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Cutaneous lymphangiosarcoma following chronic lymphedema of filarial origin.
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MedLine Citation:
PMID:  23372218     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
We are reporting a case of 36 yr old female with chronic filarial lymphedema of both legs. Subsequently she developed lymphangiosarcoma of her left leg which was confirmed histologically and on immunohistochemistry. Only three more cases of lymphagiosarcoma occurring in patients with filarial lymphedema are reviewed from available literature.
Authors:
Abhijit S Acharya; Kr Sulhyan; Rv Ramteke; Vy Kunghadkar
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Indian journal of dermatology     Volume:  58     ISSN:  1998-3611     ISO Abbreviation:  Indian J Dermatol     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-02-01     Completed Date:  2013-02-04     Revised Date:  2013-05-30    
Medline Journal Info:
Nlm Unique ID:  0370750     Medline TA:  Indian J Dermatol     Country:  India    
Other Details:
Languages:  eng     Pagination:  68-70     Citation Subset:  -    
Affiliation:
Department of Pathology, 2 Floor, RCSM Government Medical College and CPR Hospital, Kolhapur, India.
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Full Text
Journal Information
Journal ID (nlm-ta): Indian J Dermatol
Journal ID (iso-abbrev): Indian J Dermatol
Journal ID (publisher-id): IJD
ISSN: 0019-5154
ISSN: 1998-3611
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
Copyright: © Indian Journal of Dermatology
open-access:
Received Month: 1 Year: 2011
Accepted Month: 7 Year: 2012
Print publication date: Season: Jan-Feb Year: 2013
Volume: 58 Issue: 1
First Page: 68 Last Page: 70
PubMed Id: 23372218
ID: 3555379
Publisher Id: IJD-58-68
DOI: 10.4103/0019-5154.105314

Cutaneous Lymphangiosarcoma Following Chronic Lymphedema of Filarial Origin
Abhijit S Acharyaaff1
KR Sulhyanaff1
RV Ramtekeaff1
VY Kunghadkaraff1
From the Department of Pathology, 2nd Floor, RCSM Government Medical College and CPR Hospital, Kolhapur, India
Correspondence: Address for correspondence: Dr. Abhijit Acharya, Department of Pathology, 2nd Floor, RCSM Government Medical College and CPR Hospital, Kolhapur, India. E-mail: drabhijitacharya@gmail.com

Introduction

What was known?

Till date to our knowledge and available literature only three cases of lymphangiosarcoma arising in post filarial lymphedema have been reported.

Cutaneous angiosarcoma is an uncommon aggressive tumor of the capillary and lymphatic endothelial cell origin. Post mastectomy chronic lymphedema is the most frequent setting in which lymphedema related lymphangiosarcoma develops.[1] Though this tumor has also been described in traumatic, post surgical and congenital lymphedema, it has been encountered in patients with filarial lymphedema very rarely.

We are reporting a case of lymphangiosarcoma developing in a 36 year old female who had a past history of filariasis since 20 years.


Case Report

A 36 year old female patient had bilateral lymphedema following filarial infection since 20 yrs. She came with a complaint of fungating mass on left leg. To start with it was reddish nodule which progressed into a fungating mass within a span of 4 months.

On physical examination patient had extensive skin thickening and an fungating soft tissue mass measuring 10 cm × 7 cm in the medial aspect of the left leg [Figure 1].

A skin biopsy was performed which showed a tumor composed of numerous pleomorphic spindle cells lining the anastomozing blood vessels [Figure 2]. Focal areas of dystrophic calcification in the form of calcosphere were seen consistent with old filarial infection. On immunohistochemistry, tumor cells were positive for CD34, CD31, vimentin, and negative for Cytokeratin (CK) which confirmed our diagnosis of angiosarcoma.

Patient undergone below knee amputation without any adjuvant therapy. The follow up of the patient till date show no evidence of recurrence or metastasis.


Discussion

Angiosarcoma can develop from the endothelium of lymphatics (lymphangiosarcoma) or blood vessels (hemangiosarcoma). This subclassification is often very difficult to make, however, since there apparently is no difference in the clinical activity.[2]

Stewart and Terres in 1948 were the first to describe a cases of lymphangiosarcoma arising in a case of post mastectomy chronic lymphedema.[3] In a review of lymphangiosarcoma arising in a setting of chronic lymphedema in 1972.[4] infectious causes for the tumor was implicated in only two cases of which one was tuberculosis and the other unknown [Table 1].

In our case, patient gave history of chronic filarial infection in past with subsequent development of bilateral lymphedema 20 year back. There was no history of leg or groin trauma, surgery, radiation or lymphadenopathy before development of lymphedema.

We found few foci of dystrophic calcospheres spread within the lesion which represents mineralized degenerated worms[5] Although we did not identify the viable parasite, the patient's history and tissue diagnosis is suggestive of chronic filarial infection.

Filariasis which is endemic in our country often leads to lymphedema. In a review of large cases of filarial lymphedema in 1966, El Toraei[6] did not identify a single case of lymphangiosaroma associated with filaria.

Devi and Bahuleyan (1977)[1] were the first to report a case of lymphangiosarcoma of the lower extremity associated with chronic lymphedema of filarial origin.

They attributed chronic lymph stasis as the underlying mechanism for this neoplasm.

Till date to our knowledge and available literature only three cases of lymphangiosarcoma arising in post filarial lymphedema have been reported.

In all the cases, the lower limb was affected and lymphangiosarcoma developed after lymphedema of many years duration. The tumors were in the form of large cutaneous and soft tissue masses.

The exact pathogenesis of angiosarcoma in these cases is uncertain. It has been shown in animal models that the environment produced by longstanding lymphedema is conducive to atypical vascular proliferation, with mutations eventually leading to malignant transformation of the endothelial cells. Alternatively, the lymphedema may impair local immunity, preventing immune detection and destruction of the malignant-tumor cells.[2]

The natural history of angiosarcoma associated with chronic lymphedema is repeated recurrence and eventual metastasis, usually to the lungs. The clinical outcome is poor, with less than 10 percent of the patients surviving longer than five years after the diagnosis. The recommended treatment for a tumor of this type in an extremity is amputation, with or without radiation therapy and chemotherapy.[8, 9]


Conclusions

  1. Lymphangiosarcoma developing in a setting of filarial lymphedema is very rare.
  2. Patients whose lynmphangiosarcoma is associated with filarial lymphedema in endemic areas may go unnoticed and undiagnosed due to lack of recognition and proper medial care.
  3. Even though the number of cases is small and the follow up is limited, it seems that lymphangiosarcoma developing in the background of filarial lymphedema remains localized for relatively long periods.
  4. Therefore, more cases must be studied to understand the behavior of lymphangiosarcoma in this particular setting.

What is new?

The exact pathogenesis of angiosarcoma in these cases is uncertain. It has been shown in animal models that the environment produced by longstanding lymphedema is conducive to atypical vascular proliferation, with mutations eventually leading to malignant transformation of the endothelial cells. The clinical outcome is poor, with less than 10 percent of the patients surviving longer than five years after the diagnosis


Notes

Source of Support: Nil

Conflict of Interest: Nil.

Acknowledgement

We are thankful to Siddivinayak Cancer Hospital, Miraj for technical help for IHC slides.


References
1. Devi Leena KR,Bahuleyan CK. Lymphangiosarcoma of the lower extremity associated with chronic lymphedema of filarial originCancerYear: 1977141768
2. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 18-1993. A 57-year-old man with chronic lymphedema and enlarging purple cutaneous nodules of the legN Engl J MedYear: 19933281337438469255
3. Stewart FW,Treves N. Lymphangiosarcoma in post-mastectomy lymphedemaCancerYear: 19481648118867440
4. Woodward Anthony,Ivin John C,Soule Edward. Lymphangiosarcoma arising in chronic lymphedematous extremitiesCancerYear: 197230562725051679
5. Richard Muller,Hajdu Steven,Brennan Murray. Lymphangiosarcoma associated with chronic filarial lymphedemaCancerYear: 1987591791833791146
6. El Torraei I. Filarial lymphedema AngiologyYear: 196617644660
7. Sordillo EM,Sordillo PP,Hajdu SI,Good RA. Lymphangiosarcoma after filarial infecctionJ Dermatol Surg OncolYear: 1981723597229182
8. Sordillo PP,Chapman R,Hajdu SI,Magill GB,Golbey RB. LymphangiosarcomaCancerYear: 1981481674797284967
9. Komorowski Andrej,Wysocki Wojciech,Miltus Jerry. Angiosarcoma in a chronically lymphedematous leg: An unusual presentation of Stewart-Treves syndromeSouthern Medical JournalYear: 200396807814515924

Figures

[Figure ID: F1]
Figure 1 

Clinical photograph of patient with bilateral lymphedema (a) and left leg fungating mass (b)



[Figure ID: F2]
Figure 2 

Photomicrograph showing numerous pleomorphic spindle cells lining anastomozing blood Vessels with overlying skin (H and E) ×100 (a) and ×400 (b and c)



Tables
[TableWrap ID: T1] Table 1 

Cases of lymphangiosarcoma reported after filariasis




Article Categories:
  • Case Report

Keywords: Lymphangiosarcoma, filarial infection, lymphedema.

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