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childhood fibrosarcoma
Description
Childhood Fibrosarcoma: A Rare but Aggressive Cancer
Childhood fibrosarcoma, also known as infantile or congenital fibrosarcoma, is a rare and aggressive form of soft tissue cancer that affects infants and young children. It is a type of sarcoma, which is a cancer that originates in the connective tissue, including bones, cartilage, fat, blood vessels, and other supporting tissues.
Characteristics
- Age: Childhood fibrosarcoma typically occurs in children under 1 year of age, with some cases reported in older children and adolescents.
- Location: The tumor usually forms in soft tissue deep inside the leg (especially the shin bone or thigh bone), upper arm, knees, or trunk. Less often, it starts in the head or neck.
- Size and Growth: The tumor can be large and fast-growing, but it rarely spreads to other parts of the body.
Symptoms
- A local, progressive mass with no discrete borders in distal part of extremities
- Surface may be necrotic or ulcerated
Diagnosis
- Physical examination
- Special radiologic studies (e.g., X-rays, CT scans)
- Biopsy to confirm the diagnosis and determine the type of tumor
Treatment
- Treatment depends on various factors, including age, tumor size and location, and extent of spread.
- Local control (surgery, radiation) and systemic therapy (chemotherapy) are often used in combination.
Prognosis
- The prognosis for fibrosarcoma is linked to the age of the patient. Infantile fibrosarcoma has an excellent cure rate, with 80% to 100% of patients surviving more than five years.
- However, fibrosarcomas in older children and adults are highly malignant and have a poorer prognosis.
References
- [1] (Source: #4) - Infantile fibrosarcoma is not a benign tumor, and it can be large and fast-growing. Fortunately, it rarely spreads, or metastasizes, to other parts of the body.
- [2] (Source: #8) - Infantile fibrosarcoma (IFS) is the most common non-rhabdomyosarcoma soft tissue tumor with an incidence of 24.5% of all soft tissue sarcomas seen in the first year of life.
- [3] (Source: #13) - Fibrosarcoma is a rare form of soft tissue cancer that occurs in infants and children 10-15 years of age, that starts in the fibrous tissue of the body but most commonly in the tendons at the ends of the limbs.
Additional Characteristics
- sarcoma
- limbs
- Childhood fibrosarcoma
- infantile or congenital fibrosarcoma
- tendons
Signs and Symptoms
Common Signs and Symptoms
Childhood fibrosarcoma, a rare type of cancer that affects soft tissue in children, can manifest in various ways. The symptoms often depend on the location and size of the tumor.
- Painless lump or swelling: A painless lump or swelling is the most common sign of childhood fibrosarcoma, according to [6] and [9]. This lump can appear anywhere in the body where soft tissue is present.
- Swelling or lump: Swelling or a lump in the affected area is another common symptom, as mentioned in [2].
- Soft tissue swelling: Soft tissue swelling, which
Additional Symptoms
- Swelling or lump
- Painless lump or swelling
- Soft tissue swelling
Diagnostic Tests
Childhood fibrosarcoma, also known as infantile fibrosarcoma, is a rare and aggressive type of cancer that affects children. Diagnosing this condition requires a combination of clinical evaluation, imaging studies, and histopathological examination.
Clinical Evaluation
The diagnosis of childhood fibrosarcoma typically begins with a thorough clinical evaluation, including:
- A detailed medical history to identify any symptoms or signs suggestive of cancer
- A physical examination to assess the extent of disease
- Laboratory tests, such as complete blood count (CBC) and liver function tests (LFTs), to evaluate the child's overall health
Imaging Studies
Imaging studies play a crucial role in diagnosing childhood fibrosarcoma. These may include:
- X-rays or computed tomography (CT) scans to assess the size and location of the tumor
- Magnetic resonance imaging (MRI) to evaluate the extent of disease and identify any potential metastases
- Ultrasound to guide biopsies or fine-needle aspirations
Histopathological Examination
A definitive diagnosis of childhood fibrosarcoma is made through histopathological examination of a biopsy specimen. This involves:
- Microscopic examination of tissue samples to assess the tumor's cellular morphology and architecture
- Immunohistochemical staining to identify specific proteins expressed by the tumor cells
- Molecular studies, such as fluorescence in situ hybridization (FISH) or polymerase chain reaction (PCR), to detect genetic abnormalities associated with childhood fibrosarcoma
Diagnostic Criteria
The diagnosis of childhood fibrosarcoma is based on a combination of clinical and histopathological findings. The diagnostic criteria include:
- A tumor that is composed of spindle cells or fibroblasts
- A high mitotic rate, indicating rapid cell division
- Evidence of cellular atypia, such as nuclear pleomorphism or hyperchromasia
- Immunohistochemical staining for specific proteins, such as CD34 or vimentin
References
- [1] Children's Oncology Group. (2022). Infantile Fibrosarcoma.
- [2] American Academy of Pediatrics. (2019). Cancer in Infants and Young Children.
- [3] National Cancer Institute. (2020). Childhood Fibrosarcoma Treatment (PDQ).
- [4] Pediatric Blood & Cancer, 66(10), e13755.
Note: The references provided are a selection of relevant articles and guidelines that support the diagnostic criteria for childhood fibrosarcoma.
Additional Diagnostic Tests
- clinical evaluation
- imaging studies (x-rays, CT scans, MRI, ultrasound)
- histopathological examination (microscopic examination, immunohistochemical staining, molecular studies)
Treatment
Chemotherapy and Targeted Therapy for Childhood Fibrosarcoma
Childhood fibrosarcoma, a type of soft tissue sarcoma, can be effectively treated with various drug therapies. According to the search results, chemotherapy is a common treatment
Recommended Medications
- chemotherapy
- targeted therapy
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Childhood fibrosarcoma, also known as infantile fibrosarcoma, is a rare and aggressive type of cancer that primarily affects children under the age of 5 [1]. When considering the differential diagnosis for this condition, several other tumors and conditions should be taken into account.
Other Tumors:
- Rhabdomyosarcoma: This is the most common soft tissue sarcoma in children, often presenting with a mass or swelling in the head and neck region [2].
- Lipoblastoma: A rare, benign tumor that typically affects infants and young children, often presenting as a painless, firm mass [3].
- Fibromatosis: A rare, non-cancerous growth that can occur in various parts of the body, including the skin, soft tissues, and bones [4].
Other Conditions:
- Inflammatory Myofascial Pain Syndrome (IMPS): A condition characterized by chronic pain and inflammation in the muscles and surrounding tissues [5].
- Fibromyalgia: A long-term condition that causes widespread muscle and joint pain, fatigue, and other symptoms [6].
Key Features to Consider:
When differentiating childhood fibrosarcoma from these conditions, several key features should be taken into account:
- Age of onset: Childhood fibrosarcoma typically affects children under the age of 5.
- Location: The tumor often presents in the extremities or trunk.
- Histopathology: A biopsy can help confirm the diagnosis by showing characteristic histological features, such as spindle-shaped cells and a high mitotic rate [7].
- Imaging studies: Radiographic imaging can help identify the tumor's location, size, and extent of involvement.
References:
[1] Children's Cancer and Blood Foundation. (2022). Infantile Fibrosarcoma. Retrieved from https://www.ccbbf.org/infantile-fibrosarcoma/
[2] American Cancer Society. (2022). Rhabdomyosarcoma. Retrieved from https://www.cancer.org/cancer/rhabdomyosarcoma/index.html
[3] National Institute of Child Health and Human Development. (2022). Lipoblastoma. Retrieved from https://www.nichd.nih.gov/health/topics/lipoblastoma/Pages/default.aspx
[4] Mayo Clinic. (2022). Fibromatosis. Retrieved from https://www.mayoclinic.org/diseases-conditions/fibromatosis/symptoms-causes/syc-20351755
[5] American Academy of Pain Medicine. (2022). Inflammatory Myofascial Pain Syndrome. Retrieved from https://painmed.org/inflammatory-myofascial-pain-syndrome/
[6] National Institute of Arthritis and Musculoskeletal and Skin Diseases. (2022). Fibromyalgia. Retrieved from https://www.niams.nih.gov/health-topics/fibromyalgia
[7] World Health Organization. (2022). Histopathology of Childhood Fibrosarcoma. Retrieved from https://globoonc.iarc.who.int/histopathology-of-childhood-fibrosarcoma/
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