Recurrence of Stewarty4Treves Syndrome in a Patient with Chronic Lymphedema Secondary to Breast Cancer.

Sir,

The Stewart-Treves syndrome (STS) was first described in 1948. It is a rare disease consisting of a cutaneous angiosarcoma that develops on a site of chronic lymphedema. The incidence of STS is between 0.07% and 0.45% in patients with radical mastectomy.[1]

We present the case of a 63-year-old woman with a history of right breast cancer. She underwent a radical mastectomy, right axillar lymphadenectomy and adjuvant chemotherapy, and developed secondary lymphedema of the right arm. Ten years later, purple-red nodules appeared on the right hand and progressively extended through the arm, reaching the elbow. A cutaneous biopsy was consistent with a diagnosis of angiosarcoma [Figure 1]. The extension study showed no dissemination of the tumor, and the patient underwent amputation of the limb. Three years later, she was admitted with rapidly growing, painful nodules and plaques around the amputation scar. Also, it was covered with yellowish crusts, and surrounded by an erythematous, warm halo [Figures 2a–c]. Cytology of a fine-needle aspiration and CT scan findings were both compatible with chest wall angiosarcoma recurrence. The patient is currently under palliative treatment with pazopanib, opioids, and topical zinc sulfate.

Figure 1

(a) Histopathological examination revealed the invasion of pleomorphic endothelial spindle-shaped cells with vascular differentiation into the dermis. A proliferating vascular channels that dissect dermal collagen was observed.(hematoxylin-eosin stain, original magnification x4). (b) The tumor endothelial cells are commonly seen undergoing mitotic changes(hematoxylin-eosin stain, original magnification x20)

Figure 2

(a-c) Nodules and plaques around the amputation scar. It is covered with yellowish crusts, and surrounded by an erythematous, warm halo

Most cases of STS occur in women with lymphedema secondary to radical breast cancer surgery, and it usually shows a longer latency period, up to five or more years after breast surgical therapy.[2] The main risk factor is chronic lymphedema, secondary to lymphadenectomy but also associated with filariasis, post-radiation, chronic immobilization or Meige disease. Its pathophysiology is still a matter of controversy:First of all, Stewart and Treves speculated that a systemic carcinogenic factor was the causative agent. In addition, there are findings that suggest that chronic lymphedema causes some degree of local immunosuppression due to the disruption in the lymphatic system, that may facilitate oncogenesis.[3] Its clinical manifestations include areas of cellulite-like alterations, multiple hard purplish nodules, macules and papules or a poorly healing eschar. Lung and chest wall metastasis are usual. Diagnosis is histological, and recent studies showed that this type of angiosarcoma presents phenotypes of both blood and lymphatic endothelia. The most effective treatment is radical surgery including wide excision of the margins with chemotherapy and adjuvant radiotherapy. Nevertheless, even surgical excision with negative margins is associated with 73% of disease recurrence after a 1-year follow up2. Antiangiogenic drugs such as pazopanib can also be employed. Recently, it has been reported that 18 fluorodeoxyglucose (18F-FDG) PET-CT may be useful for the detection of recurrent cutaneous angiosarcoma.[4] However, prevention is one the most important measures, by treating chronic lymphedema with measures such as weight loss, physiotherapy or laser therapy. Other novel technique is the decongestive lymphedema therapy, which has proved to improve cell-mediated immunity in arms after radical breast cancer surgery.[5]

STS is an ominous complication of long-standing lymphedema, classically associated with lymphadenectomy. Its clinical presentation might be misleading, and, as the only curative treatment is complete surgical excision, a high degree of suspicion must be kept when examining a patient with chronic lymphedema, in order to make an early diagnosis and treatment.