Angiosarcoma
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: Hemangiosarcoma; Pulmonary angiosarcoma; Vascular sarcoma
Overview
Angiosarcoma is a rare malignant vascular neoplasm of endothelial-type cells that line vessel walls.[1] The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells. Common angiosarcoma locations, include: kidney, liver, lung, breast, and liver. The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, the mutation results in aberrant angiogenesis.
Historical Perspective
- Angiosarcoma was first discovered by Rosai and colleagues, in 1976.[2]
Classification
- Angiosarcoma may be classified according to staging into 4 subtypes:
Pathophysiology
- The pathogenesis of angiosarcoma is characterized by a rapid and extensively infiltrating overgrowth of vascular epithelial cells.
- Angiosarcoma is a locally aggressive tumor with a high rate of lymph node infiltration and metastases.
- Common angiosarcoma locations, include: kidney, liver, lung, breast, and liver.
- The PTPRB /PLCG1 genes have been associated with the development of angiosarcoma, as a result generate aberrant angiogenesis.
- On gross pathology, characteristic findings of angiosarcoma, may include:
- Red/dark tan lesion
- Typically poorly circumscribed
- On microscopic histopathological analysis, characteristic findings of angiosarcoma, may include:
- Spindle cell lesion.
- Occasionally an epitheloid lesion
- Very many small capillaries of irregular shape
- Pleomorphic nuclei
- May have hobnail morphology
- Usually "red" at low power (key feature)
- Mitoses
- Cytoplasmic vacuoles
- Cells trying to form lumina
Causes
- Common causes of angiosarcoma may include:
Differentiating Angiosarcoma from Other Diseases
- Angiosarcoma must be differentiated from other diseases that cause a highly vascular mass, such as:
- Atypical vascular lesions
- Hemangioma
- Glomangiosarcoma
- Carotid body tumor
- Malignant fibrous histiocytoma of soft tissue
Epidemiology and Demographics
- Angiosarcoma is uncommon.
- In 2004, the age-adjusted incidence of angiosarcoma was 3.1 per 100,000 population per year.
Age
- Angiosarcoma is more commonly observed among patients aged [age range] years old.
- Angiosarcoma is more commonly observed among middle aged adults.
Gender
- Males are more commonly affected with angiosarcoma than females.
- The male to female ratio is 2:1.
Race
- There is no racial predilection for angiosarcoma.
Risk Factors
- Common risk factors in the development of angiosarcoma, include:
- Chronic lymphedema
- Polyvinyl chloride (PVC)
- Radiation
- Sun exposure
- Thorotrast
Natural History, Complications and Prognosis
- The majority of patients with angiosarcoma remain asymptomatic for years.
- Early clinical features may include unspecific general symptoms, such as: pain, fatigue, malaise, and nausea.
- If left untreated, the majority of patients with angiosarcoma may progress to develop metastases.
- Common complications of angiosarcoma, include:
- Pathologic fractures
- Anemia
- Hepatic dysfunction
- Prognosis is generally poor and the 5-year survival rate of patients with angiosarcoma is approximately 12-33%.
- Factors related with worse prognosis, include: patient age (> 65), retroperitoneum location, and large tumor size.
Diagnosis
Symptoms
- Angiosarcoma is usually asymptomatic and found incidentally.
- There are no remarkable symptoms for angiosarcoma.
Physical Examination
- Patients with angiosarcoma usually appear cachectic, or normal.
- There are no remarkable physical examination findings for patients with angiosarcoma.
Laboratory Findings
- There are no specific laboratory findings associated with angiosarcoma.
Imaging Findings
- The imaging modality of choice for angiosarcoma will depend on the location.
- For pulmonary angiosarcoma, the imaging modality of choice is enhanced CT scan.
- For other types angiosarcoma, the imaging modality of choice is MRI.
- On CT, findings of angiosarcoma may include:
Treatment
Medical Therapy
- The mainstay of therapy for angiosarcoma is a doxorubicin-based regimen.
- For angiosarcoma, doxorubicin monotherapy is indicated as first-line therapy
- For patients with pulmonary angiosarcoma, combination of radiotherapy and immunotherapy with recombinant interleukin-2 is the treatment of choice.[3]
- Common complications of doxorubicin, include:
- Chronic cardiotoxicity
- Mucositis
- Alopecia
- Nausea
- Vomiting
Surgery
- Surgery is the mainstay of therapy for angiosarcoma.
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of angiosarcoma.
- [Surgical procedure] can only be performed for patients with [disease stage] angiosarcoma.
Prevention
- There are no primary preventive measures available for angiosarcoma.
- Once diagnosed and successfully treated, patients with angiosarcoma are followed-up every 3, 6, or 12 months depending on the stage at diagnosis.
- Follow-up testing for angiosarcoma, include:
- Periodical imaging/angiography evaluation
- Laboratory testing: complete blood count (eg. anemia)
References
- ↑ Perkins, [edited by] Vinay Kumar, Abul K. Abbas, Jon C. Aster ; artist, James A. (2013). Robbins basic pathology (9th ed. ed.). Philadelphia, PA: Elsevier/Saunders. ISBN 9781437717815.
- ↑ Barber W, Scriven P, Turner D, Hughes D, Wyld D (2010). "Epithelioid angiosarcoma: Use of angiographic embolisation and radiotherapy to control recurrent haemorrhage". J Surg Case Rep. 2010 (5): 7. doi:10.1093/jscr/2010.5.7. PMC 3649120. PMID 24946325.
- ↑ Duck L, Baurain JF, Machiels JP (2004). "Treatment of a primary pulmonary angiosarcoma". Chest. 126 (1): 317–8, author reply 318. doi:10.1378/chest.126.1.317. PMID 15249484.