Age / Sex : 63 / F

우측 팔 부종 및 색소성 병변, 10년 지속

P. Hx. : right breast ca. op. (1999)

What is your diagnosis?

Two weeks later, you can see the final diagonosis with a brief discussion of this case (Please submit only one answer)

Courtesy : 이인숙, In Sook Lee, 부산대학교병원, Pusan National University Hospital

Angiosarcoma(Angiosarcoma associated with chronic lymphedema) or Stewart-treves syndrome

Discussion

Finding
  1. Diffuse subcutaneous edema and skin thickening
  2. Multiple nodular lesions at skin layer



Stewart-treves syndrome


Introduction
1. Stewart and Treves first described the entity of angiosarcom
 - in a chronically lymphoedematous extremity following mastectomy

2. Cutaneous angiosarcoma is a rare (0.45%) complication of a chronic lymphedematous extremity

3. 90% occur in upper extremity after mastectomy
Common factor is chronic lymphoedema, and therefore other causes of chronic lymphoedema can lead to the development of this vascular sarcoma

4. Median time from mastectomy to the development of angiosarcoma is 10 yrs (5-27 yrs)

Clinical presentation
1. Bluish, slightly raised macular or polypoid appearance on a lymphoedematous extremity
2. tends to metastasize through the blood to distant organs, particularly the lungs
3. treatment of choice is amputation
4. survival is poor, ranging from 19 months to 31 months

MRI finding
1. cutaneous thickening and nodularity isointense to muscle on T1 and decreased signal on T2 in a lymphoedematous extremity
2. avid enhancement
3. low signal on T2 and avid enhancement on MR of these lesions distinguishes the tumour from background lymphoedematous tissues
4. low T2 signal of this lesion is unusual for a malignant tumour and probably reflects dense cellularity and prominent fibrous stroma

For surgical planning and to optimize biopsy yield, MRI can determine the extent of the tumour and the depth of invasion as well as identify satellite lesions

References
Oncol Lett. 2018 Jan;15(1):1113-1118
Rep Pract Oncol Radiother. 2020 Nov-Dec;25(6):934-938