Chronic Lymphedema of Filarial Origin: A Very Rare Etiology of Cutaneous Lymphangiosarcoma
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 []. On examination, the right leg showed markedly thickened skin with three brownish nodules with raw surface and oozing of blood. The larger nodule measured 3 cm and smaller one measured 2 cm in diameter. The nodules were soft to firm. Right inguinal lymph nodes (2 in number) were palpable, each measuring 2 × 2 cm. Routine laboratory investigations did not reveal any abnormality. The patient was HBsAg, HCV; and HIV1 and 2 negative. 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. Chest roentgenograms, abdominal ultrasound and CT abdomen did not reveal any abnormal findings. Biopsy was done from one of the nodules. Grossly two skin covered brownish tissues bits were received []. The histopathological examination revealed skin comprised of epidermis, dermis and subcutaneous fat. Epidermis showed irregular acanthosis and focal ulceration. 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 []. Tubular pattern of vascular architecture was confirmed by reticulin stain. Some of the cells showed prominent nucleoli. The mitotic activity was 25/10 HPF []. There were foci of hemorrhages. Dermis and subcutaneous fat also showed focal areas of fibrosis due to chronic lymphedema. The vascular nature of the tumour was confirmed immunohistochemically with endothelial markers like CD 34 and CD 31 displaying distinct membranous positivity [Figures and ].