A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema

Abstract

Background: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema.

Objectives: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS.

Methods: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively.

Results: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized.

Study limitations: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors.

Conclusions: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

Keywords: Lymphangiosarcoma; Lymphedema; Stewart Treves syndrome.

Figure 1

(A) The patient had primary lymphedema of the left lower limb for 21 years. Liposuction and excision of local lesions were performed on the left lower limb. Angiosarcoma of the left lower limb was found by pathology after operation. (B) Bone imaging before operation in 2014. (C) Bone imaging after operation in 2016.

Figure 2

MRI showed nodules distributed on the surface of the skin and subcutaneous tissue; it appeared long T1 and long T2 signals. (A) AXI SE T1. (B) AX T2 FSE. (C) AX fs T2 FSE.

Figure 3

MRI showed nodules distributed on the surface of the skin, subcutaneous tissue and some muscles; it appeared slightly long T1 and short T2 signals. (A) AX fs T2 FSE. (B) AX fs T2 FSE. (C) AXI SE T1. (D) AX T2 FSE.

Figure 4

Immunohistochemical and antibody staining of STS. (A) The histopathologic study showed irregular lumens with infiltrative growth in the dermis and subcutaneous tissue, some lumens fused and communicated with each other, and red blood cells could be seen in some lumens. (×200). (B) The epithelium in the lumen was obviously heteromorphic, protruding into the lumen like a tack, and the nucleus was large and darkly stained. (×400). (C) CD34 positive (×200). (D) CD31 positive (×200). (E) D2-40 Locally positive (×200). (F) CK negative (×200). (G) EMA negative (×200). (H) ki67 index 15% (×200).

Figure 5

Survival curve for cases of Stewart-Treves syndrome with amputees and without amputees. Survival time was defined as the survival time from diagnosis to the time of death or the end of follow-up. Survival was calculated using Kaplan-Meier analysis. For Stewart-Treves syndrome, the median survival was 25-months, and the 3^rd Quartile (Q3) survival was 6-months.