|Year : 2022 | Volume
| Issue : 3 | Page : 840-842
Remarkable response to radiation therapy with concurrent chemotherapy in Stewart–Treves syndrome
Atsuto Katano, Hideomi Yamashita
Department of Radiology, The University of Tokyo Hospital, Tokyo, Japan
|Date of Submission||04-Feb-2021|
|Date of Acceptance||29-Mar-2021|
|Date of Web Publication||15-Feb-2022|
7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655
Source of Support: None, Conflict of Interest: None
Stewart–Treves syndrome (STS) is a rare, cutaneous angiosarcoma associated with chronic lymphedema. The prognosis of this syndrome is extremely poor, with a median survival time of 5–8 months, if untreated. An 82-year-old Asian woman noticed a painless elastic mass with partial discoloration (purplish discoloration) on the left thigh. She had lower lymphedema for 15 years. Lesion biopsy and immunohistochemistry analysis led to the diagnosis of angiosarcoma, which was considered to be STS. She was referred to our department for concurrent chemoradiotherapy. Radiation therapy consisted of 25 daily fractions of 2 Gy each (prescription dose: 50 Gy). Concurrent chemotherapy consisted of 2 monthly cycles of docetaxel (75 mg/body on day 1) and recombinant interleukin-2 (700,000 units/body on days 1–5). She experienced acute adverse events such as Grade 2 dermatitis, Grade 2 anemia, and Grade 4 leukopenia. Posttreatment computed tomography images revealed that lesions had disappeared. Moreover, the accumulation patterns on positron emission tomography images were markedly weakened after the treatment. She exhibited no signs of recurrence for 4 years.
Keywords: Angiosarcoma, radiation therapy, Stewart–Treves syndrome
|How to cite this article:|
Katano A, Yamashita H. Remarkable response to radiation therapy with concurrent chemotherapy in Stewart–Treves syndrome. J Can Res Ther 2022;18:840-2
| > Introduction|| |
Stewart–Treves syndrome (STS) is an angiosarcoma that develops in chronic lymphedema caused by various causes. This syndrome was first reported as the result of chronic lymphedema following mastectomy by Stewart and Treves in 1948. The oncogenic mechanism of STS is closely related to local immunodeficiency due to chronic lymphedema, which results in the growth of malignant cells. Yoshida et al. suggested the involvement of pro-inflammatory cytokines such as interleukin (IL)-23 and IL-17 in the immunological background of oncogenesis in the host.
This disease occurs approximately 5–20 years after the onset of lymphedema, and the incidence rate of STS in patients with breast cancer after a radical mastectomy is approximately 0.03%. STS has extremely poor prognosis, with a survival time of 5–8 months, if untreated.
In the present study, we report successful treatment of STS in the lower extremities after uterine cancer with concurrent chemoradiotherapy.
| > Case Report|| |
An 82-year-old Asian woman noticed a painless elastic mass with partial discoloration (purplish discoloration) on the left thigh. She had undergone radical hysterectomy and pelvic lymph node dissection for cervical cancer 27 years ago and had bilateral lower limb lymphedema for 15 years. Computed tomography (CT) scan revealed a 10-cm long bifurcated mass invading the vastus medialis muscle [Figure 1]a with a daughter lesion along the surrounding fascia of the left femur. Positron emission tomography (PET) and-CT revealed abnormally high accumulation of 2-deoxy-2-(fluorine-18) fluoro-D-glucose (18F-FDG; maximum standardized uptake value [SUV max]: 7.0) in the subcutaneous mass and increased accumulation of 18F-FDG in the daughter lesion (SUV max: 2.0), with neither lymphatic nor distant metastasis [Figure 1]c. Lesion biopsy findings led to the diagnosis of angiosarcoma, which was considered to be STS. Immunohistochemical analysis revealed that the tumor cells were positive for CD31, D2–40, CD34 (partially), and Factor VIII-related antigen (only partially) and negative for AE1/AE3 and estrogen receptor.
|Figure 1: Axial computed tomography images of the lesion before (a) and 1 year after (b) radiation therapy. Positron emission tomography images of the lesion before (c) and 6 months after (d) radiation therapy|
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She was referred to our department for concurrent chemoradiotherapy. Radiation therapy was delivered using two parallel opposed fields (6 MV beams). A total dose of 50 Gy was delivered in 2-Gy daily fractions, as a semi-radical treatment [Figure 2], considering the age of the patient. Concurrent chemotherapy consisted of 2 monthly cycles of docetaxel (75 mg/body on day 1) and recombinant IL-2 (700,000 units/body on days 1–5). She experienced acute adverse events such as Grade 2 dermatitis, Grade 2 anemia, and Grade 4 leukopenia, as assessed by the Common Terminology Criteria for Adverse Events version 5.0 and was administered granulocyte colony-stimulating factor. Posttreatment CT images revealed that the lesions had disappeared; however, the edema was present [Figure 1]b. The accumulation patterns on PET images were markedly weakened after the treatment [Figure 1]d. She exhibited no signs of recurrence for 4 years.
|Figure 2: Axial, sagittal, and coronal views of the radiation therapy plan for the patient. Computed tomography images for the radiation therapy plan were obtained in feet first orientation. The red color indicates the area receiving 95% of the prescribed dose|
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| > Discussion|| |
No consensus exists regarding the standard treatment for angiosarcomas, including STS. Four therapies should be considered for the treatment of angiosarcoma: surgical treatment, radiation therapy, immunotherapy, and chemotherapy. Surgical resection is considered the most efficient local treatment; however, its feasibility should be carefully evaluated because of the rapid spread and high degree of invasiveness of the tumor. Radiation therapy after surgery has been shown to improve the prognosis.
In addition, radiation therapy is considered an effective local treatment. Although no clear evidence is available to determine the effective dose and extent of radiation therapy, it is known that the higher the dose, the better the local control. Generally, a dose of 60–70 Gy is required in the case of angiosarcoma of the head. In the present case, a lower dose of 50 Gy (25 daily fractions of 2 Gy each) was administered considering age of the patient, performance status, and preference.
As for chemotherapy, a Phase II study reported that taxanes have a certain therapeutic effect. Some new systemic therapies are under investigation for the treatment of angiosarcoma. In a subgroup analysis of the PALETTE study, the response rate to pazopanib, a multikinase inhibitor targeting vascular endothelial growth factor receptors, was reported to be moderate (20%, 8 of 40 patients). A multicenter, prospective single-arm observational study investigated the efficacy of eribulin mesylate, a microtubule dynamics inhibitor, as second-line therapy in 25 patients with angiosarcoma who had received first-line treatment with taxanes. The results revealed that although the occurrence rate of severe adverse events was high, the median overall survival (primary endpoint) was 8.6 months and the response rate was 20% (5 of 25 patients). Currently, another multicenter Phase II study is investigating the efficacy of regorafenib, an oral multikinase inhibitor, in patients with clinically advanced angiosarcoma (ClinicalTrials.gov Identifier: NCT02048722).
Treatment of postoperative lymphedema, the cause of STS, is essential because curative treatment of STS is difficult. The treatments of lymphedema include elastic compression, diuretic treatment, low-level laser therapy, and surgical treatment. Lymphedema lowers the quality of life of patients after cancer treatment and therefore, appropriate interventions should be performed.
STS is a disease with extremely poor prognosis, and the choice of treatment is based on the condition of the patient. In our case, a durable response to concurrent chemoradiotherapy was observed for more than 4 years.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]