Stewart-Treves Phenomenon: Lymphangiosarcoma Arising in Lymphedematous Tissue Is not a Syndrome

To the Editor

The field of lymphedema is handicapped by imprecise terminology. The major reason is that the term “lymphedema” is used generically to describe any large extremity, regardless of the etiology.^1–3 Patients with primary lymphedema are commonly labeled as having “Milroy disease,” whereas this eponym should be restricted to infant-onset lymphedema with a family history of the condition and/or a VEGFR3 mutation. ¹ Inaccurate nomenclature can cause diagnostic confusion, compromise communication and research, and increase the risk of incorrect patient management. ⁴ We propose changing the double eponym “Stewart-Treves syndrome” to “Stewart-Treves phenomenon.”

Stewart and Treves (1948) described lymphangiosarcoma arising in lymphedematous arms following treatment of breast cancer. ⁵ Their observation was soon thereafter called a “syndrome” by Jessner (1952). ⁶ The designation “Stewart-Treves syndrome” has been used indiscriminately for lymphangiosarcoma arising in any lymphedematous tissue, including primary lymphedema,^7–11 and secondary lymphedema from filariasis, trauma, or malignancy other than breast cancer.^12–14

“Syndrome” is defined by geneticists as multiple, pathogenetically-related anomalies occurring in embryonically noncontiguous areas.^15,16 Lymphangiosarcoma arising in lymphedematous tissue fails to meet this definition because: (1) sarcoma is an acquired lesion, (2) secondary lymphedema is not a congenital anomaly, and (3) the swelling and tumor occur in the same area. The observation by Stewart and Treves does not qualify as an “association” or “sequence” because these terms only apply to congenital abnormalities. An “association” refers to two or more congenital anomalies that occur together more commonly than would be expected by chance.^16,17 A “sequence” describes anomalies that occur in causative succession. ¹⁶

“Stewart-Treves phenomenon” is a more precise and useful term for a lymphangiosarcoma that develops in lymphedematous tissue, regardless of the underlying cause of the swelling. “Phenomenon” is defined as a “sign that is often associated with a specific illness or condition and is therefore diagnostically important.” ¹⁸ There is a terminological precedent: Kasabach-Merritt “syndrome” became Kasabach-Merritt “phenomenon.”^19–21 As our understanding of diseases improves, medical terminology must keep pace.