Related Terms: Lymphangiosarcoma, Stewart-Treves Syndrome, Lymphedema, cutaneous angiosarcoma, soft tissues, cancer, Atypical vascular lesion, Angiosarcoma, Kaposi’s sarcoma, Radiotherapy, Histology, VEGFR-3, MYC, MMP-9, Bisphosphonate, Docetaxel, Radiation-induced neoplasms,
When I first became active online regarding lymphedema, the only articles that were available pertaining to cancer secondary to lymphedema were ones about Stewart Treves Syndrome, also known as lymphangiosarcoma. Since that time, it has become recognized that angiosarcomas in general represent a threat to long standing lymphedematous limbs.
In 1995, I was diagnosed with mixed b-cell lymphoma, which originated in my very lymphedematous left leg. There was nothing out there to read, study or to help me understand exactly what was going on.
I gradually found a couple short articles on lymphoma and lymphedema reported on by a Spanish doctor. Also, I finally found my way to Dr. Lerner, who brought decongestive therapy to the Americas. He was then practicing in New York and was a tremendous help to me. He truly represents the finest qualities of the medical profession.
The medical literature now is full of new articles delineating secondary cancers and in my groups there have been a surprising number of us who have experienced them.
An angiosarcoma (AS) is an uncommon malignant neoplasms characterized by rapidly proliferating, extensively infiltrating anaplastic cells derived from blood vessels and lining irregular blood-filled spaces. Specialists apply the term angiosarcoma to a wide range of malignant endothelial vascular neoplasms that affect a variety of sites. The most common locations are the head and neck, the skin and soft tissue in general. They can also originate in the liver, breast, spleen, bone, or heart.
Angiosarcomas are aggressive and tend to recur locally, spread widely, and have a high rate of lymph node and systemic metastases. The rate of tumor-related death is high. (1)
The incidence rate for nagiosaroma in 2000-2004 was 3.1 per 100,000 in both men and women.
Because angiosarcomas are aggressive and generally multicentric they have a very high recurrence rate and metastasis. Also contributing to the mortality rate is the fact they they are often misdiagnosed.
The five year survival for soft tissue sarcoma is approximately 67%, which translates into a 1.6 per 100,000 population (2000).
Cutanous sarcoma affects males more often then females with a ratio of 2:1. This is true for most forms of angiosarcoma. While it is generally reported that the 07’s seem to be the most common decade for angiosarcomas to appear, none of us in the lymphedema online groups were that age. We range from the 40’s to the early 60’s, which is substantially lower then the population in general.
The risk factors for angiosarcoma includes:
(1) Toxic exposure or radiation therapy
(2) Other cancers
(3) Lymphedema – both primary and secondary
The features of an angiosarcoma may be based on where specifically they appear.
Features may include:
Anemia, hepatic dysfunction, bleeding, blood clots, compression of neurovascular structures that can be quite painful.
The emedicine page listed below has an extensive listing of the features of angiosarcoma in the soft tissues and angiosarcoma of the bone.
There are four variants that are generally recognized. They include:
(1) Cutaneous angiosarcoma of the scalp and face
(2) Cutaneous lymphedema associated with lymphedema. Initially, it was reported only in breast cancer survivors, but subsequently has been recognized in congenital lymphedema, idiopathic lymphedema and filarial lymphedema.
(3) Radiation induced angiosarcoma with the sarcoma appearing in the radiation field some 4 to 40 years afterwards.
(4) Epithelioid angiosarcoma. Death with this sarcoma generally occurs 2 – 3 years after presentation.
Epitelioid tissues are formed by closely apposed cells with little or no intercellular material and occur as membranes and as glands.
The epithelial cells are supported by connective tissue containing vessels and nerves but are separated from subjacent connective tissue by a basal lamina.
There are no blood vessels within epithelium itself and metabolism thus depends upon diffusion of oxygen and metabolites from blood vessels in the supporting connective tissue.
These include cystosarcoma phyllodes, hemangioblastoma, hemangiomas, hepatic; Kaposi sarcoma, metastic cancer of unknown primary site.
Imaging studies, inconjunction with a biopsy of the tumor provide the best chance of an accurate diagnosis for ngiosarcoma of the soft tissue.
Of the radiography tests, the preferred one is listed as the magnetic resonance imaging (MRI) . A ches CT scan is used also however as it too issensitive in the detection of lung, pleural and mediastinal metastisis.
For angiosarcoma of the bone, there may be four radliology studies done.
These include radiography, ct scan, MRI and bone scan.
Because of the poor results, preoperative chemotherapy is generally reserved for patients with high-grade sarcoma lesions.
The use of radiotherapy in conjunction with surgery is reported to have a local control rate of 80%. However, in terms of overall survival, irridation does not actually improve survival.
Neurovascular injury Range of motion and strength Complications from radiotherapy Wound complications Fractures
Complications from chemotherapy
Fever Dose-dependent myelosuppression Nausea and vomiting unresponsive to antiemetics Moderate-to-severe fatigue Alopecia or hemialopecia in intraarterial chemotherapy
Complications from surgery
Anesthetic complications Blood loss Infection, sepsis Wound complications Iatrogenic neurovascular injury Deep vein thrombosis, pulmonary embolism Limited limb function
Partial response of angiosarcoma of the scalp to sorafenib: association with decreased expression of vascular endothelial growth factors and their receptors.
Tamiya H, Kamo R, Kumei A, Yanagihara S, Ishii M, Kobayashi H.
Department of Dermatology, Osaka City University Graduate School of Medicine, Osaka, Japan.
The prognosis of angiosarcoma remains very poor, even with combined, multimodal therapy. We report a case with partial response of angiosarcoma of the scalp to sorafenib, which is a new oral, molecular, targeted, multiple-kinase inhibitor. In addition, we confirmed, using immunohistochemistry, that sorafenib suppressed the expression of vascular endothelial growth factors and their receptors on the angiosarcoma tumour cells, and decreased cell numbers by inhibiting cellular proliferation.
Liver angiosarcoma, a rare liver malignancy, presented with intraabdominal bleeding due to rupture- a case report.
Chien CY, Huang CC, Yeh CN, Chen HY, Wu JT, Cheung CS, Lin CL, Yen CL, Wang WY, Chiang KC.
ABSTRACT: Liver angiosarcoma is a rare disease, however it still ranks as the third of most common primary liver maligancies. The prognosis of liver angiosarcoma is very poor with almost all patients with this kind of disease die within 2 years after diagnosis. No specific symptoms and signs are closely associated with this disease. Here, we report a case presenting shock status at first due to rupture of liver angiosarcoma- induced internal bleeding. After emergent transarterial embolization (TAE), she received partial hepatectomy two weeks later. 4 months after operation, she is still with a good performance status without obvious recurrence or metastasis identified.
Post-radiation atypical vascular lesions and angiosarcoma: 11 cases.
[Article in French] Karkouche R, Carbonnelle-Puscian A, Rivet J, Fraitag S, Moulonguet I, Carlotti A, Havard S, Vérola O, Cavelier-Balloy B.
Service d'anatomie et cytologie pathologiques, université Paris-Descartes, hôpital Cochin, Assistance publique-Hôpitaux de Paris, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
Keywords : Atypical vascular lesion, Angiosarcoma, Kaposi’s sarcoma, Radiotherapy, Histology, VEGFR-3, MYC
BACKGROUND: Post-radiation atypical vascular lesions of the skin display clinical and morphological overlap with well-differentiated angiosarcomas, and correct diagnosis may be difficult.
PATIENTS AND METHODS: We studied clinical, histological and immuno-histochemical aspects (CD31, CD34, D2-40 and VEGFR-3) of eight post-radiation atypical vascular lesions comparatively with three post-radiation angiosarcomas.
RESULTS: All patients were female and received radiation therapy for breast carcinoma. On average, atypical vascular lesions occurred 4.3 years after radiation therapy and presented as small papulonodules or erythematous plaques. The clinical course after simple excision was benign. Histologically, they were relatively circumscribed lesions and showed slit-like vessels dissecting dermal collagen in all cases. On average, angiosarcomas occurred 5 years after radiation therapy and presented as more extensive lesions with a more aggressive clinical course. The lesions showed histological overlap with atypical vascular lesions, but were poorly circumscribed, with deeper invasion, cytological atypia and mitosis. Although the immuno-histochemical profiles were similar, expression of VEGFR-3 was greater in two cases of angiosarcoma.
CONCLUSION: Post-radiation atypical vascular lesions are benign lesions that display clinical, histological and immuno-phenotypic overlap with well-differentiated angiosarcoma, and diagnosis requires good clinicopathological correlation. VEGFR-3 may be useful for differential diagnosis, as well as amplification of the MYC gene.
Radiation Therapy for Angiosarcoma: The 35-year University of Florida Experience.
Scott MT, Portnow LH, Morris CG, Marcus RB Jr, Mendenhall NP, Mendenhall WM, Indelicato DJ.
*Department of Radiation Oncology, University of Florida College of Medicine, Gainesville †University of Florida Proton Therapy Institute, Jacksonville, FL.
BACKGROUND AND PURPOSE: We sought to identify prognostic factors and successful therapeutic approaches when treating angiosarcoma with radiotherapy.
MATERIALS AND METHODS: From 1974 to 2009, 41 patients with angiosarcoma were treated with radiotherapy. The median patient age was 67 years. Sixteen angiosarcomas were radiation induced. Tumor sites included the head and the neck in 22 patients, breast in 14, and other sites in five. Thirty-one patients were treated with both surgery and radiotherapy (12 preoperatively and 19 postoperatively) and 10 patients were treated with radiotherapy alone. The median radiotherapy dose was 60 Gy (range, 37.5 to 76 Gy).
RESULTS: The 5-year local control and overall survival rates were 64% and 54%, respectively. Median follow-up was 3.7 years. Of the 23 patients who relapsed, 15 had a local failure. Predictors of 5-year local control were nonscalp primary location, tumor size of ≤5 cm, radiation-induced tumors, and combined-modality local therapy. Predictors of 5-year overall survival were nonscalp location and a tumor size of ≤5 cm. The patients with the best outcomes were treated with surgery and radiotherapy 3 times daily for angiosarcoma that developed after breast-conserving therapy.
CONCLUSIONS: For angiosarcomas treated with radiotherapy, outcome varies widely and is impacted by tumor site, size, and resectability. In amenable sites, aggressive treatment with resection and hyperfractionated radiotherapy may offer the best prognosis.
Successful Treatment of MMP-9-Expressing Angiosarcoma with Low-Dose Docetaxel and Bisphosphonate.
Ishibashi M, Fujimura T, Hashimoto A, Haga T, Onami K, Tsukada A, Kambayashi Y, Hidaka T, Furudate S, Shimada R,Aiba S.
Department of Dermatology, Tohoku University Graduate School of Medicine, Sendai, Japan.
We describe a 78-year-old Japanese patient with angiosarcoma on the scalp. Interestingly, immunohistochemical staining revealed this tumor as positive for matrix metalloproteinase 9 (MMP-9). After conventional therapy for angiosarcoma with surgical treatment and radiation therapy, we intravenously administered docetaxel at 40 mg/m(2) body surface area together with oral administration of 17.5 mg sodium risedronate hydrate. One and a half years after the standard treatment, there was no evidence of local recurrence or metastasis.
Primary pleural angiosarcoma as a mimicker of mesothelioma: a case report
Kao YC, Chow JM, Wang KM, Fang CL, Chu JS, Chen CL.
Department of Pathology, Wan Fang Hospital, Taipei Medical University, Taiwan.
Keywords: angiosarcoma, pleura, mesothelioma, sarcoma, pathology
Primary pleural angiosarcoma is a rare and clinically aggressive tumor. Patients usually present with chest pain, dyspnea, hemoptysis and/or cough. Radiologic studies reveal diffuse pleural thickening and pleural effusion with or without mass lesion. The clinical and radiological features both resemble those of mesothelioma, and its definite diagnosis requires careful histologic examination. However, frequent epithelioid feature and immunoreactivity to cytokeratin in primary pleural angiosarcoma further complicate the pathologic diagnosis. The use of proper immunohistochemical stains is often needed to support endothelial differentiation in the tumor cells and to exclude metastatic carcinoma and mesothelioma. We report the case of a 49-year-old male patient with primary pleural angiosarcoma, who presented with initial hemothorax, followed by a rapid progress to an inoperable status.
High grade angiosarcoma arising in fibroadenoma.
Babarović E, Zamolo G, Mustać E, Strčić M.
Department of Pathology, School of Medicine, University of Rijeka, Braće Branchetta 20, Rijeka, Croatia. firstname.lastname@example.org.
Keywords: Breast, fibroadenoma, primary angiosarcoma, mastectomy
Primary angiosarcoma of the breast is a rare tumour that account for fewer than 0.05% of all malignant mammary tumours. Angiosarcoma may have an perfidious clinical onset. Radiologic findings are often nonspecific and may appear completely normal in one-third of cases with primary angiosarcoma. The prognosis is usually poor because of the high rates of local recurrence and early development of metastases. Aggressive surgical resection is the mainstay of treatment. The role of adjuvant therapy has not yet been well established.Here we present a case of a 53 year old, postmenopausal women with primary angiosarcoma arising in fibroadenoma. To our knowledge, this is the first case described in the literature to date.
Epithelioid angiosarcoma of bone and soft tissue: a report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome.
Sept Oct 2011
Chen Y, Shen D, Sun K, Bao D, Song Q, Wang G, Chen D, Yan T, Guo W.
Key words: differential diagnosis, epithelioid angiosarcoma, histopathology, immunohistochemistry, prognosis.
Aims and background: Epithelioid angiosarcoma is a rare histopathologic variant of angiosarcoma characterized by an epithelioid morphology. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching a correct diagnosis, especially in the context of limited tissue sampling (e.g., needle core biopsy). To improve recognition of epithelioid angiosarcoma - and the spectrum of morphologic diversity associated with this rare variant - and to avoid a misdiagnosis, we describe the clinical, histopathologic, and immunohistochemical findings of cases of epithelioid angiosarcoma diagnosed at our institution.
Methods and study design: Seven cases of epithelioid angiosarcoma with appropriate pathologic material were identified from our archives. Immunohistochemistry was used to detect the expression of CD31, CD34, Factor VIII, cytokeratin, epithelial membrane antigen, vimentin, HMB45, CD1a, CD68, lysozyme, CD45, desmin, and smooth muscle actin in all cases. Follow-up information was obtained by reviewing medical records or by direct communication with family members.
Results: The lesions involved the bone (n = 4) and soft tissues (n = 3). Microscopically, all tumors had a predominantly diffuse growth pattern, with a focal nested architecture in 6 cases, which closely mimicked metastatic carcinoma. The initial biopsy was performed in 2 of 6 patients and revealed the presence of a malignant neoplasm suggestive of metastatic carcinoma. Immunohistochemically, the epithelioid endothelial cells usually showed strong reactivity for CD31 (7/7), variable or focal positive staining for CD34 (5/7), Factor VIII (4/7), cytokeratin (6/7), epithelial membrane antigen (2/7), vimentin (7/7), and CD68 (3/7). In contrast, they were negative for CD1a, HMB45, lysozyme, CD45, desmin, and smooth muscle actin. Three patients died of disease within one year of the diagnosis, 2 patients developed local recurrence or metastases, and another 2 were disease-free at this writing.
Conclusions: With any unusual epithelioid neoplasm displaying some or all of the morphologic features described above, epithelioid angiosarcoma should be included in the differential diagnosis. In such an instance, endothelial markers should be incorporated in the immunohistochemical analysis to avoid misdiagnosis, particularly with limited sampling.
Primary de novo angiosarcoma of the pleura. March 2012
Primary splenic angiosarcoma. Jan 2012
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