Presenting to our institution was a 81 year old female patient with a past medical history of cervical cancer in 2008,who had undergone both radiation and chemotherapy with resulting chronic edema of bilateral lower extremities. The patient who had eight years of the chronic lymphedema, presented with worsened lower extremity swelling and a new rash on her right lower extremity that had been present for two to three months. Of note she had previous hospitalizations within the year with prior complaints of worsened lymphedema. At those visits lower extremity duplex ultrasounds revealed a fast enlargening lymph node in right lower extremity.Unfortunately both her worsened edema and the new enlarging lymph node were then overlooked.
On examination she was found to have gross non-pitting edema of both lower extremities. On the antero-medial aspect of her right lower extremity was a large erythematous area with papular and well-defined nodular, purpuric lesions. There were many areas that were tense and appeared blood filled with minimal tenderness.
Patient underwent skin biopsy by a dermatologist. The pathology later revealed CD 31+ and 40+ cells,with an unfortunate,surprise diagnosis of cutaneous angiosarcoma.
Discussion:
Stewart-Treves syndrome is a rare, fatal cutaneous angiosarcoma that develops in long-standing chronic lymphedema. This syndrome was first reported in 1948 by Drs Fred Stewart and Norman Treves, who noted 6 cases of lymphangiosarcoma that developed several years after radical mastectomy. To date there are only a few case reports that highlight lower extremity as being involved.
Cutaneous angiosarcoma is a destructive and often fatal vascular tumor. Despite treatment, the prognosis of cutaneous angiosarcoma remains poor. A high level of suspicion and early management of the lesion are very important in improving the potential for patient survival.
Conclusions:
As hospitalists, we must be intellectually curious and offer full work up to the causes of chronic symptoms such as lower extremity edema. Chronic lower extremity edema should be monitored and with the development of new skin findings, angiosarcoma should be considered. As this is an aggressive tumor swift referral is needed.