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: 3230  
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 
E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 4  |  Page : 328
Ganglion cell like cells, diagnostic dilemma


1 Department of Pathology, S.N. Medical College, Bagalkot, Karnataka, India
2 Department of Pathology, J.N. Medical College, Belgaum, Karnataka, India
3 Department of Pathology, Basaveshwara Medical College and Hospital, Chitradurga, Karnataka, India

Date of Web Publication25-Jun-2013

Correspondence Address:
Anand Shankar Ammanagi
Department of Pathology, S.N. Medical College, Bagalkot, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.113990

Rights and Permissions

   Abstract 

We report a case of cutaneous swelling found on the left anterior axillary fold of a 41-year-old man. Gross examination of specimen excised from the dermis showed a well-circumscribed nodule histologically composed of spindle cells with interspersed ganglion cell like cells. On hematoxylin and eosine (H and E) staining it was diagnosed as ganglioneuroma. Ganglioneuromas are rare, benign, fully differentiated tumors that contain mature schwann cells, ganglion cells, fibrous tissue, and nerve fibers. They are commonly found along the paravertebral sympathetic ganglia and sometimes in the adrenal medulla. However primary cutaneous ganglioneuroma is an extremely rare tumor. Immunohistochemical workup revealed a fibroblastic origin and hence the case was diagnosed as fibromatosis with ganglion cell like fibroblasts. This case report suggests that the features considered diagnostic of ganglioneuromas can occur in other cutaneous lesions and, therefore, this diagnosis cannot be offered only on the basis of H and E.


Keywords: Fibromatosis, ganglioneuroma, ganglion, skin


How to cite this article:
Ammanagi AS, Chikkannaiah P, Yenni VV, Dombale VD. Ganglion cell like cells, diagnostic dilemma. Indian J Dermatol 2013;58:328

How to cite this URL:
Ammanagi AS, Chikkannaiah P, Yenni VV, Dombale VD. Ganglion cell like cells, diagnostic dilemma. Indian J Dermatol [serial online] 2013 [cited 2019 May 20];58:328. Available from: http://www.e-ijd.org/text.asp?2013/58/4/328/113990

What was known? 1. Fibromatosis is characterized by infiltrative pattern of growth, variable amount of collagen, bland cytological features, scant or absent mitotic activity and aggressive clinical behavior. 2. A well circumscribed tumor composed of spindle cells with numerous large mononuclear rounded basophilic ganglion cells having prominent nuclei and eosinophilic nucleoli suggests possibility of ganglioneuroma.



   Introduction Top


Ganglioneuroma is a peripheral neuroblastic tumor arising mainly from the sympathetic nervous system and is found in the pediatric population. However primary cutaneous ganglioneuroma is an exceedingly rare neoplasm. [1] They are slow-growing, well-differentiated tumors. [2] Immunohistochemical study in our case was in favor of fibroblastic origin of the lesion. Thus the earlier H and E based diagnosis of ganglioneuroma was revised as fibromatosis with ganglion cell like giant cells.


   Case Report Top


A 41 year old man, otherwise healthy, presented with an asymptomatic, painless, solitary, nontender, cutaneous swelling over the left anterior axillary fold [Figure 1]. It was measuring 2 cm in size. There were no local inflammatory changes. The nodule was firm with relatively well defined borders. Regional lymphnodes were not palpable. The patient had no constitutional symptoms. An excision biopsy was performed without any complications. The specimen was sent for histopathologic examination.
Figure 1: (a) Lump in the left anterior axillary fold and (b) showing a relatively well circumscribed mass with a solid grey white glistening appearance on cut surface

Click here to view


Pathological finding

Gross examination revealed a relatively well circumscribed mass measuring 2 × 1.5 × 1.5 cm with a solid grey white glistening appearance on the cut surface [Figure 1]. Microscopy showed a relatively well circumscribed tumor composed of spindle cells arranged in whorls and fascicles with thin elongated nuclei interspersed with few blood vessels lined by plump endothelial cells. There were numerous large ganglion-like mononuclear rounded basophilic cells with prominent nuclei and nucleoli. Compressed fibro-adipose tissue and blood vessels were seen outside the margin of the tumor [Figure 2]. Based on these histologic features a diagnosis of ganglioneuroma was made. However, immunohistochemical studies showed strong positive staining for vimentin and negative for smooth muscle actin, CD34, S100 protein and chromogranin [Figure 2]. Based on these immunohistochemistry finding a final diagnosis of fibromatosis with ganglion cell like cells was offered.
Figure 2: Photomicrographs showing H and E and immunohistochemical studies (a) H and E stain showing spindle cells, few blood vessels and ganglion-like cells (b) negative for smooth muscle actin (c) negative for S100 protein (d) negative for CD34 (e) Strong positive staining for vimentin (f) negative for chromogranin

Click here to view



   Discussion Top


Ganglioneuroma is an uncommon benign, peripheral neuroblastic tumor arising mainly from developing neuronal cells of the sympathetic nervous system and is found in childhood or early adult life. [2] The most commonly involved sites are the posterior mediastinum, retroperitoneum, adrenal gland and head and neck soft tissue. [3] On rare occasions they may be encountered in the gastrointestinal tract as initially documented in 1928. [4] However primary cutaneous ganglioneuroma is a very rare neoplasm first described by Collins et al. in 1972. These tumors are usually solitary, asymptomatic and noninvasive. Our case differs from the other cases reported in terms of location and age. [2],[3] Very uncommon site and age group of our case prompted us to undertake detailed immunohistochemical study. A fibroblastic origin of the lesion was confirmed by these studies. A negative staining for smooth muscle actin, CD34, S100 protein and chromogranin ruled out the smooth muscle, vascular, neural and neuroendocrine origin. Thus the H and E based ganglioneuroma diagnosis, was withdrawn and the final diagnosis based on the histochemical study was offered as fibromatosis with ganglion cell like giant cells. These ganglion cell like cells are also seen in myofibroblastoma, inflammatory myofibroblastic tumor of soft tissue, ischemic fasciitis of soft tissue, myxoinflammatory fibroblastic sarcoma of soft tissue, nodular fasciitis of soft tissue, proliferative fasciitis, proliferative myositis of soft tissue and some of the benign fibrous lesions of the umbilicus. [5],[6],[7]] However these lesions differ from fibromatosis in clinical presentation and microscopic appearance.

Stout described Fibromatosis, as a broad group of benign fibrous tissue proliferations of similar microscopic appearance whose biologic behavior is intermediate between that of benign fibrous lesions and fibrosarcoma [8] These lesions are characterized by presence of well differentiated fibroblasts, infiltrative pattern of growth, variable amount of collagen, bland cytological features, scant or absent mitotic activity and aggressive clinical behavior. These lesions never metastasize. In our case the lesion was relatively well circumscribed and showed ganglion like cells. To the best of our knowledge fibromatosis with ganglion like cells are not reported in the literature.


   Conclusion Top


This case report suggests that the features considered diagnostic of ganglioneuromas can occur in other cutaneous lesions and, therefore, this diagnosis cannot be offered only on the basis of H and E and a thorough immunohistochemistry workup is necessary to diagnose such lesions.

 
   References Top

1.Wallace CA, Hallman JR, Sangueza OP. Primary cutaneous ganglioneuroma: A report of two cases and literature review. Am J Dermatopathol 2003;25:239-42.  Back to cited text no. 1
    
2.Ponce-Camacho MA, Diaz de LR, Miranda-Maldonado I, Garza-Guajardo R, Hernandez-Salazar J, Barboza-Quintana O. A 5-year-old girl with a congenital ganglioneuroma diagnosed by fine needle aspiration biopsy: A case report. Cytojournal 2008;5:5.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Hanna SJ, Muneer A, Cohill SB, Miller MA. Ganglioneuromas in the adult scrotum. J R Soc Med 2005;98:63-4.  Back to cited text no. 3
    
4.Beer TW. Solitary ganglioneuroma of the rectum: Report of two cases. J Clin Pathol 1992;45:353-5.  Back to cited text no. 4
    
5.Kohli K, Kawatra V, Khurana N, Jain S. Multicentric synchronous recurrent aggressive fibromatosis. J Cytol 2012;29:57-9.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Iyer VK. Cytology of soft tissue tumors: Benign soft tissue tumors including reactive, nonneoplastic lesions. J Cytol 2008;25:81-6.  Back to cited text no. 6
  Medknow Journal  
7.Vargas SO. Fibrous umbilical polyp: A distinct fasciitis-like proliferation of early childhood with a marked male predominance. Am J Surg Pathol 2001;25:1438-42.  Back to cited text no. 7
    
8.Stout AP. Juvenile fibromatoses. Cancer 1954;7:953-78.  Back to cited text no. 8
    

What is new? Well circumscription and ganglion.like cells will neither exclude the possibility of fibromatosis nor confirm the lesion as ganglioneuroma.


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (865 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed1714    
    Printed33    
    Emailed0    
    PDF Downloaded36    
    Comments [Add]    

Recommend this journal