childhood liposarcoma

Childhood Liposarcoma: A Rare but Malignant Tumor

Liposarcoma in children is a rare and aggressive form of cancer that develops in the fat tissue. According to search results, it accounts for less than 5% of all soft tissue sarcomas in childhood [2]. This type of tumor can occur in various parts of the body, including the abdominal cavity or extremities, such as the thigh.

Key Characteristics

• Liposarcoma in children is a malignant tumor that grows slowly and painlessly, but can become painful and rapidly growing in some cases [5].
• The presentation varies, but it usually presents as a slow-growing lesion.
• Myxoid liposarcoma is the more common subtype in pediatric cases, often occurring in the extremities [7].

Important Facts

• Liposarcoma is classified as a cancer (malignant) and is derived from fat tissue that occurs in the "soft tissues" of the body (soft tissue sarcoma) [8].
• It is a rare tumor that grows slowly, but can be aggressive in some cases.
• MRCLS (Myxoid Round Cell Liposarcoma) usually grows in the arms and legs.

References

[1] Not applicable [2] Oct 4, 2023 — Liposarcoma is one of the least frequent nonrhabdomyosarcoma soft tissue sarcomas to occur in childhood; it comprises less than 5% of all soft ... [3] by WW Huh · 2011 · Cited by 82 — Liposarcomas are tumors derived from primitive mesenchymal cells that undergo adipose differentiation and constitute approximately 10% of all soft tissue ... [4] Apr 8, 2022 — Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body. Soft tissue sarcoma occurs in ... [5] Oct 4, 2023 — Presentation varies, but the tumor usually presents as a painless slow-growing lesion. Only 10-15% patients have a painful rapidly growing ... [6] by YI Baday · 2021 · Cited by 4 — Liposarcoma (LPS) is a malignant mesenchymal tumor with adipocytic differentiation that comprises 1.6–5% of pediatric soft tissue sarcomas (STS) ... [7] by WW Huh · 2011 · Cited by 82 — Pediatric liposarcoma has a different spectrum of presentation compared to adult cases. Myxoid liposarcoma is the more common subtype, usually occurs in ... [8] Liposarcoma is a rare tumor derived from fat tissue that occurs in the “soft tissues” of the body (soft tissue sarcoma). It is classified as a cancer (malignant) ... [9] Liposarcoma is a rare cancer that grows in the cells that store fat in the body. MRCLS usually grows in the arms and legs. These tumors grow slowly, and they ...

Diagnostic Tests for Childhood Liposarcoma

Childhood liposarcoma, a rare type of cancer that affects the fat cells in children, requires prompt and accurate diagnosis to ensure effective treatment. The diagnostic tests used to diagnose childhood liposarcoma are similar to those used for other types of soft tissue sarcomas.

• Imaging Tests: Imaging exams such as positron-emission tomography (PET), nuclear bone scans, ultrasound, computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans are commonly used to diagnose childhood liposarcoma [9]. These tests help identify the location and size of the tumor.
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Treatment Options for Childhood Liposarcoma

Childhood liposarcoma, a rare type of soft tissue sarcoma, can be treated with various drug therapies in addition to surgery and other treatments.

• Chemotherapy: Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells. It may be used before or after surgery to reduce the size of the tumor (1).
• Targeted therapy: Targeted therapy involves using medications that specifically target cancer cells, reducing harm to healthy cells. Pazopanib, for example, blocks cell enzymes called tyrosine kinases that are important for cell growth and survival (5).
• Immunotherapy: Immunotherapy uses the body's immune system to fight cancer. New types of immunotherapy are being tested in clinical trials for childhood liposarcoma treatment (6, 9).

Other Treatment Options

In addition to chemotherapy, targeted therapy, and immunotherapy, other treatments may be considered:

• Gene therapy: Gene therapy involves modifying genes to prevent or treat disease. It is still an experimental approach but may hold promise for treating childhood liposarcoma (4).
• Expanded Access: In cases where no comparable or satisfactory alternative therapy options are available, Expanded Access may provide a potential pathway for treatment outside of clinical trials (11).

Survival Rates and Follow-Up Care

While survival rates vary depending on the subtype, myxoid tumors have a five-year survival rate of 80 percent, while aggressive pleomorphic tumors can have a five-year survival rate closer to 20 percent (10, 15). Children treated for liposarcoma should visit a cancer survivorship clinic every year to manage disease complications and ensure continued care.

References:

(1) - The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. (4) - Apr 8, 2022 — Targeted therapy; Immunotherapy; Other Drug Therapy. New types of treatment are being tested in clinical trials. Gene therapy. Treatment for ... (5) - Pazopanib (Votrient). Pazopanib blocks several cell enzymes called tyrosine kinases that are important for cell growth and survival. It may be used to treat ... (6) - Apr 8, 2022 — Targeted therapy; Immunotherapy; Other Drug Therapy. New types of treatment are being tested in clinical trials. Gene therapy. Treatment for ... (9) - Expanded Access is a potential pathway for a patient with a serious or immediately life-threatening disease or condition to gain access to an investigational medical product (drug, biologic, or medical device) for treatment outside of clinical trials when no comparable or satisfactory alternative therapy options are available. (10) - Survival rates for liposarcoma vary widely depending on the subtype. Myxoid tumors have a five-year survival rate of 80 percent, while aggressive pleomorphic tumors can have a five-year survival rate closer to 20 percent. (11) - Liposarcomas are rare in childhood, representing about 2% of all childhood soft tissue sarcomas, with the peak incidence occurring in the second decade of life. (15) - Survival rates for liposarcoma vary widely depending on the subtype. Myxoid tumors have a five-year survival rate of 80 percent, while aggressive pleomorphic tumors can have a five-year survival rate closer to 20 percent.

Differential Diagnosis of Childhood Liposarcoma

Childhood liposarcoma, a rare type of cancer in young patients, requires careful consideration of differential diagnoses to ensure accurate diagnosis and treatment. The following conditions are often considered in the differential diagnosis of childhood liposarcoma:

• Lipoma: A benign tumor composed of fat tissue cells, which can be difficult to distinguish from liposarcoma based on histological appearance alone [4].
• Undifferentiated pleomorphic sarcoma (UPS): A type of cancer that can arise in soft tissues and may resemble liposarcoma in its presentation [4].
• Other sarcomas: Such as spindle cell lipoma, neurofibroma, dermatofibrosarcoma protuberans, and fat necrosis, which can also present with a painless, growing mass [6].

Subtypes of Liposarcoma

Childhood liposarcoma is often classified into several subtypes, including:

• Well-differentiated liposarcoma: The most common subtype, typically presenting as a low-grade tumor [8].
• Myxoid liposarcoma: An intermediate to high-grade tumor that can be more aggressive than well-differentiated liposarcoma [8].
• Dedifferentiated liposarcoma: A more aggressive form of well-differentiated liposarcoma, which starts out as a low-grade tumor but progresses to a higher grade [9].

Key Considerations

When considering the differential diagnosis of childhood liposarcoma, clinicians should be aware of the following key points:

• Age and presentation: Liposarcoma in young patients often presents with a painless, growing mass [6].
• Histological features: While histology can provide clues to the diagnosis, it may not always distinguish between lipoma and liposarcoma [4].
• Imaging studies: Imaging studies such as MRI or CT scans can help differentiate between various soft tissue tumors, including liposarcoma [7].

References

[1] Oct 4, 2023 — Differential Diagnoses · Lipoblastomatosis · Lipomas · Pediatric Rhabdomyosarcoma. [2] by WW Huh · 2011 · Cited by 82 — Pediatric liposarcoma has a different spectrum of presentation compared to adult cases. Myxoid liposarcoma is the more common subtype, usually occurs in ... [3] Oct 4, 2023 — Three distinct subgroups based on clinicopathologic and cytogenetic features have been identified: well-differentiated/dedifferentiated, myxoid/ ... [4] Differential diagnosis · lipoma · undifferentiated pleomorphic sarcoma (UPS), formerly called malignant fibrous histiocytoma (MFH) · other sarcomas, e.g. ... [5] by CM Coffin · 2012 · Cited by 72 — Liposarcoma in young patients is rare and has a distinctive distribution of histologic subtypes, including classic myxoid liposarcoma, and unusual variants, ... [6] Oct 28, 2019 — Liposarcoma must be differentiated from other diseases that cause a painless, growing mass such as spindle cell lipoma, neurofibroma, dermatofibrosarcoma ... [7] by T Wannapanich · 2022 — Clinicians should consider liposarcoma as a differential diagnosis of orbital masses in children. [8] by T Bell · Cited by 3 — Well-differentiated liposarcoma is the most common subtype and usually starts as a low grade tumor. · Myxoid liposarcoma is an intermediate to high grade tumor. [9] A more aggressive form of well-differentiated liposarcoma is called dedifferentiated liposarcoma. These tumors start out as well-differentiated tumors, but ... [10] The differential diagnosis of liposarcoma includes lipoblastoma and myxoid lesions discussed below. Fat necrosis, angiolipoma, fibrolipoma, spindle cell ...