Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 3368  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Articlesmenu-bullet Search Instructions Online Submission Subscribe Etcetera Contact
 
  NAVIGATE Here 
  Search
 
  
 RESOURCE Links
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::  Article in PDF (1,101 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  References
 ::  Article Figures

 Article Access Statistics
    Viewed484    
    Printed13    
    Emailed0    
    PDF Downloaded9    
    Comments [Add]    

Recommend this journal


 


 
  Table of Contents     
CASE SNIPPET
Year : 2022  |  Volume : 68  |  Issue : 3  |  Page : 184-185

Cutaneous nodular exophytic angiosarcoma with involvement of tibia


Department of Dermatology, Venereology and Leprosy, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India

Date of Submission23-Nov-2021
Date of Decision18-Mar-2022
Date of Acceptance24-Mar-2022
Date of Web Publication20-Aug-2022

Correspondence Address:
D Manoharan
Department of Dermatology, Venereology and Leprosy, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.jpgm_1092_21

Rights and Permissions




How to cite this article:
Manoharan K, Sivaramakrishnan S, Manoharan D, Reddy D I. Cutaneous nodular exophytic angiosarcoma with involvement of tibia. J Postgrad Med 2022;68:184-5

How to cite this URL:
Manoharan K, Sivaramakrishnan S, Manoharan D, Reddy D I. Cutaneous nodular exophytic angiosarcoma with involvement of tibia. J Postgrad Med [serial online] 2022 [cited 2022 Oct 4];68:184-5. Available from: https://www.jpgmonline.com/text.asp?2022/68/3/184/354324




We report a case of a 65-year-old male who presented with an ulcerated well defined, non-tender, indurated, exophytic lesion over the anterior aspect of the right leg for 5 months with pale granulation tissue, depigmentation of the surrounding skin, and lichenification over the dorsum of the foot [Figure 1]a. There was no associated inguinal lymphadenopathy or any history of exposure to the risk of acquiring sexually transmitted disease. His Human Immunodeficiency Virus 1 and 2 status was non-reactive. On histopathological examination [Figure 2]a, [Figure 2]b, [Figure 2]c, vascular proliferation and slit-like spaces, spindle cell hyperplasia with mild nuclear atypia and hyperchromasia, and few inflammatory cells were seen. On immunohistochemistry [Figure 2]d, tumor cells expressed CD31, CD34, and ERG giving evidence of endothelial cell origin and HHV8 negative ruling out Kaposi sarcoma. Magnetic resonance imaging (MRI) [Figure 1]b and Computed tomography (CT) of the right leg showed polypoid soft tissue in the subcutaneous plane on the anterior aspect of distal 1/3rd of the tibia, irregular, mildly spiculated periosteal reaction along the tibia and fibular margins, and edema seen in the muscular compartment. CT abdomen and High-resolution computed tomography (HRCT) chest were done and it was normal ruling out metastasis to other organs. With these findings, we report this case as cutaneous angiosarcoma affecting lower leg with involvement of tibia. The patient was referred to an oncologist where below-knee amputation was done followed by chemotherapy, postoperatively.
Figure 1: (a) Well defined exophytic lesion over anterior aspect of right leg with pale granulation tissue and surrounding atrophy (red arrow); (b) MRI, right leg: polypoid soft tissue swelling with irregular, mildly spiculated periosteal reaction along tibia and fibular margins (white arrow)

Click here to view
Figure 2: (a) H and E staining, scanner view (4×), epidermal atrophy (black arrow) with proliferation of blood vessels (blue arrow) dissecting through the collagen fibres; (b and c) H and E staining, high power view (40×): proliferation of spindle cells (black arrow) around the blood vessels with mild atypical cells and mixed infiltrate (blue arrow) in upper dermis; (d) immunohistochemistry (400×): tumour cells showing ERG positive (black arrow)

Click here to view


Angiosarcoma is a rare, aggressive malignant tumor of vascular or lymphatic endothelial origin which comprises less than 2% of soft tissue sarcomas. They are clonal proliferations of malignantly transformed cells expressing endothelial differentiation. It can affect various anatomic locations, including the skin, soft tissue, bones, breasts, and liver. Predisposing factors include trauma, lymphedema, irradiation, old age, and obesity.[1]

Cutaneous angiosarcoma is the most common clinical manifestation, accounting for approximately 50% to 60% of all cases, and has mainly three clinical variants namely primary/idiopathic, post-radiation, and angiosarcoma arising in areas of chronic lymphedema. Typical manifestations of angiosarcoma are described as raised purplish-red papules, rosacea-like,[2] or a bruise-like lesions with rapid progression. Angiosarcoma can affect bones most commonly in the 2nd to 7th decade of life and long tubular bones of extremities and axial skeleton are most commonly affected.[3] It presents as a lytic lesion, which is well marginated in low-grade tumors and poorly demarcated in high-grade tumors with soft tissue mass surrounding it and periosteal new bone formation.

Histologically angiosarcoma shows irregular vascular channels, lined with atypical endothelium in a single row or several layers thick. On immunohistochemistry, these are positive for vimentin, CD34, and CD31 tumor markers with CD31 being the most specific and sensitive marker of endothelial cell differentiation and is important in diagnosis.[4]

The overall estimated 5-year survival is 15%, reflecting the fact that the treatment of angiosarcoma[5] is currently not highly effective. Treatment often involves wide excision to achieve negative margins. High-field electron beam therapy is effective in prolonging the survival of patients with localized lesions. Pegylated-doxorubicin and paclitaxel-based chemotherapy regimens have been tried. Tyrosine-kinase inhibitor sorafenib has shown promising results. Poor prognostic factors like ill-defined borders, the extension of lesion, frequency of multifocal disease, and distant metastasis pose challenges in the treatment of angiosarcoma.

Cutaneous angiosarcoma, being an aggressive malignant vascular or lymphatic tumor, has many differentials. The variability in the appearance of lesions of angiosarcoma makes it a challenging case to diagnose. Bony involvement is rare and thus early radiological investigations help in assessing the bone involvement, reducing disabilities, and thereby preventing amputation and metastasis.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 :: References Top

1.
Mathes EF, Frieden IJ. Vascular tumors. In: Weiss SW, Goldblum JR, Folpe AL, editors. Enzinger and Weiss's Soft Tissue Tumors. St. Louis Mo USA: Mosby; 2007.  Back to cited text no. 1
    
2.
Tambe SA, Nayak CS. Metastatic angiosarcoma of lower extremity. Indian Dermatol Online J 2018;9:177-81.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Kudva R, Perveen S, Janardhana A. Primary epithelioid angiosarcoma of bone: A case report with immunohistochemical study. Indian J Pathol Microbiol 2010;53:811-3.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Bajaj S, Sharma PK, Sachdev IS, Bhardhwaj M. A novel presentation of cutaneous angiosarcoma: A case report and review. Indian J Med Paediatr Oncol 2017;38:363-6.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Vora R, Anjaneyan G, Gupta R. Cutaneous angiosarcoma of head and neck. Indian J Dermatol 2014;59:632.  Back to cited text no. 5
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2]



 

Top
Print this article  Email this article
 
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow