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: 5802  
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 
QUIZ
Year : 2018  |  Volume : 63  |  Issue : 3  |  Page : 275-276
Subcutaneous left cervical nodule in an infant present since birth


Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar Islands, India

Date of Web Publication21-May-2018

Correspondence Address:
Dr. Jitendra Singh Nigam
Department of Pathology, Andaman and Nicobar Islands Institute of Medical Sciences, Port Blair, Andaman and Nicobar Islands
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_109_17

Rights and Permissions



How to cite this article:
Bharti JN, Dey B, Nigam JS, Singh A. Subcutaneous left cervical nodule in an infant present since birth. Indian J Dermatol 2018;63:275-6

How to cite this URL:
Bharti JN, Dey B, Nigam JS, Singh A. Subcutaneous left cervical nodule in an infant present since birth. Indian J Dermatol [serial online] 2018 [cited 2019 Dec 11];63:275-6. Available from: http://www.e-ijd.org/text.asp?2018/63/3/275/232715




A 1-year-old male presented with a painless subcutaneous nodule in the left cervical region since birth [Figure 1]. On physical examination, the nodule was 2 cm in diameter, mobile, and nontender. There was no other lymphadenopathy or any organomegaly. Ultrasonography revealed a hyperechoic soft-tissue mass measuring 2 cm×2 cm×1 cm. A diagnosis of benign spindle cell lesion was made on fine-needle aspiration cytology and excision was advised.
Figure 1: Subcutaneous nodule in the left cervical region

Click here to view


Local excision of the mass was done. Grossly, it was firm to soft with gray-white and gray-yellow areas. The histopathological section examined showed immature round mesenchymal cells, islands of mature adipose tissue, and spindle-shaped fibroblastic cells in a collagenous stroma [Figure 2]. No mitotic figures, necrosis, or atypia was identified. On immunohistochemistry (IHC), vimentin was positive in the spindle cells and in the mesenchymal cells [Figure 3]a. CD34 showed immunoreactivity in the mesenchymal cells [Figure 3]b.
Figure 2: Histopathological section showing immature round mesenchymal cells (Black Arrow), islands of mature adipose tissue (Blue Arrow) and spindle-shaped fibroblastic (Red Arrow) cells in a collagenous stroma. (H and E, ×100)

Click here to view
Figure 3: (a) Vimentin positivity in the spindle cells (x400) and (b) CD 34 positivity in mesenchymal cell (x400)

Click here to view



   Question Top


What is Your Diagnosis?


   Answer Top


Fibrous hamartoma of infancy.


   Discussion Top


Enzinger suggested the term fibrous hamartoma of infancy to emphasize its organoid microscopic appearance and its frequent occurrence at birth and during the immediate postnatal period.[1] It is rare in children >2 years of age and is more common in boys with a male to female ratio of 2:1.[2] The common sites of involvement are the axillary region, upper arm, upper trunk, inguinal region, and external genital areas.[1],[2]

Cytological findings of fibrous hamartoma of infancy include adipose tissue fragments and fibroblastic cells in clusters as well as lying discretely associated with the myxoid and collagenous matrix.[3] Fibrous hamartoma of infancy is histologically characterized by varying proportions of tissues arranged in an organoid pattern comprised of fibrous trabeculae or septae composed of spindle cells separated by collagen, myxoid foci-containing primitive round or stellate mesenchymal cells and interspersed mature fat.[2],[4] Morphologically, it has a varied differential diagnosis. Infantile fibromatosis arises primarily in muscle rather than in the subcutis, locally aggressive containing both myofibroblastic and adipocytic areas, but lacks the primitive mesenchymal cells seen in fibrous hamartoma.[4] Calcifying aponeurotic fibroma occurs in more distal locations such as the palm of older children and shows distinctive spotty calcifications surrounded by radiating fibroblasts and osteoclast-like giant cells.[2] The characteristic organoid pattern helps to distinguish fibrous hamartoma of infancy from rhabdomyosarcoma.[4]

Immunohistochemically, vimentin is reactive in the spindle cells and primitive mesenchymal cells, while S100 immunoreactivity is present only in the mature adipose tissue.[2],[4] CD34 is positive in the mesenchymal cells, whereas smooth muscle actin is positive in the spindle-shaped fibroblastic cells.[2],[4]

Clinically, it is often misdiagnosed as lipoma, dermatofibroma, neurofibroma, and hemangioma or as an enlarged lymph node.[2] Thus, characteristic histomorphology along with immunohistochemistry helps in arriving at a final diagnosis.

Simple excision is curative, and incomplete excision does not seem to carry a significant risk for recurrence.[2],[5] However, 16% of cases recur locally within a few months after primary excision due to incomplete excision but are nondestructive and cured by re-excision.[1],[2],[5]

Learning points

  • Fibrous hamartoma of infancy is a rare benign tumor
  • It frequently occurs at birth and during the immediate postnatal period
  • Histopathologically it is characterized by a triphasic organoid pattern
  • Clinically, it is often misdiagnosed as lipoma, dermatofibroma, neurofibroma, and hemangioma or as enlarged lymph node
  • It has an excellent prognosis with local excision being curative.


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Enzinger FM. Fibrous hamartoma of infancy. Cancer 1965;18:241-8.  Back to cited text no. 1
[PUBMED]    
2.
Yu G, Wang Y, Wang G, Zhang D, Sun Y. Fibrous hamartoma of infancy: A clinical pathological analysis of seventeen cases. Int J Clin Exp Pathol 2015;8:3374-7.  Back to cited text no. 2
[PUBMED]    
3.
Mohanty SK, Dey P. Cytologic diagnosis of fibrous hamartoma of infancy: A case report. Diagn Cytopathol 2003;28:272-3.  Back to cited text no. 3
[PUBMED]    
4.
Sheikh SS, Montgomery E. Fibroblastic lesions. In: Montgomery E, Aaron AD, editors. Clinical Pathology of Soft-Tissue Tumors. New York: Marcel Dekker; 2001. p. 149.  Back to cited text no. 4
    
5.
Carretto E, Dall'Igna P, Alaggio R, Siracusa F, Granata C, Ferrari A, et al . Fibrous hamartoma of infancy: An Italian multi-institutional experience. J Am Acad Dermatol 2006;54:800-3.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,067 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


   Question
   Answer
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed832    
    Printed9    
    Emailed0    
    PDF Downloaded67    
    Comments [Add]    

Recommend this journal