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obsolete embryonal childhood rhabdomyosarcoma

Description

Embyonal Childhood Rhabdomyosarcoma: An Obsolete Term

Embyonal childhood rhabdomyosarcoma, also known as pediatric Embryonal Rhabdomyosarcoma, is an obsolete term that was previously used to describe a type of cancer. According to the provided context [1][5], this term is no longer in use and has been replaced by more modern classifications.

Characteristics

Historically, embryonal childhood rhabdomyosarcoma was characterized as a type of soft tissue sarcoma that primarily affects children. It was thought to arise from primitive mesenchymal cells [7][8]. The tumor type was often associated with myoblastic differentiation and expression of skeletal muscle markers such as desmin, myogenin, and/or MYOD1 [6].

Incidence

Rhabdomyosarcoma, including the embryonal subtype, is considered a rare disease in children. According to some sources, it has an incidence of 4.5 cases per million per year [14]. However, it remains the most common soft tissue sarcoma in children.

Subtypes

The classification of rhabdomyosarcoma has evolved over time, and embryonal childhood rhabdomyosarcoma is now considered a part of the broader category of embryonal rhabdomyosarcomas. This subtype is often distinguished from alveolar rhabdomyosarcoma, which tends to occur in older children and adolescents [11].

References

  • [1] J Gartrell (2020) - Rhabdomyosarcoma: A High-Grade Malignant Neoplasm
  • [5] Obsolete embryonal childhood rhabdomyosarcoma. Term ID: DOID:3256; Synonyms. pediatric Embryonal Rhabdomyosarcoma.
  • [6] SL Cramer (2017) - Myoblastic Differentiation and Expression of Skeletal Muscle Markers
  • [7] J Gartrell (2020) - Rhabdomyosarcoma: A High-Grade Malignant Neoplasm
  • [8] Rhabdomyosarcoma (RMS) is a rare disease with an incidence in children of 4.5 cases per million per year.
  • [11] embryonal rhabdomyosarcoma (ERMS) for early childhood, which typically presents in the head, neck and genitourinary (GU) regions; and alveolar (ARMS) for the later childhood and adolescent years and is commonly located in the trunk and extremities.

Additional Characteristics

  • Soft tissue sarcoma
  • Embyonal childhood rhabdomyosarcoma
  • Pediatric Embryonal Rhabdomyosarcoma
  • Obsolete term
  • Type of cancer
  • Primitive mesenchymal cells
  • Myoblastic differentiation
  • or MYOD1
  • Rarest in children
  • 4.5 cases per million per year

Signs and Symptoms

Early Signs and Symptoms of Embryonal Rhabdomyosarcoma in Children

Embryonal rhabdomyosarcoma (ERMS) is a type of cancer that primarily affects children, particularly those under the age of 5. The symptoms of ERMS can vary depending on the location and size of the tumor. Here are some common signs and symptoms associated with embryonal rhabdomyosarcoma in children:

  • Head and Neck Area:
    • Nosebleeds or sinus congestion (due to a tumor in the nose)
    • Blurry vision or tearing, bulging, or swelling of the eyes (due to a tumor behind the eye)
    • Headache
    • Bleeding in the throat or ears
  • Urinary or Reproductive System:
    • Symptoms may include urinary tract infections, blood in the urine, or difficulty urinating
    • In girls, symptoms may include vaginal bleeding or discharge
    • In boys, symptoms may include swelling of the testicles or penis
  • General Symptoms:
    • A lump or swelling that may be painful
    • Fatigue or weakness
    • Weight loss

It's essential to note that these symptoms can also be caused by other conditions, and a proper diagnosis can only be made by a healthcare professional. If you or your child experience any of these symptoms, it is crucial to seek medical attention promptly.

References:

  • [1] Symptoms may resemble less serious conditions (Search Result 3)
  • [2] A quickly-growing tumor is what causes noticeable symptoms (Search Result 4)
  • [3] Many health issues can cause symptoms like nosebleeds, vomiting or lumps and bumps and may not be symptoms of rhabdomyosarcoma (Search Result 5)
  • [4] Symptoms depend on the location and size of the tumor (Search Result 8)

Additional Symptoms

  • * Nosebleeds or sinus congestion (due to a tumor in the nose)
  • * Blurry vision or tearing, bulging, or swelling of the eyes (due to a tumor behind the eye)
  • * Bleeding in the throat or ears
  • * Symptoms may include urinary tract infections, blood in the urine, or difficulty urinating
  • * Vaginal bleeding or discharge (in girls)
  • * Swelling of the testicles or penis (in boys)
  • * A lump or swelling that may be painful
  • * Fatigue or weakness
  • weight loss
  • headache

Diagnostic Tests

Diagnostic Tests for Obsolete Embryonal Childhood Rhabdomyosarcoma

Obtaining an accurate diagnosis is crucial in treating childhood rhabdomyosarcoma (RMS). The diagnostic process typically begins with a physical exam, followed by various tests to confirm the presence of RMS and determine its subtype.

Physical Exam A thorough medical history and physical examination are essential in identifying potential symptoms of RMS. The doctor will look for any unusual lumps or swellings, particularly in areas where RMS is more likely to occur, such as the head, neck, or limbs [1].

Imaging Tests Imaging tests create pictures of the inside of the body, allowing doctors to visualize the tumor and its location. These may include:

  • Computed Tomography (CT) scans: CT scans use X-rays and computer technology to produce detailed images of the body's internal structures [4].
  • Magnetic Resonance Imaging (MRI): MRI uses a strong magnetic field and radio waves to create detailed images of the body's soft tissues [4].

Biopsy A biopsy involves removing a sample of tissue from the tumor for further examination. This is usually done under local anesthesia, and the sample is then sent to a laboratory for histopathological analysis [6]. The biopsy results help determine the subtype of RMS, which is essential in planning treatment.

Other Diagnostic Tests Additional tests may be performed to rule out other conditions or to assess the tumor's response to treatment. These may include:

  • Blood tests: Blood tests can help identify any potential complications or monitor the effectiveness of treatment [3].
  • Bone scans: Bone scans use small amounts of radioactive material to detect any abnormalities in the bones, which can indicate metastasis [5].

Staging Evaluations The Children's Oncology Group (COG) uses Clinical Group (CG) and modified Tumor Node Metastasis (TNM) stage to classify RMS. Staging evaluations help determine the extent of disease spread and guide treatment decisions [10].

It is essential to note that diagnostic tests for childhood RMS are continually evolving, and new technologies may become available in the future.

References:

[1] The Children's Oncology Group. (2022). Rhabdomyosarcoma: A Guide for Parents and Caregivers.

[3] American Cancer Society. (2020). Blood Tests.

[4] Mayo Clinic. (2022). Computed Tomography (CT) Scan.

[5] Mayo Clinic. (2022). Bone Scan.

[6] MedlinePlus. (2022). Biopsy.

[10] The Children's Oncology Group. (2022). Rhabdomyosarcoma: A Guide for Parents and Caregivers.

Additional Diagnostic Tests

  • Blood tests
  • Biopsy
  • Magnetic Resonance Imaging (MRI)
  • Computed Tomography (CT) scans
  • Bone scans

Treatment

Current Treatment Options for Obsolete Embryonal Childhood Rhabdomyosarcoma

While there are no specific treatment options mentioned in the search results for "obsolete" embryonal childhood rhabdomyosarcoma, we can infer some information from the context.

  • Combination Therapy: Giving dasatinib and ganitumab may work better in treating patients with embryonal and alveolar rhabdomyosarcoma compared to standard treatment [4].
  • Carboplatin and Etoposide: Based on study data, carboplatin with etoposide has shown effectiveness in embryonal rhabdomyosarcoma [5].
  • Chemotherapy Regimens: Chemotherapeutic regimens frequently include alkylating agents (ifosfamide and cyclophosphamide), vincristine, actinomycin D, and others for the treatment of RMS [9].

Note on Obsolete Treatment

It's worth noting that the term "obsolete" is not explicitly mentioned in any of the search results. However, we can infer that the treatments listed above may be considered outdated or less effective compared to newer therapies.

  • Improved Survival Rates: Despite this, improvements in survival rates for children and adolescents with cancer have been achieved, with a 50% decrease in childhood cancer mortality between 1975 and 2020 [10].

Current Treatment Guidelines

For current treatment guidelines and recommendations, it's essential to consult the latest clinical trials, research studies, or reputable medical resources.

References:

[4] - Giving dasatinib and ganitumab may work better in treating patients with embryonal and alveolar rhabdomyosarcoma compared to standard treatment. [5] - Based on study data, carboplatin with etoposide has shown effectiveness in embryonal rhabdomyosarcoma. [9] - Chemotherapeutic regimens frequently include alkylating agents (ifosfamide and cyclophosphamide), vincristine, actinomycin D, and others for the treatment of RMS.

💊 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

The differential diagnosis for obsolete embryonal childhood rhabdomyosarcoma (ERMS) includes various subtypes and variants of RMS, as well as other soft tissue tumors.

  • Sclerosing and spindle cell variants of RMS: These variants are characterized by a sclerotic or spindle cell morphology, which can make them difficult to distinguish from ERMS. According to [10] and [12], these variants should be considered in the differential diagnosis of ERMS.
  • Solid pattern of alveolar RMS: Alveolar RMS is a subtype of RMS that is characterized by a solid or alveolar growth pattern. While it is more commonly associated with older children and adolescents, it can also occur in younger children. The solid pattern of alveolar RMS should be considered in the differential diagnosis of ERMS, particularly in cases where the histology is not clearly diagnostic [10] and [12].
  • Other soft tissue tumors: In addition to these variants and subtypes of RMS, other soft tissue tumors such as fibrosarcoma, leiomyosarcoma, and neurofibrosarcoma should also be considered in the differential diagnosis of ERMS.

It is worth noting that the differential diagnosis for ERMS can be complex and requires a thorough evaluation of clinical, radiological, and histopathological features. A multidisciplinary approach involving pediatric oncologists, pathologists, and radiologists is often necessary to accurately diagnose and manage these cases.

References: [10] - The differential diagnosis of embryonal RMS includes the sclerosing and spindle cell variants of RMS, as well as the solid pattern of alveolar RMS [10]. [12] - The differential diagnosis of embryonal RMS includes the sclerosing and spindle cell variants of RMS, as well as the solid pattern of alveolar RMS [12].

Additional Information

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