<unk>  is an aggressive tumor in the pediatric population. It commonly starts in the kidney (rhabdoid tumor of the kidney) but it can occur in other soft tissues (extrarenal<unk>  and the brain (atypical teratoid rhabdoid tumor). It was originally believed to be a rhabdomyosarcomatoid variant of Wilms' tumor because of the cells resemblance to rhabdomyoblasts. It was later determined to be its own distinct clinicopathologic entity because the cells could not demonstrate myogenic differentiation on immunohistochemical studies.<unk>  of the kidney accounts for about 2% of all pediatric renal<unk> ies. The exact cause is unknown but it has been shown to be associated with the mutation of SMARCB1/INI-1 which is a tumor suppressor gene. It may be associated with conditions such as ependymoma and astrocytoma. Some of the histological characteristics of<unk>  include eosinophilic cytoplasmic inclusions, vesicular nucleus with prominent nucleoli, and moderate to abundant eosinophilic cytoplasm. The incidence of<unk>  of the kidney is approximately 0.019 per 100,000 individuals per year in the US. Though the tumor is seen mostly in infants, some cases have been reported in adults. The prognosis is poor with the overall survival rate being low. Metastasis is a common cause of death in these patients and most of the tumors metastasize early. Patients experience symptoms based on the location of the tumor. Patients with tumors in the liver may experience abdominal pain, fever, irritability. Those with tumors in the brain present with headache, ataxia, nystagmus. Imaging modalities that may be used in the diagnosis of<unk>  include CT scan and MRI. They have different findings depending on the tumor location. The treatment depends on the character of the tumor and the circumstances that surround it. Some may be treated with surgical excision and chemotherapy/radiotherapy. Some tumors cannot be treated surgically and may need to rely on chemotherapy. Some common chemotherapeutic agents used include vincristine, cyclophosphamide, doxorubicin, and ifosfamide.
Malignant rhabdoid tumor
Malignant rhabdoid tumour
Primitive neuroectodermal tumor
Atypical teratoid rhabdoid tumor
Endodermal sinus tumor
Ameloblastoma
Neurofibroma
Esthesioneuroblastoma