Epithelioid hemangioendothelioma (EHE) is an extremely rare cancer, accounting for less than 1% of all vascular tumors, demonstrating features between those of hemangioma and angiosarcoma [1]. Although first described by Dail and Liebow in 1975, the term EHE was only first proposed by Weiss and Enzinger in 1982 [1]. These tumors can occur at any age, with 38 years being the median age at diagnosis [2]. The most common presenting symptom is pain, along with less commonly reported symptoms such as cough, palpable mass, or fatigue. Nearly one-third of patients with EHE are asymptomatic and tumors are discovered incidentally [2]. While likely endothelial in origin, EHE is extremely heterogeneous in presentation and prognosis, complicating diagnosis and clinical decisions [3]. EHE can occur nearly anywhere in the body. Primary cutaneous EHE is rare and should prompt suspicion of metastatic disease, especially if multifocal in the skin [4]. Owing to their rarity and similarities to other diagnoses, cutaneous EHE lesions are commonly misdiagnosed [5]. Previous studies suggest the diagnosis of strictly cutaneous EHE incurs a 17% mortality rate at 3 years, highlighting its relatively aggressive nature [6]. It is paramount for dermatologists and dermatopathologists to be aware of EHE and its defining characteristics to minimize the risk of missing this crucial diagnosis.

We report a case of two periauricular lesions with dermal proliferation consistent with EHE, leading to the discovery of underlying metastatic EHE with pulmonary and hepatic involvement in a 24-year-old woman. The aim of presenting this case is to enhance understanding of EHE, an uncommon cancer that is not well studied.

A 24-year-old woman presented to our dermatology clinic with a left posterior auricular papule and left preauricular papule present for 8 and 4 months, respectively (Figure 1). The patient had no significant medical or social history, including no tobacco or heavy alcohol use. Both lesions were painful and progressively enlarging. The patient denied any other symptoms.

Shave biopsy was taken of both lesions. The histology of both lesions demonstrated cellular dermal proliferations of epithelioid cells with the eosinophilic cytoplasm arranged in cords within a myxohyaline stroma (Figure 2). Subtle vacuoles containing red blood cells were present within some of the cells (Figure 3). Histological and immunohistochemical findings were consistent with the diagnosis of EHE (Table 1).

Due to multifocal cutaneous disease, there was high clinical suspicion of metastatic disease. Our patient was referred to medical and surgical oncology for further evaluation, and computed tomography (CT) scans of the head, neck, chest, abdomen, and pelvis were performed. Innumerable bilateral pulmonary nodules, a 1.8-cm hypoattenuated hepatic lesion, and prominent bilateral axillary lymph nodes were noted, all consistent with metastatic disease. After seeking multiple opinions from oncology, our patient elected the watchful waiting approach. Serial CT scans every 3 months were recommended to monitor disease progression.

The patient provided consent to publish information regarding her case, including photographs and relevant findings. Identifiable patient information has been appropriately masked or omitted to comply with ethical standards and patient privacy.