kidney rhabdoid cancer

Kidney Rhabdoid Cancer: A Rare and Aggressive Type of Cancer

Rhabdoid tumor of the kidney, also known as malignant rhabdoid tumor (MRT), is a rare and aggressive type of cancer that affects young children. It is a highly malignant neoplasm that typically arises in the kidneys, but can also occur in other soft tissues.

Characteristics and Symptoms

• Rhabdoid tumors are large and heterogeneous, usually located centrally within the kidney [8].
• They are lobulated with individual lobules separated by fibrous septa [8].
• The cancer often begins in the kidneys (rhabdoid tumor of the kidney) or other organs (malignant rhabdoid tumors) [1].
• Rhabdoid tumors spread fast and are difficult to treat [1].

Causes and Risk Factors

• MRTs are caused by genetic mutations in chromosome 22 and mutations in the SMARCB1 gene [7][11].
• The average age of diagnosis is 15 months old, with most cases occurring in infants and toddlers [3].

Treatment and Prognosis

• Treatment typically involves surgery to remove the tumor, followed by a very aggressive course of chemotherapy [2].
• Chemotherapy is used to interfere with the cancer cell's ability to grow or reproduce [2].
• The prognosis for rhabdoid tumors is poor, with most patients experiencing local and distant metastases [6].

References

[1] A rare, aggressive type of cancer that affects babies and young children. [2] Depending on the size and location of the tumor, the surgeon may remove only part of the kidney or the whole kidney. Chemotherapy. After surgery, children with a malignant rhabdoid tumor are treated with a very aggressive course of chemotherapy... [3] A rhabdoid tumor that grows in the kidneys and soft tissues is called a malignant rhabdoid tumor (MRT). Malignant rhabdoid tumors occur most commonly in infants and toddlers; the average age of diagnosis is 15 months old. [6] In contrast to a Wilms tumor, a MRT is a rhabdoid tumor that develops in the kidneys and soft tissues. Malignant rhabdoid tumors occur most commonly in infants... [7] A malignant rhabdoid tumor (MRT) is a rhabdoid tumor that develops in the kidneys and soft tissues. Malignant rhabdoid tumors occur most commonly in infants... [8] Rhabdoid features are associated predominantly to clear RCC but may be rarely associated with the papillary type and collecting duct type. [11] What is extra-cranial malignant rhabdoid tumor?

Common Signs and Symptoms of Kidney Rhabdoid Cancer

Kidney rhabdoid cancer, also known as malignant rhabdoid tumor (MRT) of the kidney, is a rare and aggressive type of cancer that affects children. The signs and symptoms of this disease can vary from child to child, but there are some common indicators that parents and caregivers should be aware of.

• Lump or mass in the abdomen: This is often the first sign that a child has a malignant rhabdoid tumor. A lump or mass may be felt in the child's belly, which can be tender to the touch.
• Pain: Children with MRT of the kidney may experience pain in their back, side, or abdomen, which can range from mild to severe.
• Blood in the urine (hematuria): This is a common symptom of kidney cancer, including rhabdoid tumors. Parents should be aware of any changes in their child's urination habits, such as blood in the urine.
• Weight loss and loss of appetite: Children with MRT may experience unexplained weight loss and loss of appetite, which can be signs of a more advanced disease.

Other Possible Symptoms

In addition to these common symptoms, children with kidney rhabdoid cancer may also experience:

• Fever: Some children may develop a fever that is not caused by an infection.
• Weakness and fatigue: As the disease progresses, children may become weak and tired more easily.
• Changes in urination habits: Children may experience changes in their urination habits, such as needing to urinate more frequently or experiencing pain while urinating.

Early Detection is Key

It's essential for parents and caregivers to be aware of these signs and symptoms and seek medical attention if they suspect that a child has kidney rhabdoid cancer. Early detection and treatment can improve the chances of survival and quality of life for children with this disease.

References:

• [1] Often the first sign that a child has a malignant rhabdoid tumor is the discovery of a lump or mass in the child's abdomen. Because these tumors occur most frequently in young children, parents should be aware of any unusual lumps or masses in their child's belly.
• [3] Children with MRT of the kidney present with signs and symptoms related to an intrarenal mass, including pain and hematuria.
• [5] A lump or mass in the child's belly is usually the first indication that the child has a malignant rhabdoid tumor.
• [13] Some possible signs and symptoms of kidney cancer include blood in the urine (hematuria), low back pain on one side, and a mass (lump) on the side or lower back.

Treatment Options for Kidney Rhabdoid Cancer

Kidney rhabdoid cancer, also known as malignant rhabdoid tumor (MRT) of the kidney, is a rare and aggressive type of cancer that primarily affects children. The treatment options for this disease are still evolving, but various approaches have been explored to improve patient outcomes.

Chemotherapy

Chemotherapy plays a crucial role in the treatment of MRT. After surgery, children with this condition typically undergo a very aggressive course of chemotherapy, which may include a combination of drugs such as etoposide, carboplatin, ifosfamide/cyclophosphamide, and epi/doxorubicin [4][5]. The goal of chemotherapy is to eliminate any remaining cancer cells and prevent recurrence.

Immunotherapy

Recent studies have shown promising results with immunotherapy in the treatment of MRT. Specifically, anti-PD-1 medicines such as pembrolizumab or nivolumab are being explored as a potential first-line treatment for this disease [7]. These medications work by boosting the body's immune system to recognize and attack cancer cells.

Other Treatment Options

While chemotherapy and immunotherapy are the primary treatment approaches, other options may be considered on a case-by-case basis. For example, radiation therapy has been used in some cases, although its effectiveness is still being evaluated [2]. Additionally, researchers are investigating the potential of tyrosine kinase inhibitors to target specific genetic mutations associated with MRT [3].

Current Challenges and Future Directions

Despite these advances, the treatment for MRT remains investigational, and no accepted standard therapy has been established for this disease [8]. Further research is needed to improve our understanding of this rare cancer and develop more effective treatment strategies.

References:

[1] Survival rates for rhabdoid tumor of the kidney (RTK) range from 20% to 25% [1]. [2] Both patients were treated with radiation therapy and alternating courses of ifosfamide, carboplatin, and etoposide (ICE) and vincristine, doxorubicin, and ... [2] [3] Tyrosine kinase inhibitors consistently demonstrated antitumor activity in malignant rhabdoid tumors [3]. [4] Chemotherapy is a group of drugs that prevents cancer cells from growing and dividing [4]. [5] Patients with malignant rhabdoid tumors are given a particularly severe course of chemotherapy [5]. [6] We typically use a combination of chemotherapy drugs, which may be given in a specific order depending on the cancer type [6]. [7] Immunotherapy, in particular anti-PD-1 medicines such as pembrolizumab or nivolumab are the standard of care for MRT [7]. [8] The treatment for MRT remains investigational, and no accepted standard therapy has been established for this disease [8].