From diagnosis to treatment: Navigating a rare case of angiosarcoma of the scalp

Angiosarcoma is a very rare malignant soft tissue tumor that arises from endothelial cells and has poor prognosis. The presentation of angiosarcoma varies from a small plaque to multifocal nodules and can occur anywhere in the body, particularly in the head, neck, and scalp and most commonly in the elderly. Scalp angiosarcoma is a rare but aggressive tumor with poor prognosis and lower median survival rates, especially in older individuals; hence, recognizing the risk of local recurrence is crucial. Our case adds to the understanding of this rare tumor and its diagnosis and comprehensive management in improving outcomes for affected individuals. Due to its rare occurrence and benign clinical presentation frequently results in delayed diagnosis, resulting in lower survival rates, as only eight cases of angiosarcoma of scalp have been reported in India over the past two decades. This case report emphasizes that, in diagnosing angiosarcoma, the integration of markers, alongside clinical and morphological findings is essential. A 66-year-old male presented with a grayish, soft, growth on the scalp located over the left frontolateral aspect of the scalp for 6 months, with fragile epidermis which bleeds on manipulation and revealed atrophic scarring on one side with mild induration on peripheral margin of the plaque. Initially thought to be a pyogenic granuloma, on biopsy, it was diagnosed as angiosarcoma with microscopic involvement of the margins. The lesion was excised with clear margins and defect was initially closed by performing advance flap followed by a rotational flap surgery to prevent recurrence and to provide better aesthetic outcome. Any growth on the scalp which bleeds easily and clinically does not have features of granuloma pyogenicum should be investigated to rule out angiosarcoma. This case study provides a comprehensive overview of the patient’s presentation, histopathology, immunohistochemistry, radiological findings, and the treatment plan. It showcases the challenges faced during surgery and the subsequent management to address complications. It also emphasizes the importance of a multidisciplinary approach in the diagnosis and treatment of angiosarcoma of the scalp.