Sarcoma Awareness Month is held annually in July, and in Spain, 13 July is also celebrated as National Sarcoma Day. This is a time when the global sarcoma community comes together with a unified mission: to raise public awareness of this type of cancer, its causes, risk factors, symptoms and possible treatments, as well as the unique challenges faced by patients.
WHAT IS SARCOMA?
Sarcoma is a rare type of cancer that originates in soft tissues, such as muscles, tendons, fatty tissues, nerves, blood and lymphatic vessels, or in the bones. It can also affect other organs, such as the lungs, digestive system, or reproductive system. It can occur in both children and adults and accounts for 1% of all malignant tumours.
TYPES OF SARCOMA
This type of cancer has more than 150 varieties and is formed by changes or mutations in DNA that occur inside cells. Some of the subtypes include liposarcoma (which originates in fatty tissues), and angiosarcoma (which originates in blood and lymphatic vessels).
WHAT IS ANGIOSARCOMA?
Angiosarcoma is a rare subtype of soft tissue sarcoma of vascular or lymphatic endothelial cell origin. Although angiosarcoma can occur in any organ, it usually arises in the following clinical settings: 1. face and scalp, 2. lymphedematous regions (associated with lymphedema - lymphangiosarcoma), and 3. previously irradiated skin (post-irradiation angiosarcoma). Read more about angiosarcoma in chronic lymphedema HERE.
WHAT IS LYMPHANGIOSARCOMA?
Lymphangiosarcoma is a rare, rapidly progressive cutaneous angiosarcoma arising from chronic lymphedema that usually develops in atrophic, pachydermatous, hyperkeratotic skin of the extremities affected by long-term chronic lymphatic blockage (Lymphedema).
WHAT IS LYMPHEDEMA?
Lymphedema (Organic Lymphatic Vascular Disease) is due to impaired lymphatic drainage resulting in skin disease, characterized by persistent and progressive swelling, and abnormal thickening of the skin and subcutaneous connective tissue. Lymphedema is not just a localized swelling problem, but a condition that can affect multiple body systems, structures and functions, leading to a range of physical, physiological and psychosocial symptoms and complications. Read about the symptoms and complications of lymphedema HERE.
IMPORTANCE OF DIAGNOSIS AND EARLY INTERVENTION
While it´s not always possible to prevent lymphangiosarcoma, which is a type of cancer associated with chronic lymphedema, strategies to manage and prevent lymphedema itself can significantly reduce the risk of developing this cancer.
The diagnosis of sarcoma is based on a combination of medical tests and studies, which may include: 1) Clinical history and physical examination; 2) Imaging tests; and 3) Biopsy. Once the diagnosis of sarcoma has been confirmed, the stage of the disease and the presence or absence of metastases are determined. Treatment of sarcoma may include a combination of surgery, radiotherapy, and chemotherapy, depending on the type and stage of sarcoma.
Regardless of the type of sarcoma, treatment must be multidisciplinary, and the pathology needs to be promptly assessed by medical experts in different areas in order to decide on the best treatment.
REFERENCES:
Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition?
Lymph Leakage Promotes Immunosuppression by Enhancing Anti-Inflammatory Macrophage Polarization
Transformación maligna de linfedema crónico en miembro inferior en linfangiosarcoma
Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020
Chronic oedema/lymphoedema: under‐recognised and under‐treated
