Angiosarcoma

Classification according to ICD-10
C49	Malignant growth of other connective and soft tissues - blood vessels
ICD-10 online (WHO version 2019)

Canine spleen hemangiosarcoma, surgical site

Highly differentiated angiosarcoma of the female mammary gland on mammography

The angiosarcoma is a sarcoma , d. H. a malignant tumor of the supporting and connective tissue that originates from the endothelium of vessels. It is one of the malignant vascular tumors . If the tumor originates from blood vessels , it is called a hemangiosarcoma , and if the tumor is lymphatic it is called a lymphangiosarcoma .

Occurrence

Angiosarcomas are about equally common in men and women. The skin , connective tissue , breast , liver, and spleen are most commonly affected . In humans they occur preferentially in the head and neck region. Angiosarcomas of the skin tend to occur in old age, while angiosarcomas of the soft tissue can form at any age. The extremities and the retroperitoneal space are particularly affected. In domestic dogs , hemangiosarcomas are located in about half of the cases in the spleen, almost a quarter in the right atrium , 14% in the subcutaneous tissue and 10% in the liver.

Angiosarcomas more often develop in irradiated tissue after radiation therapy . They can also occur as lymphangiosarcomas of the lymphatic vessels, almost always as a result of chronic lymphedema that can persist after breast cancer and other cancers ( Stewart-Treves syndrome ). This is where shallow changes and bleeding nodes develop, from which ulcers can develop. Diagnosis, therapy and prognosis are analogous to angiosarcomas of the blood vessels.

Hemangiosarcomas can develop through exposure to thorium dioxide ( Thorotrast ), vinyl chloride, or arsenic , for example .

Symptoms, diagnosis and therapy

Angiosarcomas of the skin first appear as reddish spots that later turn bluish, similar to bruises, and can eventually disintegrate into ulcers . Hemangiosarcomas of the liver or spleen can cause the upper abdomen to swell. Often, however, hemangiosarcomas of the internal organs - especially in animals - go unnoticed for a long time and are only detected after the tumor has ruptured, whereby the patient is already in a very critical condition.

Diagnosis is based on clinical and histological findings. Histopathologically, endothelial differentiated tumor cells can be seen, which can be detected immunohistochemically by CD31 antibodies. The microscopic picture ranges from well-differentiated, anastomosing, vessel-like structures to undifferentiated, solid tumor areas. Therapy is usually radical surgery followed by radiation and / or chemotherapy . However, the tumor boundaries are difficult to determine, making it difficult to surgically remove the cancer cells. There is currently no consensus among doctors about radical operations with extensive tissue removal to the amputation of individual limbs. To chemotherapy mainly be taxanes used.

The prognosis is usually unfavorable. The five-year survival rate depends on the degree of differentiation: grade 1 (great similarity to normal tissue) means 80% tumor-free survival after 5 years, grade 3 (hardly any similarity to normal tissue) means 10%. G3 sarcomas grow very quickly and metastasize early.

Individual evidence

↑ ^a ^b Böcker, Werner., Aguzzi, Adriano .: Pathology . 4th, completely revised Urban & Fischer, Munich 2008, ISBN 978-3-437-42382-6 .
↑ Martin Kessler, Alan S. Hammer: Canine hemangiosarcoma: diagnosis and therapy. In: Small Animal Practice. 36: 637-648 (1991).
^ Vinyl Chloride: Still a Cause for Concern (Angiosarcoma of the Liver).
↑ Brief guideline - Angiosarcoma and Kaposi's sarcoma
^ F. Fata, E. O'Reilly, D. Ilson, D. Pfister, D. Leffel: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face . In: Cancer . tape 86 , no. 10 , November 15, 1999, ISSN 0008-543X , p. 2034-2037 , PMID 10570428, ( nih.gov [accessed November 16, 2017]).
^ F. Fata, E. O'Reilly, D. Ilson, D. Pfister, D. Leffel: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face . In: Cancer . tape 86 , no. 10 , November 15, 1999, ISSN 0008-543X , p. 2034-2037 , PMID 10570428, ( nih.gov [accessed November 16, 2017]).

[:0-1] Böcker, Werner., Aguzzi, Adriano .: Pathology . 4th, completely revised Urban & Fischer, Munich 2008, ISBN 978-3-437-42382-6 .

[2] Martin Kessler, Alan S. Hammer: Canine hemangiosarcoma: diagnosis and therapy. In: Small Animal Practice. 36: 637-648 (1991).

[VC-3] Vinyl Chloride: Still a Cause for Concern (Angiosarcoma of the Liver).

[4] Brief guideline - Angiosarcoma and Kaposi's sarcoma

[5] F. Fata, E. O'Reilly, D. Ilson, D. Pfister, D. Leffel: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face . In: Cancer . tape 86 , no. 10 , November 15, 1999, ISSN 0008-543X , p. 2034-2037 , PMID 10570428, ( nih.gov [accessed November 16, 2017]).

[6] F. Fata, E. O'Reilly, D. Ilson, D. Pfister, D. Leffel: Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face . In: Cancer . tape 86 , no. 10 , November 15, 1999, ISSN 0008-543X , p. 2034-2037 , PMID 10570428, ( nih.gov [accessed November 16, 2017]).