Angiosarcoma is a rare and aggressive tumor with high rates of metastasis and relapse. It shows a particular predilection for the skin and superficial soft tissues. We report three distinct and typical cases of angiosarcoma that were diagnosed in a single dermatology clinic over the course of less than a year: i) Angiosarcoma in lower limb affected by chronic lymphedema, featuring Stewart-Treves syndrome; ii) a case of the most common type of angiosarcoma loated in the scalp and face of elderly man and; iii) a skin Angiosarcoma in previously irradiated breast. Read More
Case Rep Surg 2017 9;2017:4056459. Epub 2017 Feb 9.
Department of General Surgery "A", Dr. Tidjani Damerdji University Hospital of Tlemcen, Tlemcen, Algeria; Experimental Surgery Laboratory N°38, Medical School of Tlemcen, University of Abou Bekr Belkaid, Tlemcen, Algeria.
Steward-Treves syndrome is a cutaneous angiosarcoma that usually appears after long evolution of a lymphoedema after mastectomy for mammary neoplasia associated with an axillary dissection. This is a rare disease develop most of the time in upper arm and often confounded with cutaneous metastasis. Only the biopsy and immunohistochemical study confirm the diagnosis. Read More
Angiosarcoma (AS) is an uncommon vascular malignancy with an aggressive clinical course. Radiation-associated angiosarcoma (RAAS) and Stewart-Treves syndrome are associated with MYC gene amplification and protein overexpression, while other radiation-associated vascular lesions including atypical vascular lesions (AVL) are not associated with MYC overexpression. In contrast, de novo AS represent a group of molecularly heterogeneous tumours, for which MYC expression has not been extensively examined. Read More
aDivision of Gastroenterology and Hematology/Oncology, Department of Medicine bDepartment of Dermatology, Asahikawa Medical University, Hokkaido cGastroenterology Medicine Center, Shonan Kamakura General Hospital, Kanagawa dCenter for Clinical and Biomedical Research, Sapporo Higashi Tokushukai Hospital, Sapporo, Japan.
Background: Pancreatic involvement of angiosarcoma is extremely rare.
Methods: We herein report a rare case of angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) with pancreatic metastasis that was diagnosed using endoscopic ultrasound (EUS)/fine needle aspiration (FNA).
Results: A 43-year-old woman with a history of radical hysterectomy with bilateral inguinal lymphadenectomy and chemoradiotherapy for cervical cancer 15 years prior noticed the presence of erythematous indurative plaques on her right femoral region, where chronic lymphedema had developed. Read More
The Stewart-Treves syndrome is a rare and deadly entity, which is defined as angiosarcoma arising in the setting of chronic lymphedema. It typically presents in women who develop lymphedema in the upper extremity secondary to axillary lymph node dissection for breast cancer surgery. It is extremely uncommon in the lower extremities as a result of idiopathic chronic lymphedema. Read More
Stewart Treves Syndrome is an angiosarcoma generally seen long time after radical mastectomy in breast carcinoma patients in chronic lymphoedema area. It's an angiosarcoma developed on a long standing lymphoedema, following a radical mastectomy. An 86-year-old woman was referred to our Dermatology outpatient clinic which developed a giant erythematous, oedematous, purplish lobulated plaque on her right forearm anteromedially with a few satellite erythematous papulonodules on her arm. Read More
Lymphangiectasia is characterized by vesicular dilation of lymphatic vessels and is generally a complication of radiotherapy or surgery of various malignant tumors such as breast and cervical cancers. Although it is not a precancerous disease, correct diagnosis is important to rule out Stewart-Treves syndrome which is defined as lymphangiosarcoma that develops in long-standing chronic postmastectomy lymphedema. Observation alone or many therapeutic options including cryosurgery, electrocauterization, sclerotherapy, and excision have been widely used in the management. Read More
Aims: To study the clinicopathologic features of Stewart-Treves syndrome (STS) in postmastectomy patients including the epidemiology, presentation, morphology, differentiation, pathogenesis and therapeutic options.
Methods And Results: Ten cases of STS in postmastectomy patients were retrospectively identified in our archives, and immunohistochemistry for CD34, CD31, D2-40, HHV-8, CK, EMA and Ki-67 was performed. All ten patients presented with lymphedema after mastectomy as the first sign. Read More
Background: Stewart-Treves syndrome is a rare complication of breast cancer treatment, representing a lymphangiosarcoma commonly associated with lymphedema and severely impacting patient's outcome. The tumor typically develops in the atrophic, pachydermatous, hyperkeratotic skin of limbs affected by long-standing lymphedema. Clinical data associated with Stewart-Treves syndrome and lymphedema management have rarely been published. Read More
Stewart-Treves syndrome is a rare cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Though most commonly this angiosarcoma is a result of post mastectomy lymphoedema, it also develops in Milroy disease, idiopathic, congenital, traumatic and filarial lymphoedema. Despite the rarity of this syndrome and its poor prognosis, early diagnosis associated with radical surgery can provide improved survival. Read More
Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. Read More
Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Read More
Background: Breast angiosarcomas are rare neoplasm. Due to its rarity, our therapeutic strategy is extremely limited. Therefore, we investigated the clinicopathologic features and examined the treatment for angiosarcoma compared with some literatures. Read More
Patients with skin complaints secondary to oedema are commonly encountered and can be a diagnostic challenge. Here, we present the case of a 78-year-old lady with Stewart-Treves syndrome, a rare cutaneous angiosarcoma. The histology showed angiocutaneous sarcoma with poorly defined margins. Read More
The Stewart-Treves syndrome was first described in 1948, it's an angiosarcoma developed on a longstanding lymphadenomatous limb, more often after radical mastectomy. Diagnosis is made on skin biopsy and the prognosis is poor when radical surgery can't be performed. We report the case on a Stewart-Treves syndrome in a sixty-six years old woman who underwent radical mastectomy for breast carcinoma ten years earlier. Read More
Stewart-Treves syndrome is an angiosarcoma that occurs because of chronic lymphedema, which in most cases is a complication after mastectomy with axillary node dissection and postoperative radiation. Prognosis for this rare tumor is poor. The best therapy is early and radical excision. Read More
Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. Read More
Chronic lymphedema has a permissive effect with certain types of malignancies, particularly angiosarcomas, in what is known as Stewart-Treves syndrome. The presumed mechanism of this effect is an immunocompromised district of the affected area. Most other cutaneous malignancies have also been described in lymphedematous areas, including basal cell carcinoma, squamous cell carcinoma, melanoma, Kaposi sarcoma, Merkel cell carcinoma, and several cutaneous lymphomas. Read More
Besides the systemic immune deficiency, a sectorial default in immune control may occur in immunocompetent subjects. This regional immune defect can appear and remain confined to differently damaged skin areas, lately labeled immunocompromised districts (ICDs). An ICD is a skin area more vulnerable than the rest of the body for genetic or acquired reasons. Read More
A woman with postmastectomy lymphoedema presented with purple-red nodules on the right upper arm. Histopathology of a skin biopsy showed a cutaneous angiosarcoma. Angiosarcoma arising in an oedematous arm is called the Stewart-Treves syndrome and is a rare and late complication of breast cancer treatment. Read More
From the *Department of Nuclear Medicine and PET Center, China Medical University Hospital, Taichung, Taiwan; †Department of Biomedical Imaging and Radiological Science, China Medical University, Taichung, Taiwan; and ‡School of Medicine, China Medical University, Taichung, Taiwan.
This 17-year-old woman had chronic congenital lymphedema in the left lower extremity since childhood. She underwent surgeries to remove excessive lymphedematous tissues more than 15 times previously. Histopathology of the specimen from the recent surgery revealed angiosarcoma; therefore, FDG-PET/CT scan was arranged to determine the extent of tumor spread, and distant metastases were discovered. Read More
Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Read More
We present a case of chronic lymphoedema that progressed to Stewart-Treves syndrome in a 63-year-old woman with a previous modified radical mastectomy, associated lymph node dissection, chemotherapy and radiotherapy. While producing stabilisation of most cutaneous lesions initially, chemotherapeutic treatment of the angiosarcoma did not prevent subsequent metastasis and patient death. We urge vigilance and regular follow-up appointments for patients following a mastectomy with chronic lymphoedema to facilitate prevention or early treatment of this devastating syndrome. Read More
Angiosarcomas account for less than 0.05% of the malignant neoplasms of the breast. We present here 7 cases of malignant vascular neoplasms of the breast, including 2 cases of postmastectomy Stewart-Treves syndrome. Read More
Cutaneous angiosarcomas (CAS) are uncommon, aggressive tumours. Very rarely, they arise from the lower extremity. Such tumours are usually associated with chronic lymphedema, a phenomenon known as Stewart-Treves Syndrome. Read More
Hemangioendotheliomas are vascular neoplasms occupying a spectrum of biological potential ranging from benign to low-grade malignancy. Composite hemangioendothelioma (CH) is one of the less commonly encountered variants exhibiting a mixture of elements of other hemangioendothelioma subtypes, such as epithelioid, retiform, and spindle cell. Some authors have identified areas histopathologically equivalent to angiosarcoma within CH, raising the question of the true nature of this neoplasm. Read More
Lymphedema (LE) is a chronic medical condition characterized by lymphatic fluid retention, resulting in tissue swelling. Cancer treatments involving lymph nodes can damage lymph drainage routes, causing accumulation of lymph fluid in the interstitial tissue of related limbs and body areas and secondary LE. Basically, the LE has a negative impact on physical and mental quality of life. Read More
Background: The development of angiosarcoma in oedematous tissue is referred to as Stewart-Treves syndrome (STS). This rare and fatal complication is associated with chronic post mastectomy lymphoedema and radiotherapy for breast cancer. Angiosarcoma spread is facilitated by the formation of blood vessels (angiogenesis) and lymph vessels (lymphangiogenesis). Read More
Several randomized trials have shown that breast-conserving therapy (BCT) is as effective as mastectomy and should be a standard treatment for early-stage breast cancer. Recently, there has been an increase in reports of angiosarcoma (AS) after BCT. Herein, we report a case of AS which developed after BCT and a case of Stewart-Treves syndrome with a focus on lymphedema. Read More
Angiosarcoma is an uncommon but aggressive tumor of endothelial origin that may occur in the upper extremities of patients with postmastectomy lymphedema (Stewart-Treves syndrome) as well as in other regions. We present an unusual case of angiosarcoma associated with congenital nonhereditary lymphedema in an 18-year-old man. Our case underscores the need for a careful clinical examination and shows the importance of appropriate sampling and thorough pathologic examination of suspicious areas to exclude the presence of a malignant process. Read More
Background: Chronic lymphoedematous limbs have an increased propensity for infections and primary or secondary malignant tumours. It has been attributed to suppressed delayed-type hypersensitivity measured in lymphoedemas related to Stewart-Treves syndrome, Kaposi's sarcoma or breast cancer treatment. Cell-mediated immunity is an effective defence mechanism against bacteria, fungi, viruses and tumour cells. Read More
We report a case of a Stewart-Treves syndrome of the lower limb. The tumor is best described in the upper limb following breast cancer treatment but a small number of cases have arisen in lymphedema of the lower limb. Electrochemotherapy could be useful in the palliative treatment of this lymphangiosarcoma. Read More
Stewart-Treves syndrome is a malignancy that arises within chronic lymphedema. Although classically described as a consequence of radical mastectomy, this lymphangiosarcoma has been documented to occur in cases of congenital and other causes of chronic secondary lymphedema. The development of this aggressive lymphangiosarcoma at sites of chronic lymphedema renders it a possible model for Kaposi sarcoma. Read More
Stewart-Treves syndrome is a rare cutaneous angiosarcoma with a poor prognosis. Physicians must be aware of this lethal syndrome, especially in patients who have been treated for breast cancer with radiation and lymph-node dissection (such as the case reported here). Patients who develop unexplained enlarging plaques of coalescing purple papules should have immediate biopsy for early diagnosis of Stewart-Treves syndrome. Read More
Backgrounds: The cancerogenic effect of ionizing radiation was documented just several years after it started to be used as a treatment option. Ionizing radiation produces a small but detectable risk of carcinoma as well as bone and soft tissue sarcomas. Over the past 20 years angiosarcomas arising from the area of the irradiated breast have been reported with increasing frequency as the number of women undergoing the breast conserving surgery with consecutive radiotherapy has increased also. Read More