A 56-year-old woman comes to the office due to skin lesions on her right arm that she noticed 4 weeks ago. The patient has no fever or chills. The lesions do not itch. She underwent a right mastectomy for breast cancer 7 years ago and was treated with radiation and chemotherapy. Since her treatment, the patient has had swelling of the right arm. Vital signs are normal. There is nonpitting edema of the right upper extremity. On the inner surface of the right upper arm is a red plaque with no distinct borders, studded with 3 purple papules. The right breast has a well-healed scar, and the left breast is normal. Which of the following is the best next step in management of this patient?
This patient was treated for breast cancer 7 years ago and now has a rapidly progressive, purple skin lesion with indistinct borders, indicating likely angiosarcoma, a rare malignant tumor derived from vascular endothelial cells. Although risk of primary breast angiosarcoma is quite low, patients with breast cancer who undergo radiation therapy or axillary lymph node dissection (with subsequent chronic lymphedema) are at substantially increased risk of secondary angiosarcoma approximately 4-8 years after therapy.
Secondary angiosarcoma typically presents with purpuric papulonodular lesions and red, bruise-like plaques on the breast, axilla, and/or upper arm. Because the tumor spreads within the vasculature, most lesions have indistinct borders. The diagnosis is confirmed by biopsy. Secondary angiosarcoma is an aggressive tumor that readily metastasizes; however, early surgical resection can often be curative.
(Choice B) Lymphocutaneous sporotrichosis, a fungal infection most common in gardeners, often occurs on the arm. However, lesions are erythematous, ulcerated nodules that arise along the lymphatic channels over weeks. The presence of purple nodules on an erythematous plaque with indistinct borders in the area of lymphedema is much more consistent with angiosarcoma.
(Choice C) Chronic lymphedema increases risk of skin and soft tissue infection (eg, Staphylococcus aureus) due to loss of the cutaneous barrier from skin weeping. However, cellulitis typically causes rapidly progressive skin symptoms (eg, erythema, warmth, induration) over days, not weeks. A lesion present for 4 weeks makes bacterial skin infection unlikely.
(Choice D) Secondary angiosarcoma is an aggressive tumor; biopsy should be performed as soon as possible so surgical intervention can be planned. Waiting would increase risk of death.
(Choice E) Topical corticosteroid therapy is used to treat lichen planus, which presents with purple, polygonal papules and plaques with distinct (not indistinct) borders. Furthermore, lesions are intensely pruritic.
Educational objective:
Secondary angiosarcoma is a malignant endothelial tumor that develops 4-8 years after breast cancer therapy. Risk factors include radiation therapy and chronic lymphedema. Typical lesions are red, bruise-like plaques and purple papules and nodules. Timely biopsy is recommended for diagnosis because the cancer readily metastasizes.