Malignant diseases Flashcards
Most common malignancy
Leukemia - ALL
Second most common malignancy
Brain tumors
Cancers associated with EBV
- Burkitt lymphoma
- Hodgkin lymphoma
- Nasopharyngeal carcinoma
Definition of allogeneic transplantation
Transplantation from a compatible donor
Definition of autologous transplantation
Transplantation for the patient him/herself, harvested beforehand, while the marrow is uninvolved or in remission
Infections associated with children under cancer therapy
- Pneumocystis jiroveci/carinii pneumonia
- Disseminated fungal infection (e.g. aspergillosis, candidiasis)
- Coagulase-negative staphylococcal infections of CV catheters
Age group acquiring ALL
Peaks at 2-5 years of age
Symptoms of ALL
- General:
- Malaise, anorexia
- Bone marrow infiltration:
- Anemia –> pallor, lethargy
- Neutropenia –> infection
- Thrombocytopenia –> bruising, petechiae, nose bleeds
- Bone pain
- Reticluo-endothelial infilatration:
- Hepatosplenomegaly
- Lymphadenopathy
- Other organ infiltration (more common at relapse):
- CNS –> headaches, vomiting, nerve palsies
- Testes –> testicular enlargement
How does the CBC look like in ALL?
- Low Hb
- Low platelets - thrombocytopenia
- Circulating leukemic blast cells
In ALL, a mediastinal mass is characteristic for?
T-cell disease
Most common subtype of ALL
Precursor B-cell –> B-lymphoblasts
Factors contributing to prognosis in ALL
- Patient age
- WBC count at presentation
- Cytogenetics of the leukemic cell
- Response to treatment
Poor prognosis in ALL, indicated when
- Age <1 or >10 years of age
- WBC >100.000/mm3
- Slow response to initial treatment
- Chromosomal abnormalities
What drug is given to protect real function in tumor lysis?
Allopurinol
What does remission mean?
Eradication of the leukemic blasts and restoration of normal marrow function
For how long is continuing therapy for ALL given?
Up to 3 years from diagnosis
Most common location of brain tumors
Infratentorial (located below the tentorium cerebelli)
Most common solid tumor in children
Brain tumor
Most common brain tumor in children
Astrocytoma
* Juvenile pilocytic astrocytoma (most often localized in cerebellum)
Clinical features in spinal tumors
- Back pain
- Peripheral weakness of arm or legs
- Bladder/bowel dysfunction
- -> depending on level of lesion
Clinical presentation of brain tumor
- Persistent or recurrent vomiting
- Problems with balance, coordination or walking
- Behavioral change
- Abnormal eye movement
- Seizures
- Abnormal head position - wry neck, head tilt, persistent stiff neck
First treatment of brain tumors
Surgery, if anatomically possible
Clinical features in Hodgkin lymphoma
- Classically presents with painless lymphadenopathy, most frequently in the neck. Large and firm nodes.
- Long clinical history
- System symptoms (‘B’ symptoms) - uncommon
Prognosis of Hodgkin lymphoma
80%
With disseminated disease - 60%
Clinical features of a mediastinal mass causing SVC obstruction
- Dyspnea
- Facial swelling and flushing
- Venous distention in the neck
- Distended veins in the upper chest and arms
Most common non-hodgkin lymphoma
Aggressive lymphoma –> large b-cell lymphoma
Histology of Burkitt lymphoma
B-cell non-Hodgkin lymphoma
The three variants of Burkitt lymphoma
- Endemic variant - in children living in malaria endemic regions. Malaria reduces resistance to EBV.
- Sporadic - can be associated with EBV
- Immunodeficiency-associated - associated with HIV
Histologic subtypes of non-hodgkin lymphomas
- Lymphoblastic (usually T-cell)
- Small, non cleaved cell lymphoma (B-cell)
- Large cell (T cell, B cell or indeterminate)
The histological hallmark of Hodgkin lymphoma
Reed-Sternberg cell
What does neuroblastoma arise from?
- Neural crest tissue in the adrenal medulla
2. Sympathetic nervous system
At which age is neuroblastoma most common?
Before the age of 5 years
Clinical features of neuroblastoma
Initial presentation often as metastasis:
- Abdominal mass (can lie anywhere along the sympathetic chain from the neck to the pelvis), classically from adrenal origin
- Weight loss
- Pallor
- Hepatomegaly
- Bone pain
- Limp
Elevation in urine of which catecholamine indicate neuroblastoma?
Homovanillic acid (HVA)
Elevation of urine Vanillylmandelic acid (VMA) indicate which tumor?
Pheochromocytoma
Neurologic signs in neuroblastoma
- Ataxia
- Myoclonus
- ‘Dancing eyes’
- ‘Dancing feet’
- Horner syndrome
Tumor lysis syndrome
- Hyperuricemia
- Electrolyte imbalance (hyperkalemia, hypophosphatemia, hypocalcemia)
- Renal failure
Most common renal tumor of childhood
Wilms tumor
Wilms tumor originates from
Embryonal renal tissue
Most common age group of Wilms tumor
2-5 years of age
Clinical features of Wilms tumor
- Asymptomatic abdominal mass (found on routine examination/bathing)
- Hematuria
Wilms tumor association
- Hemihypertrophy
- Aniridia
- Genitourinary anomalies
- WAGR
What are sarcomas?
Cancers of connective tissue, such as muscle or bone
Most common soft tissue cancer
Rhabdomyosarcoma
Rhabdomyosarcoma originate from
Primitive mesenchymal tissue
Most common site of rhabdomyosarcoma
Head and neck (40%)
- Proptosis
- Nasal obstruction
- Bloodstained nasal discharge
Different presentations of rhabdomyosarcoma
- Head and neck (40%)
- Proptosis
- Nasal obstruction
- Bloodstained nasal discharge
- Genitourinary tract (20%)
- Dysuria, urinary obstruction
- Scrotal mass
- Bloodstained vaginal discharge
- Extremities (20%)
- Trunk (10%)
- Metastatic (lung, liver, bone or bone marrow)
Histological types of rhabdomyosarcoma
- Embryonal (60%)
* Botryoid - Alveolar (15%)
- Pleomorphic - adult form
Most common bone tumor
Osteosarcoma
* Appear in 2nd decade (adolescence) - when rapid bone growth
Symptoms of osteosarcoma
- Persisten localized bone pain
Genetics of retinoblastoma
Rb gene on chromosome 13. Autosomal dominant.
All bilateral tumors are hereditary, as ar about 20% of unilateral cases.
Clinical features of retinoblastoma
- White pupillary reflex
- Squint
Kaposi sarcoma is associated with which virus?
Human herpes virus-8
