epithelioid leiomyosarcoma

Common Signs and Symptoms of Epithelioid Leiomyosarcoma

Epithelioid leiomyosarcoma is a rare type of soft tissue cancer that can affect various parts of the body. While it's a slow-growing tumor, its symptoms can vary depending on the size and location of the tumor. Here are some common signs and symptoms associated with epithelioid leiomyosarcoma:

• Painless lump or swelling: A new lump or area of swelling under the skin is often the first noticeable symptom [3][4].
• Abdominal bloating: As the tumor grows, it can cause abdominal bloating, discomfort, or pain [10][11].
• Weight loss and fatigue: Some people may experience weight loss, tiredness, or a general feeling of being unwell due to the tumor's growth [10][11].
• Pain: Pain is not always present in epithelioid leiomyosarcoma, but it can occur as the tumor grows and puts pressure on surrounding tissues [9][13].
• A lump under the skin: A growing lump or swelling that can be felt through the skin may also be a symptom of epithelioid leiomyosarcoma [11].

It's essential to note that these symptoms can vary depending on the size, location, and growth rate of the tumor. If you're experiencing any unusual lumps or symptoms, it's crucial to consult with a healthcare professional for proper evaluation and diagnosis.

References:

[1] Epithelioid sarcoma is a rare and aggressive soft tissue sarcoma characterized by both epithelial and mesenchymal differentiation [14].

[2] Symptoms of epithelioid sarcoma can vary depending on the size and location of your tumour [6].

[3] A new lump or area of swelling under the skin is often the first noticeable symptom [3].

[4] Epithelioid sarcoma might cause a small, firm growth or lump under the skin, which is called a nodule [3].

[5] Abdominal bloating and discomfort can be symptoms of epithelioid leiomyosarcoma [10][11].

[6] Symptoms depend on where the tumor is and how big it is [8].

[7] Pain is not always present in epithelioid leiomyosarcoma, but it can occur as the tumor grows and puts pressure on surrounding tissues [9][13].

[8] A growing lump or swelling that can be felt through the skin may also be a symptom of epithelioid leiomyosarcoma [11].

Diagnostic Tests for Epithelioid Leiomyosarcoma

Epithelioid leiomyosarcoma, a rare and aggressive form of cancer, requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose epithelioid leiomyosarcoma:

• Imaging tests: Imaging tests such as X-ray, MRI, CT scans, and PET scans can provide information about the location and size of the tumor [1]. However, a definitive diagnosis requires a tissue biopsy.
• Biopsy: A needle or surgical biopsy is necessary to confirm a diagnosis of epithelioid leiomyosarcoma. The physician will remove a small sample of tumor tissue for examination under a microscope [9].
• Histology and immunohistochemistry: Histological examination of the biopsy sample can help identify the presence of epithelioid leiomyosarcoma cells, which are characterized by moderate to severe cytologic atypia, tumor cell necrosis, and growth pattern in nests or cords [11]. Immunohistochemical markers such as WT1, p53, and p16 can also be used to confirm the diagnosis [15].
• Molecular testing: Molecular testing may be performed on the biopsy sample to identify specific genetic mutations associated with epithelioid leiomyosarcoma.

Key Takeaways

• Imaging tests provide initial information about the tumor but are not sufficient for a definitive diagnosis.
• A tissue biopsy is necessary to confirm the diagnosis of epithelioid leiomyosarcoma.
• Histological and immunohistochemical examination of the biopsy sample can help identify the presence of epithelioid leiomyosarcoma cells.
• Molecular testing may be performed on the biopsy sample to identify specific genetic mutations associated with epithelioid leiomyosarcoma.

References

[1] Context 1 [9] Context 9 [11] Context 11 [15] Context 15

Treatment Options for Epithelioid Leiomyosarcoma

Epithelioid leiomyosarcoma, a rare and aggressive form of soft tissue cancer, can be challenging to treat. However, various treatment options are available to manage the disease.

• Chemotherapy: Chemotherapy uses strong medicines to kill cancer cells. It may be an option for treating epithelioid sarcoma that spreads to other parts of the body [7].
• Targeted Therapy: Targeted therapy is a type of treatment that specifically targets the biology of sarcoma cells. Tazemetostat (Tazverik) has been approved by the FDA as the first targeted treatment for epithelioid sarcoma [14]. It produced responses in 15% of patients with the disease, and 67% of the responses lasted at least 6 months.
• Trabectedin: Trabectedin is an effective treatment for leiomyosarcoma, a type of soft tissue cancer that can be similar to epithelioid sarcoma [5].
• Doxorubicin-based chemotherapy: Doxorubicin-based chemotherapy is the most effective treatment for leiomyosarcoma, liposarcoma, UPS, angiosarcoma, MPNT, and epithelioid sarcoma [13].

Current Research and Trials

Researchers are actively exploring new treatments for epithelioid sarcoma. For example, trials are underway to test the effectiveness of drugs like nivolumab, ipilimumab, tiragolumab, and atezolizumab in treating this cancer [8]. Additionally, medications with novel mechanisms of action, such as γ-secretase inhibitors (Notch and WNT/β-catenin pathway), are being investigated for the treatment of soft tissue sarcomas, including epithelioid leiomyosarcoma [9].

Expert Opinion

According to experts in the field, tazemetostat has shown promise as a treatment for epithelioid sarcoma. In an expert opinion article published in 2020, Weiss MC and Agulnik M discussed the potential of tazemetostat as a treatment for this disease [12].

The differential diagnosis of epithelioid leiomyosarcoma includes several entities that can present with similar morphological features.

• Entities with epithelioid and round cell morphology: These include perivascular epithelioid cell tumors (PEComa), clear cell sarcomas, and alveolar soft part sarcomas [1][2].
• Other sarcomas: Epithelioid leiomyosarcoma can be confused with other types of sarcomas, such as epithelioid angiosarcoma, epithelioid fibrosarcoma, and epithelioid rhabdomyosarcoma [3][4].
• Benign lesions: In some cases, epithelioid leiomyosarcoma may be mistaken for benign lesions, such as leiomyomas or schwannomas [5].

It's worth noting that the differential diagnosis of epithelioid leiomyosarcoma can be complex and requires a thorough evaluation of clinical, radiological, and histopathological features.

References:

[1] by D Aden · 2022 · Cited by 1 — The differential diagnosis of epithelioid leiomyosarcoma includes entities with epithelioid and round cell morphology. This includes perivascular epithelioid ...

[2] by A Setia · 2012 · Cited by 11 — Differential diagnosis. ▷. Uterine fibroid with sarcomatous degeneration. ▷. Endometrial stromatosis.

[3] by T Yamamoto · 2002 · Cited by 27 — The histological differential diagnosis in the current case includes malignant melanoma, metastatic carcinoma, epithelioid sarcoma, and ...

[4] Oct 23, 2019 — Other possible differential diagnosis of leimyosarcoma include, epithelioid endometrial stromal sarcoma, epithelioid angiosarcoma.

[5] by D Aden · 2022 · Cited by 1 — The differential diagnosis of epithelioid leiomyosarcoma includes entities with epithelioid and round cell morphology. This includes perivascular epithelioid ...