Primary Epithelioid Hemangioendothelioma of the Penis

A Case Report and Literature Review

Alberto Artiles Medina; Agustín Fraile Poblador; Manuel Hevia Palacios; Irene Carretero Barrio; María Eugenia Reguero; Rafael Rodríguez-Patrón Rodríguez; Francisco Javier Burgos Revilla


Transl Androl Urol. 2021;10(9):3697-3703. 

In This Article

Abstract and Introduction


Epithelioid hemangioendothelioma (EHE) is a rare vascular tumour with an intermediate behaviour between benign hemangioma and malignant angiosarcoma. There is scarce data on the penile EHE's management and its natural history, as our knowledge is based on few reported cases with a short follow-up period. We present a case report and conducted a literature review, including 17 cases. The relevance of this case report derives from the need for better clinical characterization of patients with penile EHE and the importance of defining the outcomes. We report the case of a 53-year-old male with a 1-year history of sleep-related painful erections. Imaging techniques showed a well-defined hypoechoic and hypervascular solid nodule on the dorsal aspect of the penis. It was surgically removed, and the histopathological study revealed a low-risk EHE of the penis. Follow-up magnetic resonance imaging (MRI) and computed tomography did not demonstrate local recurrence nor metastases. According to the literature review, most of the patients were in their fifth and sixth decades of life at the time of diagnosis and lesions were usually located in the glans. The most common clinical presentation was as a painful mass. Follow-up period ranged from 2 months to 5 years. Three patients showed systemic metastases, two of which died due to cancer. The conclusions from the literature review are limited by the reduced number of cases and the short follow-up. This case report highlights the importance of understanding the diagnosis and treatment of this type of rare non-squamous malignant tumours of the penis. Penile EHE is a malignant vascular tumour that is very rare in this location. The best treatment is local excision, with re-excision or intraoperative margins assessment. Occasionally, systemic chemotherapy and radiation therapy can be useful. There is consensus on the importance of very strict follow-up of these patients.


Epithelioid vascular tumours are rare vascular neoplasms. They are a subtype of mesenchymal tumours, defined by their epithelioid morphology, which differentiates them from other vascular tumours.[1] Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumour that was first described in 1975 by Dail and Liebow in the lung as an aggressive bronchoalveolar cell carcinoma.[2] EHE demonstrates an intermediate behaviour between benign hemangioma and malignant angiosarcoma. It can occur in soft tissues, bones and visceral organs, but also as a primary tumour of the blood vessels.

Non-squamous cell carcinoma primary malignancies represent fewer than 5% of penile cancers. Soft tissue malignancies of the penis are mainly vascular tumours, such as Kaposi sarcoma, EHE and angiosarcoma, followed by other sarcomas like rhabdomyosarcoma and leiomyosarcoma.[3]

There is a scarcity of published data on penile EHE's management and its natural history. Until 2015, 17 cases of penile EHE have been reported in the literature.[4] Of these cases, two presented with metastasis and two with multifocal penile EHE lesions. Furthermore, the benign type of the spectrum, the penile epithelioid hemangioma (EH), is also a very rare vascular neoplasm. Until 2015, only 28 cases in the literature have been reported.[5]

To date, reported characteristics of EHE are based on this small number of published cases. The rates of local recurrences, metastases and mortality of this tumour are 10%, 20–30% and 15%, respectively. Treatment decisions should be based on pathological findings. Treatment options include excision or multimodal therapy.[1]

We reported the case of an EHE involving the penis in a 53-year-old male treated with complete resection. This case report contributes to widen our knowledge of this rare tumour and the literature review offers an update on its management.

Literature Review

We carried out a literature review in PubMed (MEDLINE) of reported cases and analysed therapeutic arsenal (surgery, antitumour drugs and radiation) used in the treatment of these unusual tumours.

The search strategy was as follows: ("Hemangioendothelioma, Epithelioid"[Mesh] OR "Hemangioendothelioma"[Title/abstract]) AND ("Penis"[Mesh] OR "penile"[title/abstract] OR "Penile Neoplasms"[Mesh]). Inclusion criteria were: histologically confirmed cases of penile EHE, and no language or temporal restrictions were applied. Two authors independently reviewed the literature and decided which case reports to include in this study. We summarized the case reports in Table 1, and we extracted the most important aspects: age, clinical presentation, size and location, management, follow-up period, local recurrence or metastases, and survival time.

We present the following case in accordance with the CARE reporting checklist (available at