This report presents a rare case of cutaneous angiosarcoma in a pediatric patient in scalp location. Angiosarcoma is a malignant tumor of the vascular endothelial cells with a dismal prognosis and clinical course. It is one of the rarest soft tissue tumors and accounts for 1% of the soft tissue sarcomas [2]. It commonly presents in the elderly age group (7th decade) in the sun-exposed regions (head and neck), deep soft tissue or in a previous history of clinical intervention (viz., lymphedema or radiation therapy). Although benign vascular and mesenchymal tumors are more common in pediatric age group, there are rare instances for angiosarcomas in this age group (0.3% of overall sarcoma incidence). Only countable number of pediatric cutaneous angiosarcomas are reported in literature [3–5]. It can be challenging to diagnose angiosarcoma in a patient under the age of 21, partly because the condition is uncommon in this age group [1] and in several instances, the morphology is misleading towards a benign lesion. Several benign and malignant tumors which are considered in its differential include kaposi sarcoma, epithelioid hemangioendothelioma, hemangiopericytoma, and spindle cell hemangioendothelioma and kaposiform hemangioendothelioma [6, 7]. The literature has mostly documented pediatric angiosarcomas as case reports and short series, including descriptions of their emergence in the heart, liver, head and neck, spleen, abdomen/pelvis and bone [1]. According to published literature, the average age of presentation varied from 1 to 16 years old (mean 8 years; median 9 years), with a male preponderance (male to female ratio − 3 : 1) [5]. Majority of cases present as incidental painless mass in an otherwise asymptomatic patient [8]. In the index case, radiology revealed an extradural mass with dural involvement and intracranial extension. Histopathological examination is the preferred modality for diagnosis as CT and MRI has its own limitations [9]. Kaposi sarcoma shows prominent spindle cell proliferation around the pre-existing blood vessels with bland cytology in contrast to the infiltrative vasoformative tumor with marked nuclear atypia as observed in angiosarcoma [10]. The epithelioid hemangioendothelioma is another entity posing diagnostic dilemma with epithelioid cells arranged in cord-like pattern dispersed in a myxohyaline stroma and tumor share similar immunohistochemical profile for vascular markers along with positivity for epithelial membrane antigen (EMA) which is lacking in angiosarcoma [10]. The composite hemangioendothelioma may also be confused with angiosarcoma at times however presence of cavernous vessels are a notable distinguishing feature between these two tumors.
Pediatric angiosarcomas are always high grade in nature with local aggression and incidence of metastasis (4). Soft tissue sarcomas are thought to have a poor prognosis for tumors larger than 5 cm, with tumor size being a major prognostic factor these cases [11]. Mitotic count and degree of differentiation are individual prognostic factors [12]. Presentation status is also predictive of tumor-related mortality [6, 11].
In conclusion, we present a rare example of cutaneous angiosarcoma in an adolescent male who presented with a scalp swelling along with intracranial extension. The rarity of this tumor at this site and age group makes it a challenging diagnosis to render. However, an accurate and timely diagnosis is of utmost importance in providing the optimum treatment to these patients.