Make the Diagnosis

Make the Diagnosis - November 2017

A 62-year-old healthy man presented with a skin lesion located on the left scalp. The lesion was swollen and painful and had been present for 4 months. This lesion had not been treated in the past.

Courtesy Parteek Singla, MD, and Susannah McClain, MD
Upon physical examination, a violaceous plaque was present on the right forehead and scalp. The patient reported the lesion bled easily with minimal trauma. A biopsy by punch technique was performed.

Make the diagnosis:

Angiosarcoma

Kaposi's sarcoma

Retiform hemangioendothelioma

 



Angiosarcoma is also known as malignant hemangioendothelioma, hemangiosarcoma, and lymphangiosarcoma. It is an uncommon, high-grade malignant vascular neoplasm of the inner lining of blood vessels. Unlike most sarcomas, it occurs more superficially, most often on the head and neck (particularly on the scalp). This neoplasm occurs twice as often in males as it does in females. Angiosarcomas can occur in the breast after radiation therapy, as well as in the liver and spleen, but 60% are cutaneous.
 

Angiosarcoma Courtesy Parteek Singla, MD, and Susannah McClain, MD
Most commonly, angiosarcomas are seen in the elderly on sun-damaged sites, such as the head and neck, without lymphedema. Other risk factors include radiation therapy, with lesions typically occurring 5-10 years after treatment. Also, chronic lymphedema (Stewart-Treves syndrome), most commonly following after breast cancer treatment with axillary node dissection, increases the risk of angiosarcomas. This condition has a poor prognosis, with metastases to the lung as the most frequent cause of death.

Clinical exam findings may show a violaceous lesion similar to a bruise on the head and neck that does not heal or bleeds when scratched; this is of particular concern when the lesion has appeared in an area of prior radiation therapy. Deeper tumors may be felt as a soft nodule. Ulceration may be present. Biopsy of the lesion will show hyperchromatic, pleomorphic tumor cells that dissect between collagen bundles with endothelial cells that are multilayered along with hemorrhage. Malignant cells stain positive for CD31, CD34, ERG, and FLI1.

For localized disease, surgery with wide local excision plus adjuvant radiation therapy can be used. For metastatic disease, chemotherapy is the treatment modality of choice. Unfortunately, prognosis is poor, with a 5-year survival rate of about 35% in nonmetastatic angiosarcoma cases. The majority of recurrences – approximately 75% – occur within 24 months of local treatment.

This case and photo were submitted by Parteek Singla, MD, of the division of dermatology at Washington University and at Barnes-Jewish Hospital, both in St. Louis, and by Susannah McClain, MD, of Three Rivers Dermatology, Coraopolis, Pa.

Dr. Donna Bilu Martin, a dermatologist in private practice in Aventura, Fla.
Dr. Donna Bilu Martin

Dr. Bilu Martin is a board-certified dermatologist in private practice at Premier Dermatology, in Aventura, Fla. More diagnostic cases are available at edermatologynews.com. To submit a case for possible publication, send an email to dermnews@frontlinemedcom.com.

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