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Multiple recurrent staphylococcal skin abscesses treated with IV antibiotics and serial I&Ds
34%
42/122
Progressively debilitating polyarticular osteoarthritis
11%
14/122
Modified radical mastectomy with subsequent radiation therapy 12 years ago
36%
44/122
Remote anterior shoulder subluxation after an accidental injury 30 years ago
2%
2/122
Former 1 pack-per-day smoker, who quit 10 years ago
13/122
Select Answer to see Preferred Response
This patient's presentation is consistent with a diagnosis of lymphangiosarcoma. Lymphangiosarcoma is of particular concern in patients suffering from chronic lymphedema, a rare but serious complication of either axillary lymph node dissection or irradiation in the setting of breast cancer treatment. Lymphangiosarcoma is a rare neoplasm of the endothelial lining of lymphatic channels. When the condition occurs following mastectomy with lymph node dissection or axillary radiation, it is termed Stewart-Treves syndrome. It occurs roughly 10 years following these preceding interventions. The pathogenesis of the condition remains unknown; however, it is often preceded by chronic lymphedema. The tumor metastasizes quickly and often presents with purple discoloration that rapidly progresses to ulceration and necrosis of tissue. Maughan et al. discuss the current management of breast cancer. They state that, for cancers with negative axillary lymph nodes by physical exam, sentinel lymph node biopsy is the preferred course of action. Sentinel node biopsy does not share the adverse effects of pain and arm swelling that can be commonly seen with axillary lymph node dissection. Thus, chronic lymphedema and lymphangiosarcoma are waning problems in the setting of these changing strategies for treating breast cancer. Sharma et al. review the pathogenesis and management of Stewart-Treves syndrome. They conclude that, while this lymphangiosarcoma is classically a consequence of prior radical mastectomy, it also occurs in the setting of both congenital as well as other causes of secondary chronic lymphedema. The incidence of the syndrome has been on the decline due to improvements in the techniques for breast cancer treatment; however, its prognosis remains poor. Chemoradiation therapy is ineffective at improving outcomes; wide local excision or amputation offers the best opportunities for survival. Figure A depicts the cutaneous presentation of lymphangiosarcoma in a woman's left upper extremity. Illustration A depicts a higher resolution presentation of the malignancy; note the ulceration and developing necrosis of the surrounding tissue. Illustration B shows the distribution of axillary lymph nodes. Incorrect Answers: Answers 1, 2, 4, & 5: These elements of the patient's history would not be expected to result in chronic lymphedema, which is the postulated preceding pathology for the development of lymphangiosarcoma.
4.0
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