Abstract
Cutaneous angiosarcoma is a very aggressive tumor with poor prognosis. We report a case of cutaneous angiosarcoma in a patient with elephantiasis. To the best of our knowledge, there are very few cases reported in the literature. As there is no ideal treatment for cutaneous angiosarcoma, early detection can lower the morbidity and mortality from this malignancy.
Keywords: Cutaneous angiosarcoma, Lower extremities, Filarial lymphedema
Introduction
In endemic areas, the common cause of lymphedema is filariasis. Cutaneous angiosarcoma occurring in the background of elephantiasis is very rare [1].
Case History
A 38-year-old female with elephantiasis of the right leg, for 20 years, presented with an ulcerative lesion measuring 12 × 10 cm, rapidly increasing in size with foul smelling discharge for the last 2 months. The ulcer was black in color with necrotic base and everted friable margins that used to bleed on touch (Fig. 1), with associated right inguinal lymphadenopathy.
Fig. 1.
A clinical photograph of an ulcer measuring 12 × 10 cm with a depth of 2 cm, everted friable margins, necrotic base, and black in color with three satellite lesions
Fine needle aspiration biopsy smears of the right inguinal lymph nodes were done and differential diagnosis of poorly differentiated carcinoma, melanoma, and angiosarcoma were offered. Microscopic examination of the edge biopsy of the ulcer leg showed pleomorphic malignant tumor cells arranged in solid sheets and around anatomizing vascular channels with foci of lymphangiomatosis. The tumor cells were positive for CD31. The histological features were consistent with “angiosarcoma” in a known case of elephantiasis. Sections from the right inguinal lymph nodes showed angiosarcomatous deposits.
An above knee amputation was done to radically treat the condition. Now she is doing well after 6 months of postoperative follow-up
Discussion
More than 400 cases of angiosarcoma related to chronic lymphedema have been reported so far in English literature [2]. Cutaneous angiosarcoma arising in chronic lymphedema after filarial infection is very rare. Muller et al. [3] mentioned three cases. Sordillo et al. [4], Sinclair et al. [5], Hallel-Halevy et al. [6], Komorowski et al. [7] have also reported cases of angiosarcoma in long-standing elephantiasis.
Clinically, angiosarcoma can present as a simple bruise that resembles hemangioma to large nodular or ulcerative lesion. The presence of lymphangiomatosis is a precursor lesion for angiosarcoma, which was seen in our case. Even though immunohistochemistry is confirmatory, histopathological examination is the mainstay for diagnosis.
Conclusion
In endemic areas, cutaneous angiosarcoma should be considered in the clinical differential diagnosis of malignant lesions, as it is a very aggressive tumor with a poor prognosis. Early detection of the lesion can potentially lower the morbidity and mortality.
References
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