chronic filarial lymphedema is an uncommon etiology for the development of cutaneous angiosarcoma . to the best of our knowledge
lymphedema - associated angiosarcoma also known as lymphangiosarcoma is the commonest type of cutaneous angiosarcoma .
post - mastectomy lymphedema is the most frequent cause , while chronic filarial lymphedema is one of the most uncommon etiology for development of lymphangiosarcoma .
we report the case of cutaneous lymphangiosarcoma developing in chronic filarial lymphedema , because of its rarity and review previously reported three cases .
the prognosis is very poor , with a 5-year survival rate of between 12 and 33% .
a 50 year old male patient was admitted with swelling of right leg since 25 years .
painful nodules and ulcerations were observed on the same leg since three months [ figure 1 ] . on examination ,
the right leg showed markedly thickened skin with three brownish nodules with raw surface and oozing of blood .
right inguinal lymph nodes ( 2 in number ) were palpable , each measuring 2  2 cm .
right leg venous doppler study revealed subcutaneous edema with normal wall to wall colour flow .
patient was a known case of filariasis and had chronic lymphedema of 25 years duration .
aspiration cytology of right inguinal lymph nodes showed chronic lymphadenitis with no evidence of any live or dead microfilariae .
dermis showed a tumour comprised of ill - defined lobules of malignant spindle cells with intervening stroma showing proliferating slit - like vascular channels lined by plump cells with scant cytoplasm and hyperchromatic nucleus [ figure 3 ] .
the vascular nature of the tumour was confirmed immunohistochemically with endothelial markers like cd 34 and cd 31 displaying distinct membranous positivity [ figures 5 and 6 ]
. marked thickening of the skin of the right lower leg with two ulcerated nodular lesions gross photograph showing two brownish tissue bits photomicrograph showing a dermal tumour comprised of ill - defined lobules of malignant spindle cells with intervening stroma showing proliferating slit - like vascular channels lined by plump cells with scant cytoplasm and hyperchromatic nucleus ( h and e , 40 ) photomicrograph showing high mitotic activity ( h and e , 400 ) photomicrograph showing distinct membranous positivity by malignant endothelial cells ( cd34 , 100 ) photomicrograph showing distinct membranous positivity by malignant endothelial cells ( cd31 , 40 )
angiosarcoma includes lymphangiosarcoma and malignant hemangioendothelioma , as there are no currently reliable means of distinguishing blood vascular from lymphatic endothelial differentiation ( or origin ) .
it is likely that individual cases of angiosarcoma may differentiate in either or both directions .
cutaneous angiosarcoma are further divided into ( 1 ) idiopathic angiosarcoma of head and face ; ( 2 ) lymphedema - associated angiosarcoma also known as lymphangiosarcoma ; and ( 3 ) post radiation angiosarcoma .
lymphangiosarcoma arises in arm of females 1 - 30 years after mastectomy with removal of axillary lymph nodes , with or without radiation therapy ( stewart - treves syndrome ) .
more rarely this type can also occur in other types of chronic lymphedema , including congenital , iatrogenic , lymphatic malformations and filarial lymphedema .
the prognosis is very poor , with a 5-year survival rate of between 12 and 33% .
the association of lymphangiosarcoma with chronic filarial lymphedema is very rare . to the best of our knowledge
only three cases have been reported in the world literature till date and ours is the fourth case.[24 ] we report this case because of its rarity and to highlight this rare complication of chronic filarial lymphedema , so that the treating physician is aware of this impending fatal complication and early radical surgery may save the life of the patient . in our case
, there was no history of leg or groin trauma , surgery , radiation , or lymphadenopathy before the development of lymphedema .
although we did not find viable parasites , the patient 's history and tissue findings support a diagnosis of chronic filariasis . in two previously reported cases ,
areas of calcifications were noted consistent with prior filarial infection , which was not seen in our case .
histologically , all the four cases including ours , showed above described characteristic histological features.[24 ] we have ruled out epithelioid hemangioendothelioma , which is usually seen in middle aged people in soft tissue and visceral organs with no report of any cutaneous location .
kaposi sarcoma can be ruled out , as it has never been described in association with chronic lymphedema .
our patient was hiv 1 and 2 negative and there was no history of receiving any immunosuppressive therapy .
patients suffering from chronic filarial lymphedema should be monitored for possible development of lymphangiosarcoma and any suspicious lesion should be biopsied .
the prognosis of lymphangiosarcoma due to chronic lymphedema is similar to idiopathic angiosarcoma of head and face , however the behaviour of lymphangiosarcoma due to chronic filarial lymphedema is not known and therefore more cases need to be studied for proper understanding of its course .
 lymphedema - associated angiosarcoma also known as lymphangiosarcoma is the commonest type of cutaneous angiosarcoma . 
 post - mastectomy lymphedema is the most frequent cause , while chronic filarial lymphedema is one of the most uncommon etiology for development of lymphangiosarcoma . 
 we report a case of a 50 year old male suffering from chronic filarial lymphedema of right lower extremity , presented with brownish nodules on the right leg , which were diagnosed histopathologically as lymphangiosarcoma . 
Introduction
Case Report
Discussion
Conclusion