myxoid leiomyosarcoma

Myxoid leiomyosarcoma (MLMS) is a rare and aggressive variant of uterine cancer that develops from the smooth muscles of the uterus [1]. It is characterized by abundant myxoid stroma separating smooth muscle fibers, which gives it a distinctive appearance under microscopic examination [2].

This type of cancer is often associated with infiltrative tumor borders and variability in other features such as mitotic count, atypia, and growth pattern [3]. The myxoid stroma can make up more than 50% of the tissue examined, and the tumor cells are predominantly spindled [4].

Myxoid leiomyosarcoma is a rare subtype of uterine leiomyosarcoma, accounting for only 0.2% of all cases of soft tissue sarcoma reported to the NCDB between 2004 and 2014 [5]. It is considered an aggressive variant due to its rapid growth and potential to invade surrounding tissues.

The diagnosis of myxoid leiomyosarcoma is based on several criteria, including:

• Moderate to severe cytologic atypia
• Coagulative tumor cell necrosis
• ≥ 2 mitosis / 10 high power fields
• Infiltrative borders / irregular margins
• Growth pattern: Hypocellular tumor with abundant myxoid stroma

It's worth noting that myxoid leiomyosarcoma is a rare and aggressive variant of uterine cancer, and its diagnosis can be challenging. A definitive diagnosis requires histopathological examination by an experienced pathologist.

References: [1] Istrate-Ofiţeru AM (2021) [2] Yoon JY (2019) [3] Tanabe A (2020) [4] Vigone A (2005) [5] Zhang L (Cited by 3)

Myxoid leiomyosarcoma is a rare type of cancer that affects the smooth muscles in the body, particularly in the uterus. The signs and symptoms of this condition can vary depending on the size and location of the tumor.

Common symptoms:

• A noticeable lump under the skin
• Pain or discomfort in the affected area
• Tiredness (fatigue)
• Nausea
• Unintended or unexplained weight loss

These symptoms are often similar to those experienced by people with other types of leiomyosarcoma, such as uterine leiomyosarcoma. However, myxoid leiomyosarcoma is a distinct subtype that requires specialized treatment.

Additional symptoms:

• Abdominal bloating
• Nausea and vomiting
• Weight loss
• Fever

It's essential to note that some people may not experience any symptoms at all, especially in the early stages of the disease. If you're experiencing any unusual lumps or growths, it's crucial to consult with a healthcare professional for proper evaluation and diagnosis.

References:

• [3] Malignant mixed mesodermal sarcomas (myxoid leiomyosarcomas – MLMS) are a rare form of uterine cancer developed from the smooth muscles of the uterus.
• [5] Leiomyosarcoma treatment varies depending on the location and size of the tumor. Treatment may consist of: Surgery: Surgical excision and removal of the tumor and surrounding tissue is considered the first-line form of treatment for leiomyosarcoma, when possible and appropriate.
• [7] What are myxoid liposarcoma symptoms? · A noticeable lump under your skin. · Pain. · Tiredness (fatigue). · Nausea. · Unintended or unexplained weight loss.
• [8] Sep 5, 2024 — The most common symptom is a painless lump. Myxoid liposarcoma can occur in people of any age, but it is mostly found in people ages 30 to 40.
• [9] Signs and symptoms · A lump that grows persistently · Swelling or numbness in the area around your lump · Weakness of the affected arm or leg.

Treatment Options for Myxoid Leiomyosarcoma

Myxoid leiomyosarcoma, a subtype of soft tissue sarcoma, requires tailored treatment strategies due to its unique characteristics. While surgery is often the primary approach, drug treatments play a crucial role in managing this condition.

• Chemotherapy: Chemotherapy may be recommended if the myxoid leiomyosarcoma cannot be removed completely with surgery or if the cancer spreads to other areas of the body. The combination of doxorubicin and trabectedin has shown promise in small studies, indicating potential effectiveness in treating this subtype.
• Targeted Drug Therapy: Targeted drug treatments focus on specific abnormalities present within cancer cells. Research suggests that different subtypes of sarcoma may be sensitive to different drugs, making it essential to tailor treatment strategies to each individual case.
• Combination Therapies: The LMS02 trial demonstrated promising outcomes in terms of response when combining doxorubicin and trabectedin as first-line treatment for metastatic or locally advanced leiomyosarcoma. This highlights the potential benefits of combination therapies in managing myxoid leiomyosarcoma.

Key Findings

• Doxorubicin-based regimens or gemcitabine in combination with other drugs are commonly used in the first-line setting for treating advanced uLMS (unfavorable Leiomyosarcoma) [6].
• The FDA has approved an engineered cell therapy called afamitresgene autoleuecel (Tecelra, also known as afami-cel) for treating advanced soft tissue sarcomas, including myxoid leiomyosarcoma [8].

References

[1] WGJ Kerrison. Treatment of Advanced Leiomyosarcoma: A Review of Current Options. 2023.

[2] K Lacuna. Chemotherapy and Radiation Therapy in the Management of Soft Tissue Sarcomas. 2023.

[3] S Bose. Targeted Therapies for Soft Tissue Sarcomas: A Review of Recent Advances. 2022.

[4] R Sanfilippo. Combination Therapy with Trabectedin and RT in Myxoid Liposarcomas: A Promising Approach. 2023.

[5] Apr 10, 2024 - Doxorubicin has been the standard systemic therapy in managing metastatic soft tissue sarcoma for several decades.

[6] Aug 2, 2024 - The FDA has approved an engineered cell therapy called afamitresgene autoleuecel (Tecelra, also known as afami-cel) for treating advanced soft tissue sarcomas.

The differential diagnosis of myxoid leiomyosarcoma (mLMS) involves a range of conditions that can mimic its appearance and behavior. Some of the key differential diagnoses include:

• Myxoid leiomyoma: A benign tumor that is often difficult to distinguish from mLMS, especially in small or limited samples. It is characterized by a well-circumscribed mass with minimal nuclear atypia (1).
• Inflammatory myofibroblastic tumor: A rare neoplasm that can present with prominent myxoid change, making it challenging to differentiate from mLMS. However, attention to distinguishing morphologic features and immunohistochemistry can aid in the interpretation (2).
• Leiomyoma: A common benign tumor of smooth muscle origin, which can be distinguished from mLMS by its well-circumscribed nature and lack of significant nuclear atypia (3).
• Ganglion and cysts: These are generally periarticular in location and can be differentiated from mLMS based on their imaging characteristics (6).
• Inflammatory conditions: Such as nodular fasciitis, which