Background

Stewart-Treve syndrome is described by Fred Stewart and Norman Treves, hence the name. Six people with lymphangiosarcoma and chronic lymphedema after breast surgery have been studied by them. Angiosarcoma usually happens after mastectomy lymphedema, but this can also occur due to longstanding lymphedema. Early detection or recognition of angiosarcoma is important but the prognosis for this disease is bad or poor. 

Epidemiology

The development of angiosarcoma can happen in any region of the body in which 60% occurs in soft tissues of the skin’s surface which have various causes. Exposure to radiation is one of the causes. Older individuals get them on their head, neck, or scalp spontaneously. Angiosarcomas development from chronic lymphedema happens after surgery due to complications or birth defects. Most of the angiosarcoma cases, nearly 90% after mastectomy surgical procedures refers to Stewart-Treves syndrome. 

Anatomy

Pathophysiology

It is a rare and fatal disease which results from the longstanding problems of lymphedema. Six patients with lymphangiosarcoma been studied by Stewart and Treves after mastectomies. After radical mastectomies, people who develop angiosarcomas are about 0.07% to 0.45% in 5 years. Chronic lymphedema and lymphedema patients mostly get angiosarcoma. The cause for angiosarcoma is not known till now. 

Etiology

The form of cancer which is rare are soft tissue sarcomas which account for less than 1 in 100 cases. Angiosarcomas, a soft tissue sarcoma is dangerous which develops from blood vessels or lymph vessel cells containing rapidly growing cells that are aggressive which can give benign appearance initially which leads to incorrect diagnoses. 

Genetics

Prognostic Factors

There is a low survival rate for this disease in which only 35% of people with angiosarcoma diagnosis live 5 years and 55% people live 3 years. There is overall a poor prognosis. 

Clinical History

Age Group:   

Adults and geriatrics are the susceptible individuals for Stewart-Treves syndrome who had lymphedema after mastectomy which is common in them. 

Physical Examination

The physical examination is done for lymphedema in Stewart-Treves syndrome patients who underwent mastectomy. Lymphedema will happen first in which patients feel tenderness or discomfort in the limb which gets affected. Regions of the body with nodules or plaques hurt more and doctors also check for size of lymph nodes near the armpit. Blood vessels are checked in angiosarcoma. Affected limbs are checked for pulses and blood flow. 

Age group

Associated comorbidity

Those who underwent mastectomy surgery for breast cancer develop Stewart-Treves syndrome which is a condition that arises after this kind of procedure. The development takes place in the limbs after the surgery. Those with this syndrome often received chemo or radiation therapy.

Associated activity

Acuity of presentation

Differential Diagnoses

• Cutaneous Metastasis  
• Cellulitis  
• Lymphangitis  
• Chronic venous dermatitis  

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Treatment strategies involved in the management of Stewart-Treves syndrome include combining surgery with chemotherapy and radiation therapy. The approach is to target the growth and spread of angiosarcoma when lymphedema is managed. Cancer cells are targeted or attacked by surgical excision, radiation, or chemotherapy, along with the management of lymphedema. Follow-ups and close monitoring are required by the healthcare teams because of the aggressive nature of the disease. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-non-pharmacological-approach-for-stewart-treves-syndrome

Individuals with lymphedema should be safe and avoid injuries to prevent swelling. They need to avoid staying in hot and humid climates which can be fatal. Gentle exercises that are helpful for lymphatic circulation are useful and control lymphedema. Staying healthy and eating a balanced diet with proteins, vitamins, and minerals to heal the tissues. 

Role of Chemotherapy Agents

• Doxorubicin: It is an anthracycline drug used in the treatment of Stewart-Treves syndrome. IT binds to DNA and inerferes with topoisomerase II actions which damages caner cells’ DNA. 

Role of tyrosine kinase inhibitor

• Pazopanib: It inhibits the actions of a few receptors which are important for cancer growth. 
• Bevacizumab:  It is an antibody which blocks VEGF-A which inhibits the formation of new blood vessels aorund the tumors. 

use-of-intervention-with-a-procedure-in-treating-stewart-treves-syndrome

Debulking is a surgical procedure used for localized angiosarcoma with major symptoms. The goal is to cut out as many tumors as possible. This decreases pain, improves action, and improves quality of life. This all leads to less compression or invasion from the growth. Procedures like lymphovenous anastomosis or vascularized lymph node transfer which could help in improved lymph drainage and also to cut down the lymphedema swelling in affected limbs. 

use-of-phases-in-managing-stewart-treves-syndrome

Patients with lymphedema or a history of breast cancer surgery are often diagnosed with Stewart-Treves syndrome because of the development of uncommon growths on their skin and swelling happens in the limb. Careful examination is done in order to diagnose and rule out other skin problems where a sample has been taken out for test. Treating the condition is the right approach before going for surgery. After surgery, radiation or chemotherapy is prescribed in which immunotherapy drugs are sued to strengthen immunity in weak patients. Regular follow-ups and treatment monitoring are necessary. Management of complications, palliative care, emotional support, and improving quality of life are important. 

Medication

Future Trends

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK507833/ 

Stewart-Treves Syndrome – StatPearls – NCBI Bookshelf (nih.gov)