Cutaneous nodular exophytic angiosarcoma with involvement of tibiaK Manoharan, S Sivaramakrishnan, D Manoharan, DI Reddy
Department of Dermatology, Venereology and Leprosy, Sree Balaji Medical College and Hospital, Chennai, Tamil Nadu, India
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/jpgm.jpgm_1092_21
Source of Support: None, Conflict of Interest: None
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.
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.
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, 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. 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.
The overall estimated 5-year survival is 15%, reflecting the fact that the treatment of angiosarcoma 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.
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[Figure 1], [Figure 2]