A 60-year-old man is referred for evaluation of a “bleeding mole” on his arm. The patients first noticed the lesion approximately 3 weeks ago and it has rapidly increased in size since then. The patient previously underwent extensive surgery accompanied by axillary and cervical lymph node dissection for melanoma that had metastasized to the regional lymph nodes. Following surgery, he developed persistent lymphedema that was unresponsive to compression therapy. Examination reveals marked brawny edema as well as a firm 2cm nodule on the proximal upper left arm that is purple-to-red in color.
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Angiosarcoma is an uncommon soft tissue sarcoma that arises from either lymph or vascular channels.1 Histologically, lesions may be low- or high-grade with the latter manifesting as dense cellularity composed of atypical cells with a high mitotic rate.2 Lower grade tumors are comprised primarily of vascular spaces.
Angiosarcoma can occur in any organ but over half of reported cases are cutaneous in origin followed by the liver.3 The neoplasm is most prevalent in older men, aged 70 to 80 years.2 The scalp is the most common location; prior radiation therapy and chronic lymphedema are both risk factors.3
Angiosarcoma is an aggressive tumor with a 5-year survival rate of less than 40%.4 The major determinants of survival are tumor size and patient age. Other characteristics associated with poor prognosis include infiltration of muscle and a solid histologic pattern comprised of an abundance of cell mitoses.5 Wide surgical excision is the treatment of choice.
Greg Forsyth, PA-C, is a staff member of the DermDox Centers for Dermatology in Camp Hill and Mechanicsburg PA. Stephen Schleicher, MD, is director of the DermDox Center for Dermatology in Pennsylvania, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
1. Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11(10):983-991.
2. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 1998;22(6):683-697.
3. Gaballah AH, Jensen CT, Palmquist S, et al. Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol. 2017;90(1075):20170039.
4. Buehler D, Rice SR, Moody JS, et al. Angiosarcoma outcomes and prognostic factors: a 25-year single institution experience. Am J Clin Oncol. 2015;37(5):473-479.
5. Requena C, Sendra E, Llombart B, et al. Cutaneous angiosarcoma: clinical and pathology study of 16 cases. Actas Dermosifiliogr. 2017;108(5):457-465.