
Figure 2: Subepithelial lesion composed of sheets and nests of cells in zellballen pattern (H and E stain, ×10)

Click here to view
Figure 3: Cells with clear to eosinophilic cytoplasm and vesicular nuclei with prominent nucleoli arranged in nested pattern (H and E stain, ×40)

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

Click here to view



   Discussion Top


The term PDMT was coined by Deyrup et al. in 2004. [1] There are only 10 PDMT cases reported in literature and 8 of them were retrospectively diagnosed in the study by Deyrup et al. while reviewing all dermal melanocytic tumors that did not qualify histologically as a previously described entity. It is a benign dermal lesion found predominantly in females aged 18-35 years. [3] PDMT clinically present as single or multiple nodules usually over the extremities. No local recurrence or nodal metastasis was reported in previously described cases. [3]

The lesion may be confused with other benign dermal tumors such as cellular blue nevus, schwannoma, granular cell tumor or malignant lesions like melanoma and clear cell sarcoma. [2] But histopathological and immunohistochemical analysis helps to clinch the diagnosis. Two important microscopic features were present in all cases described until now: [3] (i) no evidences of melanocytic atypia or epidermal hyperplasia in the overlying epithelium; (ii) presence of a distinctive partitioning of the tumor into small and large packets, nests, or short cords by delicate fibrous septa (zellballen pattern). Tumors express S-100, melan A, HMB 45 and lack pancytokeratin markers. [1],[4] There is no necrosis but increased mitotic activity can be rarely encountered. [2] These histopathologic features are reminiscent of those of paraganglioma but tumor cells of paraganglioma are usually negative for melanocytic markers. Other malignant and potentially malignant dermal tumors, such as melanoma and dermal melanocytic tumor of uncertain potential can be excluded because of the absence of any atypical features, such as nuclear atypia, macronucleoli, increased mitotic activity, and necrosis. [2]

This case has been presented for its rarity and hence to sensitize the dermatology community to this recently described entity that can be confused with other common melanocytic lesions. Since the literature on PDMT is scarce, close follow up of such cases is advised to establish the true nature of this distinct, benign-looking tumor.

 
   References Top

1.
Deyrup AT, Althof P, Zhou M, Morgan M, Solomon AR, Bridge JA, et al. Paraganglioma-like dermal melanocytic tumor: A unique entity distinct from cellular blue nevus, clear cell sarcoma, and cutaneous melanoma. Am J Surg Pathol 2004;28:1579-86.  Back to cited text no. 1
    
2.
Deba PS, Bryan T, Bo W. Paraganglioma-like dermal melanocytic tumor: A case report. Cases J 2008;1:48.  Back to cited text no. 2
    
3.
Anca MC, Raluca C, Marius R. Paraganglioma-like dermal melanocytic tumor: A case report with particular features. Int J Clin Exp Pathol 2010;3:222-5.  Back to cited text no. 3
    
4.
Weedon D. Lentigines, nevi, and melanomas. In: Weedon D, editor. Weedon's skin pathology. 2 nd ed. China: Elsevier Publishing; 2010. p. 731.  Back to cited text no. 4
    

What is new?
To the best of our knowledge this is the first case of PDMT reported from India


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,583 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1956    
    Printed33    
    Emailed0    
    PDF Downloaded54    
    Comments [Add]    

Recommend this journal