24 Questions
What is the primary objective of the induction phase of treatment in ALL?
To induce remission
What is the most common age range of patients with T-cell ALL that exhibit respiratory distress due to large mediastinal mass?
Adolescent boys
What laboratory test is used to detect chromosomal abnormalities that may influence the prognosis and the choice of therapy in ALL?
Cytogenetic studies
What is the significance of a high initial leukocytic count in ALL patients?
It is a poor prognostic factor
Which of the following chemotherapy options is NOT used in the induction phase of treatment in ALL?
Cyclophosphamide
What is the primary purpose of lumbar puncture in the diagnosis of ALL?
To examine the CSF for malignant cells
Which of the following is a differential diagnosis of ALL?
Acute myelogenous leukemia
What is the duration of the induction phase of treatment in ALL?
4 weeks
What is the initial treatment of choice for patients with localized neuroblastoma?
Complete surgical excision
What is the significance of urinary catecholamines in neuroblastoma?
They are elevated in at least 90% of patients at diagnosis
What is the purpose of bone marrow aspiration in neuroblastoma?
To exclude infiltration of bone marrow
What is a paraneoplastic syndrome associated with neuroblastoma?
Secretory diarrhea and profuse sweating
What is the role of imaging studies in neuroblastoma?
To define primary tumor extent
What is the significance of cytogenetics and histopathology in neuroblastoma?
They are prognostic factors
What is the treatment approach for patients with advanced neuroblastoma?
Combination chemotherapy followed by delayed resection of primary tumor
What are common chemotherapeutic agents used in neuroblastoma?
Vincristine, cyclophosphamide, doxorubicin, cisplatin, and etoposide
What is the primary site of neuroblastoma in most cases?
Abdomen
What is the age incidence of neuroblastoma?
90% are less than 5 years
What is the treatment phase that involves vincristine and actinomycin D?
Preoperative chemotherapy
In what week is surgery typically done after preoperative chemotherapy?
5th week
What is the prognosis for localized disease?
90%
What is the percentage of cases with a familial history of neuroblastoma?
1-2%
What is the presentation of neuroblastoma due to widespread bone metastasis?
Pancytopenia, arthritis, and bone pain
What is the reason for radiotherapy in neuroblastoma treatment?
For incompletely resected diseases and lung metastasis
Study Notes
Acute Lymphoblastic Leukemia (ALL)
- Signs of mediastinal compression: respiratory distress, typically seen in adolescent boys with T-cell ALL
- Signs of increased intracranial pressure: papilledema, retinal hemorrhages, and cranial nerve palsies
- Laboratory diagnosis:
- Hemogram shows anemia, thrombocytopenia, and total leucocytic count < 10,000/mm3 in most patients
- Bone marrow examination shows > 25% homogeneous population of lymphoblasts
- Lumbar puncture examines CSF for malignant cells
- Serum uric acid, liver function tests, and renal function tests before onset of treatment
- Cytogenetic studies detect chromosomal abnormalities influencing prognosis and therapy choice
- Immunophenotyping
- Chest X-ray for mediastinal lymph nodes in T-cell leukemia
Differential Diagnosis
- Acute myelogenous leukemia
- Tumors that infiltrate the bone marrow (neuroblastoma, lymphomas, retinoblastoma)
- Diseases of bone marrow failure (aplastic anemia)
- Infectious mononucleosis
Treatment of Standard Risk ALL
- The single most important prognostic factor is treatment
- Important prognostic factors: age (1-10 years), initial leukocytic count, and speed of response to treatment
- Drug therapy includes 3 phases + intrathecal therapy
- Induction of remission (4 weeks): vincristine, dexamethasone or prednisone, L-asparaginase, and intrathecal cytarabine or methotrexate
Neuroblastoma
- Embryonal tumor derived from neural crest cells that form the adrenal medulla and 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 to tumors containing mature ganglion cells
Clinical Manifestations
- Primary sites: adrenal gland, abdomen, chest, pelvis, and neck
- Diverse presentations due to different site of origin:
- Abdominal mass (commonest presentation)
- Posterior mediastinal mass
- Proptosis (unilateral or bilateral)
- Multiple skin nodules in infancy
- Spinal cord compression
- Pancytopenia, arthritis, and bone pain
- Cervical neuroblastoma
- Paraneoplastic syndromes
Investigations
- Plain X-ray: may show calcification in abdominal neuroblastoma
- Ultrasound scan, CT, or MRI: define primary tumor extent
- Bone marrow: multiple site aspirates necessary to exclude infiltration
- Urinary catecholamines: elevated in at least 90% of patients at diagnosis
- Biopsy: routine histology, immunocytochemistry, and molecular/genetic study required
Differential Diagnosis
- Wilms tumor
- Leukemia
Treatment
- Complete surgical excision is the initial treatment of choice for localized neuroblastoma
- Combination chemotherapy for advanced diseases
- Delayed resection of primary tumor after numerous courses of chemotherapy
- Chemotherapeutic agents: vincristine, cyclophosphamide, doxorubicin, cisplatin, and etoposide
- Radiation therapy for primary tumor bed and area of metastatic disease
Prognosis
- Affected by age of patient at presentation
- Stage of the disease
- Primary site
- Presence or absence of metastasis
- Cytogenetics and histopathology
This quiz covers the signs and symptoms of leukemia, including mediastinal compression and increased intracranial pressure. It also covers laboratory diagnosis, including hemogram results and blast cells in peripheral blood.
Make Your Own Quizzes and Flashcards
Convert your notes into interactive study material.
Get started for free
