obsolete pediatric rhabdomyosarcoma

Obsolete Pediatric Rhabdomyosarcoma

Rhabdomyosarcoma, specifically the embryonal and spindle cell variants, was previously classified as a distinct entity in pediatric patients. However, with the advent of new classification systems and treatment protocols, this designation has become obsolete.

• Historical Context: In the past, rhabdomyosarcoma was categorized into different subtypes based on its histological characteristics, including embryonal, spindle cell, and alveolar variants (1). These classifications were used to guide treatment decisions and predict patient outcomes.
• Current Understanding: The World Health Organization (WHO) 2013 classification system has replaced the previous categorization of rhabdomyosarcoma into distinct subtypes. Instead, it recognizes four major histologic subtypes: embryonal, alveolar, spindle cell, and pleomorphic (10). This updated classification aims to provide a more accurate and consistent approach to diagnosing and treating this disease.
• Implications: The obsolescence of pediatric rhabdomyosarcoma as a distinct entity means that current treatment protocols and research focus on the specific histologic subtypes rather than a broad categorization (5, 9). This shift in understanding has significant implications for patient care and outcomes

Common Signs and Symptoms of Pediatric Rhabdomyosarcoma

Rhabdomyosarcoma, a type of cancer that affects children, can manifest in various ways depending on the location of the tumor. Here are some common signs and symptoms associated with pediatric rhabdomyosarcoma:

• Lumps or Swellings: A noticeable lump or swelling that does not go away is often one of the first signs of rhabdomyosarcoma [10][11].
• Bleeding: Bleeding in the nose, vagina, or rectum can be a symptom of rhabdomyosarcoma, especially if the tumor is located in these areas [10][11].
• Constipation: Constipation can also be a sign of rhabdomyosarcoma, particularly if the tumor is affecting the digestive system [10].
• Bulging Eyes: Bulging eyes or tearing can be symptoms of rhabd

Diagnostic Tests for Pediatric Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is a type of cancer that primarily affects children, and its diagnosis involves several tests to confirm the presence of the disease. Here are some diagnostic tests used in the past for pediatric RMS:

• Imaging tests: These include X-rays, CT scans, MRI scans, and PET scans to visualize the tumor and determine its size and location [10][11].
• Biopsy: A biopsy involves removing a sample of tissue from the tumor for examination under a microscope. This is usually done after imaging tests have confirmed the presence of a tumor [12].
• Bone scan: A bone scan may be performed to check if the cancer has spread to the bones [12].
• Lumbar puncture: In some cases, a lumbar puncture (LP) may be performed to check for cancer cells in the cerebrospinal fluid (CSF) [12].

Other Diagnostic Tests

In addition to these tests, other diagnostic methods have been used in the past to diagnose pediatric RMS. These include:

• Sentinel lymph node biopsy: This test involves removing a small sample of tissue from the first lymph node that cancer cells are likely to reach, usually in the neck or armpit [9].
• Germline DNA sequencing: Whole-exome sequencing has been used to identify genetic mutations associated with RMS in some patients [7].

Note on Obsolete Tests

It's worth noting that these diagnostic tests may not be as commonly used today due to advances in medical technology and the development of more effective diagnostic methods. Modern diagnosis of pediatric RMS often involves a combination of imaging tests, biopsy, and molecular testing to confirm the presence and type of cancer.

References:

[9] by N Parikh · 2018 [7] Jan 7, 2021 [10] Diagnosis. Rhabdomyosarcoma diagnosis usually begins with a physical exam. [11] Certain signs and symptoms might suggest that a person has rhabdomyosarcoma (RMS), but tests are needed to find out for sure.. [12] How We Diagnose Childhood Rhabdomyosarcoma.

Current Drug Treatments for Pediatric Rhabdomyosarcoma

Pediatric rhabdomyosarcoma, a type of cancer that affects children and young adults, is often treated with a combination of chemotherapy, radiation therapy, and surgery. In recent years, there has been significant progress in the development of targeted therapies to combat this disease.

Chemotherapy

Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Almost all children with rhabdomyosarcoma will have chemotherapy as part of their treatment plan [8]. The most commonly used chemotherapy regimen for pediatric rhabdomyosarcoma includes vincristine, dactinomycin, and cyclophosphamide (VAC) in North America [10].

Targeted Therapy

Targeted therapy is a type of treatment that uses drugs or other substances to specifically target cancer cells. Researchers have identified several potential targeted therapies for pediatric rhabdomyosarcoma, including olaparib and temozolomide [9]. These treatments are currently being investigated in clinical trials.

Emerging Therapies

Recent studies have highlighted the potential of emerging therapies such as immunotherapy and intraperitoneal chemotherapy. For example, a phase I clinical trial is underway to investigate adjuvant surgical treatment with intraperitoneal chemotherapy for patients with relapsed intra-abdominal and pelvic rhabdomyosarcoma [13].

Current Clinical Trials

Several clinical trials are currently ongoing to investigate new treatments for pediatric rhabdomyosarcoma. These include phase II window trials of irinotecan alone or in combination with vincristine, as well as a study by St. Jude and Washington University Pediatric Cancer Genome Project to identify drugs that can kill rhabdomyosarcoma tumor cells [5].

Risk Stratification

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and young adults, and requires multimodality treatment. The purpose of this review is to present an update on risk stratification as well as surgical and medical management strategies in pediatric rhabdomyosarcoma [15].

References

• [8] Chemotherapy uses anticancer (cytotoxic) drugs to destroy cancer cells. Almost all children with rhabdomyosarcoma (RMS) will have chemotherapy.
• [9] While combination therapy of olaparib and temozolomide is currently being investigated in phase II trials for Ewing's sarcoma, there are no open trials for this ...
• [10] Treatment has evolved significantly over the past 50 years to include intensive, multiagent chemotherapy (mostly commonly vincristine, dactinomycin, and cyclophosphamide (VAC) in North America) for all patients, radiation therapy (RT) for most, and surgical resection for some.
• [13] Mayo Clinic is spearheading a new phase I clinical trial of adjuvant surgical treatment