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What is the duration of the preoperative chemotherapy for nonmetastatic disease?
What is the duration of the preoperative chemotherapy for nonmetastatic disease?
When is surgery usually done for nonmetastatic disease?
When is surgery usually done for nonmetastatic disease?
What is the next step after surgery for nonmetastatic disease?
What is the next step after surgery for nonmetastatic disease?
What percentage of neuroblastoma cases occur in children under the age of 5?
What percentage of neuroblastoma cases occur in children under the age of 5?
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What is the most common primary site of neuroblastoma?
What is the most common primary site of neuroblastoma?
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What is the common presentation of neuroblastoma in infants under 6 months?
What is the common presentation of neuroblastoma in infants under 6 months?
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What is the prognosis for localized neuroblastoma?
What is the prognosis for localized neuroblastoma?
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What percentage of neuroblastoma cases have a familial component?
What percentage of neuroblastoma cases have a familial component?
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What is the risk factor for neuroblastoma development?
What is the risk factor for neuroblastoma development?
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What is the prognosis for metastatic neuroblastoma?
What is the prognosis for metastatic neuroblastoma?
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Study Notes
Acute Lymphoblastic Leukemia (ALL)
- High-risk cases receive daunomycin at weekly intervals
- Remission is defined as less than 5% blasts in the marrow and a return of neutrophil and platelet counts to near-normal levels after 4-5 weeks of treatment
- Treatment consists of three phases:
- Induction of remission (4 weeks)
- Consolidation intensification phase (14-28 weeks)
- Maintenance therapy (2-3 years)
Types of Relapse
- Bone marrow relapse: most common and serious, treated with bone marrow transplantation following intensive chemotherapy
- CNS relapse: manifests as increased intracranial tension, convulsions, and cranial nerve paralysis, treated with intrathecal medication, craniospinal irradiation, and systemic chemotherapy
- Testicular relapse: painless swelling of one or both testes, treated with systemic chemotherapy and local irradiation
Lymphomas
- Third most common cancer in children
- Includes non-Hodgkin lymphomas and Hodgkin disease
- Non-Hodgkin lymphoma:
- Malignant clonal proliferation of primarily T or B-lymphocytes
- Increased frequency in children with immunodeficiency diseases
- Subtypes:
- Small non-cleaved cell lymphoma (Burkitt and non-Burkitt subtypes, B-cell origin)
- Lymphoblastic lymphoma: T-cell type
- Large cell NHL: T-cell, B-cell or indeterminate cell origin
- Clinical manifestations:
- Abdominal lymphoma: presents with abdominal mass or distention, nausea, vomiting, or change in bowel habits
- Mediastinal lymphoma: presents with mediastinal mass, pleural effusions, or respiratory distress
- Head and neck regions: painless, firm lymph nodes
- Involvement of other systems:
- Bone marrow: anemia or pancytopenia
- CNS: manifestations of increased intracranial pressure and cranial nerve paralysis
Hodgkin Lymphoma
- Incidence: not common in children, occurs in late childhood to adolescence, males more affected than females
- Pathologic classification:
- Nodular sclerosing type
- Mixed cellularity type
- Lymphocytic predominant type
- Lymphocytic depletion type: least common and least favorable
- Clinical manifestations:
- General symptoms and signs: lethargy, anorexia, unexplained fever, night sweating, and weight loss
- Painless enlargement of lymph nodes
- Mediastinal syndrome: a common presentation
Wilms Tumor (Nephroblastoma)
- Embryonal tumor of the kidney
- May arise in one or both kidneys
- Most common genitourinary malignancy affecting children, accounts for about 6% of childhood malignancies
- Occurs in equal frequency in boys and girls, most commonly between 2-5 years
- Triphasic appearance, with varying proportions of three cell types (blastemal, stromal, epithelial)
- Clinical features:
- Asymptomatic abdominal mass usually discovered accidentally by parents or routine abdominal examination
- Associated symptoms: abdominal pain, fever, and hematuria
- Hypertension may occur in 25% of cases
- Bilateral Wilms tumor may occur
Neuroblastoma
- Embryonal tumor derived from neural crest cells that form the adrenal medulla and the sympathetic nervous system
- Third most common solid tumor of childhood
- Age incidence: 90% are less than 5 years
- Etiology: unknown, may be attributed to environmental and genetic factors
- Pathology: ranges from undifferentiated small round cells (neuroblastoma) to tumours containing mature ganglion cells (ganglioneuroblastoma or ganglioneuroma)
- Clinical manifestations:
- Abdominal mass: commonest presentation, hard, irregular, and non-tender mass located in right or left upper quadrant
- Posterior mediastinal mass: may present with mediastinal syndrome, progressive dyspnea, dysphagia, and stridor
- Proptosis: proptosis may be unilateral or bilateral
- Multiple skin nodules in infancy: firm and purple in color, mainly occur in infants under the age of 6 months
- Spinal cord compression: may cause paralysis and bowel or bladder dysfunction
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Description
This quiz covers the different phases of treatment for Acute Lymphoblastic Leukemia (ALL), including induction, consolidation, and maintenance therapy.