SARCOMA – INTERNATIONAL AWARENESS MONTH 2024 - LYMPHANGIOSARCOMA - ANGIOSARCOMA IN LYMPHEDEMA - STEWART-TREVES SYNDROME - Severe Complications of Lymphedema - Primary Lymphedema - Secondary Lymphedema - Podoconiosis / Lymphatic Filariasis - Elephantiasis - Organic Lymphatic Vascular Disease - Patient Association Awareness Campaign (Spain)

 

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.

SOME FACTS

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).

IMPORTANCE OF DIAGNOSIS AND EARLY INTERVENTION

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:

• Stewart-Treves Syndrome

• Vascular sarcomas

• Lymphangiosarcoma: Is Stewart-Treves Syndrome a Preventable Condition?

• Lymph Leakage Promotes Immunosuppression by Enhancing Anti-Inflammatory Macrophage Polarization

• The immunocompromised district: a unifying concept for lymphoedematous, herpes-infected and otherwise damaged sites

• Lymphedema and subclinical lymphostasis (microlymphedema) facilitate cutaneous infection, inflammatory dermatoses, and neoplasia

• Electrochemotherapy for advanced cutaneous angiosarcoma: A European register-based cohort study from the International Network for Sharing Practices of electrochemotherapy (InspECT)

• Lymphedema-related angiogenic tumors and other malignancies

• Malignant tumors as complications of lymphedema

• Transformación maligna de linfedema crónico en miembro inferior en linfangiosarcoma

• Incidence and Presenting Characteristics of Angiosarcoma in the US, 2001-2020

• Lymphangiosarcoma after filarial infection

• Angiosarcoma in Chronic Filarial Lymphedema

• Chronic oedema/lymphoedema: under‐recognised and under‐treated

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