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Wilms tumor is a malignancy caused by proliferation of metanephric blastema in the kidneys and is the most common renal malignancy in children. Wilms tumor usually arises sporadically, but it can also occur as a result of a specific congenital anomaly like WAGR (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation) syndrome, Denys–Drash syndrome, or Beckwith–Wiedemann syndrome. Wilms tumor commonly presents as a firm, nontender, smooth mass that does not cross the midline. Wilms tumor can also rarely present with abdominal pain, hematuria, and/or hypertension. The malignancy is diagnosed with abdominal ultrasonography and histopathologic studies (from biopsy or resection). Wilms tumor is treated with multimodal therapy (surgery, chemotherapy, radiation). Influenced by patient age, molecular markers, and pathologic findings, prognosis is favorable overall, with 5-year survival rates approaching 90%.

Retinoblastoma

Retinoblastoma is a rare tumor but the most common primary intraocular malignancy of childhood. It is believed that the condition arises from a neuronal progenitor cell. Retinoblastoma can be heritable or nonheritable. The condition typically presents as unilateral or bilateral leukocoria (abnormal white reflection in the eye) in a child under the age of 2. Retinoblastoma is fatal if not treated but early recognition accounts for a high survival rate in resource-rich countries.

Central Nervous System (CNS) Tumors in Pediatrics

CNS tumors in pediatrics are rare but account for approximately 20 % of cancers in children. Most patients with CNS tumors have supratentorial brain tumors. Patients usually present with headaches, focal neurological deficits with or without seizures. Brain imaging is essential in the diagnosticworkup and a histological examination should be performed on an excisional or incisional biopsy. The histological classification of the tumor has an impact on the decision to offer adjunctive chemotherapy or radiotherapy after surgical resection.

Medullablastoma, Primitive Neuroectodermal Tumor (PNET), Ependymoma and Glioma in Children

Medulloblastomas, primitive neuroectodermal tumors and ependymomas constitute a significant number of central nervous system tumors in children. Medulloblastomas and PNETs are more likely to have been metastasized at the time of diagnosis, compared to ependymomas. The clinical presentation of medulloblastomas and ependymomas is usually due to increased intracranial pressure, while PNETs usually present with head and neck masses. Surgical resection of the tumors is the mainstay of treatment which should be followed by radiotherapy in most cases. Chemotherapy in medulloblastoma and PNET has a proven effect on survival, but its role in ependymomas is still unknown.

Lymphoma in Children — Symptoms and Prognosis

Pediatric lymphomas are rare compared to other malignancies in children. They can be classified into Hodgkin's and non-Hodgkin's lymphomas. Hodgkin's lymphoma is more likely to present with systemic features such as fever, weight loss and night sweats. Non-Hodgkin's lymphoma is more likely to present with primary extranodal involvement. Histologic examination is essential in both conditions to confirm the diagnosis. Staging of the disease is important as it can change the treatment plan.

Neuroblastoma — Symptoms, Classification and Survival Rate

Neuroblastoma is a peripheral tumor of the sympathetic nervous system. After brain tumors, it is the second most common malignant solid tumor in children. Since neuroblastoma can manifest with various symptoms, like ecchymosis of the eyelid or paraplegia, it is crucial to keep it in mind as a differential diagnosis and to be aware of clinical diagnostics and the basic treatment options.

Leukemia in Children — Symptoms and Treatment

Leukemias are considered as the most common form of malignant disorders in children. Acute lymphoblastic leukemia is responsible for approximately 70% of the cases of leukemia in children, while acute myeloid leukemia account for 30% of the cases. Patients with leukemia can present with symptoms related to the loss of normal functioning cells such as anemia, bleeding disorders, and infections, or symptoms related to the expansion of the leukemic cells and metastasis. Cytogenetic and molecular testing is essential as it can have an impact on the treatment and prognosis of the patient. Intensive combination chemotherapy is usually needed for the induction therapy, followed by consolidation therapy with or without stem cell transplantation. Patients with the Philadelphia chromosome should be administered a tyrosine kinase inhibitor and the option for stem cell transplantation.

Central Nervous System Cancers — Diagnosis and Treatment

This article explains basic facts about tumors of the CNS. It includes neuroepithelial tumors like astrocytomas and fibrillary astrocytomas. It also runs through oligodendroglioma, medulloblastoma and meningeal tumors like meningioma and primary CNS lymphoma. The article covers the definition, epidemiology, etiology and causes of CNS tumors, in addition to classification, pathophysiology, symptoms, diagnosis (including history and examination), therapy and prognosis.

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Pediatric Oncology

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Your Educators of course Pediatric Oncology

Brian Alverson, MD

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