Stewart-Treves syndrome was initially identified by doctors Fred Stewart and Norman Treves in a case series documenting six individuals with lymphangiosarcoma and chronic lymphedema following mastectomy.
Although cases of Stewart-Treves syndrome are attributable to post-mastectomy lymphedema, angiosarcoma development has been linked to persistent lymphedema of any cause. An accurate identification of angiosarcoma is crucial due to the likelihood of metastatic spread, and a generally unfavourable prognosis.
Epidemiology
No region in the body is immune to angiosarcomas, but 60% of them originate on superficial soft tissues or on the skin.
Most cutaneous angiosarcomas develop due to the following conditions:
Exposure to radiation
Most cases arise spontaneously and present themselves on the head and neck region of elderly individuals, particularly the scalp
Due to chronic lymphedema from post surgical issues or in individuals with congenital abnormalities
Stewart-Treves syndrome refers to the 90% of cases of angiosarcomas which develop after a mastectomy.
Anatomy
Pathophysiology
Stewart-Treves syndrome is an uncommon and lethal condition caused by persistent lymphedema complications. In 1948, Stewart and Treves published a case series describing six patients with lymphangiosarcoma following mastectomy and persistent lymphedema.
Between 0.07%-0.45% of patients who are operated for radical mastectomies develop angiosarcomas within the following 5 years. Although most angiorsarcomas associated with Stewart-Treves syndrome occur from post-mastectomy lymphedema, angiosarcomas have been known to develop from persistent lymphedemas despite its origin.
Researchers are still unaware of the association between angiosarcoma and chronic lymphedema.
Stewart and Treves hypothesised that a systemic carcinogenic component is accountable for this process. It has also been claimed that lymphedematous areas undergo a neoplastic change during the establishment of collateral circulation.
Other theories involve a malignant transformation as a result of obstructed lymphatic drainage and reduced antigen presentation, leading to cancer evading immune surveillance at a immunologically privileged site.
Etiology
Soft tissue sarcomas are infrequent malignancies that don’t even make up 1% of all cancers. Angiosarcomas are a form of soft-tissue sarcoma and are malignant endothelial cell tumours of vascular or lymphatic origin with a poor prognosis.
Most angiosarcomas manifest as lesions of middle or high grade. As angiosarcomas can manifest as seemingly benign-appearing tumours, misdiagnosis is unfortunately rather common.
Genetics
Prognostic Factors
Factors which suggest a favourable prognosis include:
Age below 50
Localized stage of the tumor
Tumor in the trunk
According to an institutional review, the overall 5-year survival rate is only 35%, and the 3-year survival rate is 55%.
Clinical History
Physical Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
by Stage
by Modality
Chemotherapy
Radiation Therapy
Surgical Interventions
Hormone Therapy
Immunotherapy
Hyperthermia
Photodynamic Therapy
Stem Cell Transplant
Targeted Therapy
Palliative Care
Medication
Future Trends
Media Gallary
References
https://www.ncbi.nlm.nih.gov/books/NBK507833/
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Stewart-Treves syndrome was initially identified by doctors Fred Stewart and Norman Treves in a case series documenting six individuals with lymphangiosarcoma and chronic lymphedema following mastectomy.
Although cases of Stewart-Treves syndrome are attributable to post-mastectomy lymphedema, angiosarcoma development has been linked to persistent lymphedema of any cause. An accurate identification of angiosarcoma is crucial due to the likelihood of metastatic spread, and a generally unfavourable prognosis.
No region in the body is immune to angiosarcomas, but 60% of them originate on superficial soft tissues or on the skin.
Most cutaneous angiosarcomas develop due to the following conditions:
Exposure to radiation
Most cases arise spontaneously and present themselves on the head and neck region of elderly individuals, particularly the scalp
Due to chronic lymphedema from post surgical issues or in individuals with congenital abnormalities
Stewart-Treves syndrome refers to the 90% of cases of angiosarcomas which develop after a mastectomy.
Stewart-Treves syndrome is an uncommon and lethal condition caused by persistent lymphedema complications. In 1948, Stewart and Treves published a case series describing six patients with lymphangiosarcoma following mastectomy and persistent lymphedema.
Between 0.07%-0.45% of patients who are operated for radical mastectomies develop angiosarcomas within the following 5 years. Although most angiorsarcomas associated with Stewart-Treves syndrome occur from post-mastectomy lymphedema, angiosarcomas have been known to develop from persistent lymphedemas despite its origin.
Researchers are still unaware of the association between angiosarcoma and chronic lymphedema.
Stewart and Treves hypothesised that a systemic carcinogenic component is accountable for this process. It has also been claimed that lymphedematous areas undergo a neoplastic change during the establishment of collateral circulation.
Other theories involve a malignant transformation as a result of obstructed lymphatic drainage and reduced antigen presentation, leading to cancer evading immune surveillance at a immunologically privileged site.
Soft tissue sarcomas are infrequent malignancies that don’t even make up 1% of all cancers. Angiosarcomas are a form of soft-tissue sarcoma and are malignant endothelial cell tumours of vascular or lymphatic origin with a poor prognosis.
Most angiosarcomas manifest as lesions of middle or high grade. As angiosarcomas can manifest as seemingly benign-appearing tumours, misdiagnosis is unfortunately rather common.
Factors which suggest a favourable prognosis include:
Age below 50
Localized stage of the tumor
Tumor in the trunk
According to an institutional review, the overall 5-year survival rate is only 35%, and the 3-year survival rate is 55%.
https://www.ncbi.nlm.nih.gov/books/NBK507833/
medtigo
Stewart Treves Syndrome
Updated :
August 9, 2023
Stewart-Treves syndrome was initially identified by doctors Fred Stewart and Norman Treves in a case series documenting six individuals with lymphangiosarcoma and chronic lymphedema following mastectomy.
Although cases of Stewart-Treves syndrome are attributable to post-mastectomy lymphedema, angiosarcoma development has been linked to persistent lymphedema of any cause. An accurate identification of angiosarcoma is crucial due to the likelihood of metastatic spread, and a generally unfavourable prognosis.
No region in the body is immune to angiosarcomas, but 60% of them originate on superficial soft tissues or on the skin.
Most cutaneous angiosarcomas develop due to the following conditions:
Exposure to radiation
Most cases arise spontaneously and present themselves on the head and neck region of elderly individuals, particularly the scalp
Due to chronic lymphedema from post surgical issues or in individuals with congenital abnormalities
Stewart-Treves syndrome refers to the 90% of cases of angiosarcomas which develop after a mastectomy.
Stewart-Treves syndrome is an uncommon and lethal condition caused by persistent lymphedema complications. In 1948, Stewart and Treves published a case series describing six patients with lymphangiosarcoma following mastectomy and persistent lymphedema.
Between 0.07%-0.45% of patients who are operated for radical mastectomies develop angiosarcomas within the following 5 years. Although most angiorsarcomas associated with Stewart-Treves syndrome occur from post-mastectomy lymphedema, angiosarcomas have been known to develop from persistent lymphedemas despite its origin.
Researchers are still unaware of the association between angiosarcoma and chronic lymphedema.
Stewart and Treves hypothesised that a systemic carcinogenic component is accountable for this process. It has also been claimed that lymphedematous areas undergo a neoplastic change during the establishment of collateral circulation.
Other theories involve a malignant transformation as a result of obstructed lymphatic drainage and reduced antigen presentation, leading to cancer evading immune surveillance at a immunologically privileged site.
Soft tissue sarcomas are infrequent malignancies that don’t even make up 1% of all cancers. Angiosarcomas are a form of soft-tissue sarcoma and are malignant endothelial cell tumours of vascular or lymphatic origin with a poor prognosis.
Most angiosarcomas manifest as lesions of middle or high grade. As angiosarcomas can manifest as seemingly benign-appearing tumours, misdiagnosis is unfortunately rather common.
Factors which suggest a favourable prognosis include:
Age below 50
Localized stage of the tumor
Tumor in the trunk
According to an institutional review, the overall 5-year survival rate is only 35%, and the 3-year survival rate is 55%.
https://www.ncbi.nlm.nih.gov/books/NBK507833/
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