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Pediatrics > Oncology > Flashcards

Oncology Flashcards

1
Q

Most common childhood cancer

A

Leukemia

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2
Q

Second most common childhood cancer

A

Brain tumors

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3
Q

Most common solid tumors

A

Brain tumors

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4
Q

Most common brain tumors

A
  1. Glial cell tumors
  2. Primitive neuroectodermal tumors
  3. Ependymomas
  4. Craniopharyngiomas
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5
Q

Glial cell tumors

A
  • Include astrocytomas
  • High grade tumors often arise in the supratentorial region
  • Low grade tumors arise in the infratentorial region
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6
Q

Primitive neuroectodermal tumors

A
  • Include medullowblastomas

- Arise from cerebellum

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7
Q

Most common infratentorial tumor

A

Medulloblastoma

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8
Q

Most common supratentorial tumor

A

Astrocytoma

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9
Q

Optic gliomas are associated with

A
  • Diminished vision
  • Visual field deficits
  • Strabismus
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10
Q

Craniopharyngiomas are associated with

A
  • Growth retardation
  • Delayed puberty
  • Visual changes
  • Diabetes insipidus
  • Hormonal problems because of involvement of the hypothalamic-pituitary axis
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11
Q

Second most common solid tumor

A

Neuroblastoma

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12
Q

Neuroblastoma

A
  • Malignant tumor of neural crest cells may arise anywhere along the sympathetic ganglia chain and within the adrenal medulla
  • Peak incidence in first 5 years
  • 50% in abdomen or pelvis
  • 20% in posterior mediastinum
  • 5% in neck
  • Deletion on short arm of chromosome 1
  • Translocation between 1p and 17q
  • Anomalies on 14q and 22q
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13
Q

Abdominal and pelvic location of neuroblastoma

A
  • Firm abdominal mass that often crosses the midline (most common presentation)
  • Abdominal pain
  • Anorexia
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14
Q

Mediastinal location of neuroblastoma

A
  • Respiratory distress

- Incidental radiographic finding

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15
Q

Cervical location of neuroblastoma

A
  • Tracheal compression
  • Horner’s syndrome (ie miosis, ptosis, enophthalmus [backward displacement of eye in orbit], anhidrosis [absence or decreased sweating])
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16
Q

Catecholamines effects of neuroblastoma

A
  • Flushing
  • Hypertension
  • Headache
  • Sweating
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17
Q

Nonspecific signs and symptoms of neuroblastoma

A
  • Fever

- Weight loss

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18
Q

Acute cerebellar atrophy associated with neuroblastoma

A

“Dnaging eyes and feet”:

  • Ataxia
  • Opsoclonus (random eye jerks)
  • Myoclonus
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19
Q

Metastatic disease associated with neuroblastoma

A
  • Present in up to 70% at time of diagnosis
  • Periorbital ecchymosis and propotosis (periorbital metastases)
  • Skin nodules with blueberry muffin appearance (skin mets)
  • Bone pain or limp (cortical bone mets)
  • Hepatomegaly (liver mets)
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20
Q

Diagnosis of neuroblastoma

A
  • Urine excretion of excessive catecholamines, including VMA and HVA, is characteristic
  • Definitive diagnosis is by positive bone marrow biopsy plus elevated urine catecholamines or by results of biopsy
  • CT or MRI to asses tumor spread
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21
Q

Staging of neuroblastoma

A
  • Stage I: localized tumor confined to the structure of origin
  • Stage II: tumor extends beyond structure of origin but does not cross midline
  • Stage III: tumor extends past midline
  • Stage IV: mets to the bone, lymph nodes, bone marrow or soft tissue
  • Stage IVS: localized tumor at stage I or II but with distant mets to any organ but bone
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22
Q

Prognosis of neuroblastoma

A
  • GOOD prognosis occurs in children younger than 1 year of age and in patients with stage I and II disease. Spontaneous regression without treatment may occur in young infants with stage IVS disease.
  • POOR prognosis sis associated with stage III and IV disease, amplification of oncogene N-myc (chromosome 2), tumor cell diploidy, and high levels of the serum markers ferritin, lactic dehydrogenase, and neuron-specific enolase
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23
Q

Wilms’ tumor

A
  • Most common childhood renal tumor
  • 75% younger than 5 years
  • Associated genetic findings or syndromes including Beckwith-Wiedmann syndrome (hemihypertrophy, macroglossia, visceromegaly), deletion of short arm chromosome 11 and WAGR syndrome (Wilms’ tumor, aniridia, genitourinary abnormalities, and mental retardation)
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24
Q

Beckwith-Wiedmann syndrome

A
  • Wilms’ tumor
  • Hemihypertrophy
  • Macroglossia
  • Visceromegaly
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25
Q

WAGR syndrome

A
  • Wilms’ tumor
  • Aniridia
  • Genitourinary abnormalities
  • Mental retardation
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26
Q

Clinical features of Wilms’ tumor

A
  • Abdominal mass that rarely crosses midline
  • Abdominal pain with or without vomiting
  • Hematuria
  • Hypertension
  • Nonspecific findings include fever, anorexia, and weight loss
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27
Q

Staging of Wilms’ tumor

A
  • Stage I: tumor limited to kidney and completely excised intact without rupture
  • Stage II: tumor extends locally but can still be completely excised without residual disease
  • Stage III: residual tumor remains in abdomen or spillage of tumor occurs during resection
  • Stage IV: distant mets to lung (most common), liver, bone, and brain
  • Stage V: bilateral renal involvement
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28
Q

Most common soft tissue sarcoma in childhood

A

Rhabdomyosarcoma

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29
Q

Rhabdomyosarcoma

A
  • 2/3 occur in children younger than 10

- Patients with neurofibramotsis at higher risk of developing these

30
Q

Clinical features of rhabdomyosarcoma

A
  • Head and neck: most common sites; orbital tumors (propotosis, chemosis, eyelid swelling, cranial nerve palsies), nasopharyngeal tumors (epistaxis, airway obstruction and chronic sinusitis), laryngeal tumors (hoarseness)
  • Genitourinary tract - hematuria, urinary tract obstruction, vaginal bleeding, or abdominal mass
  • Extremities - painless growing mass
  • Other sites of involvement include the trunk, retroperitoneum, mediastinum, and peritesticular and perianal regions
31
Q

Most common malignant bone tumor

A
  1. Osteogenic sarcoma

2. Ewing’s sarcoma

32
Q

Osteogenic sarcoma

A
  • Peak incidence during rapid growth spurts
  • Males commonly affected
  • Associated with retinoblastoma, Paget’s disease of bone, radiation therapy and fibrous dysplasia
33
Q

Clinical features of osteogenic sarcoma

A
  • 50% at knee
  • Locations: 1. distal femur, 2. proximal tibia, 3. proximal humerus, 4. proximal femur
  • Occur in metaphysis of tubular long bones
34
Q

Ewing’s sarcoma

A
  • Most commonly occurs during adolescence

- Characterized as a small, round, blue cell tumor (undifferentiated, monomorphous cell appearance)

35
Q

Clinical features of Ewing’s sarcoma

A
  • Occurs at flat bones and diaphysis of tubular bones
  • Occasionally extraosseous
  • Locations: 1. axial skeleton, 2. humerus, 3. femur
36
Q

Most common liver tumor in childhood

A

Hepatoblastoma

37
Q

Hepatoblastoma

A
  • Almost always occurs before 3 years

- Associated with Beckwith-Wiedmann syndrome

38
Q

Hepatocellular carinoma

A
  • Occurs in young children and adolescents
  • Associated with chronic active HepB infection, biliary atresia, glycogen storage disease type I, alpha-1 anti-trypsin deficiency, hereditary tyrosinemia
39
Q

Clinical features of hepatoblastoma and hepatocellular carcinoma

A
  • Right upper abdominal mass
  • Loss of appetite
  • Weight loss
  • Jaundice generally absent
40
Q

Teratoma

A
  • Contain more than 1 of 3 of the 3 primary germ cell layers (ectoderm, mesoderm and endoderm)
  • Mature often contain skin, hair or teeth
  • Immature often contain fetal or embryonal type structures
41
Q

Most common teratoma during 1st year of life

A

Sacrococcygeal teratoma

42
Q

Sacrococcygeal teratoma

A
  • Most common teratoma in 1st year of life
  • Majority in females
  • Arise from coccyx and present as soft tissue mass
  • Most benign
  • Surgical excision of both tumor and coccyx to prevent recurrence
43
Q

Anterior mediastinal teratoma

A
  • Generally benign

- Presents with signs and symptoms of airway obstruction

44
Q

Ovarian teratoma

A
  • Most common ovarian tumor
  • Generally benign
  • Suggested by presence of calcium within the tumor
45
Q

Most common testicular tumors

A
  1. Yolk sac
  2. Teratomas
  3. Seminomas
  4. Embryonal carcinoma
46
Q

Disorders of langerhans cell histiocytosis

A
  • Eosinophilic granuloma
  • Hand-Schuller-Christian disease
  • Letterer-Siwe disease
47
Q

Clinical features of langerhans cell histiocytosis

A
  • Skeletal involvement: skull most common, single or multiple bony lesions, pathologic fracture, chronic draining ears (involvement of mastoid)
  • Skin involvement: seborrheic dermatitis of the diaper area and scalp
  • Pituitary or hypothalamic involvement
  • Lymphadenopathy
  • Hepatosplenomegaly
  • Exophthalmos
  • Anemia
  • Pulmonary infiltrates
  • Nonspecific symptoms include weight loss, fatigue, fever and failure to thrive
48
Q

Down syndrome

A

Leukemia (ALL or AML)

49
Q

Turner syndrome

A

Gonadoblastoma

50
Q

Trisomy 13

A
  • Leukemia

- Teratoma

51
Q

Trisomy 18

A
  • Wilms’ tumor

- Neurogenic tumors

52
Q

Klinefelter syndrome

A
  • Leukemia
  • Germ cell tumors
  • Breast cancer
53
Q

Fanconi anemia

A

Leukemia

54
Q

Xeroderma pigmentosa

A
  • Basal and squamous cell carcinoma

- Melanoma

55
Q

Ataxia telangiectasia

A
  • Hodgkin’s and non-Hodgkin’s lymphoma
  • Leukemia
  • Sarcomas
56
Q

Bloom syndrome

A
  • Leukemia
  • Lymphomas
  • Gastrointestinal malignancies
  • Solid tumors
57
Q

Beckwith-Wiedmann syndrome

A
  • Wilms’ tumor
  • Hepatoblastoma
  • Rhabdomyosarcoma
  • Adrenocortical carcinoma
58
Q

Neurofibromatosis I

A
  • Brain tumors
  • Lymphoma
  • Leukemia
  • Malignant schwannoma
59
Q

Neurofibromatosis II

A

Acoustic neuroma

60
Q

Most common childhood cancer

A

Acute lymphocytic leukemia (ALL)

61
Q

Classification of ALL

A

Cell morphology:

  • L1: lymphocytes are small with little cytoplasm and indistinct nucleoli, most common
  • L3: large, one or more nucleoli

Immunophenotype:

  • T cell phenotype: 25%
  • B cell phenotype: less than 5%
  • Pre-B cell phenotype: 70% - can be CALLA positive (70%) or CALLA negative (30%)

CALLA = common acute lymphocytic leukemia antigen

62
Q

Clinical features of ALL

A
  • Fever and bone or joint pain (most common symptoms)
  • Pallor, bruising, hepatosplenomegaly and lymphadenopathy (most common signs)
  • Epistaxia, anorexia, fatigue, testicular pain, swelling, and abdominal pain
63
Q

Diagnosis of ALL

A

Bone marrow evaluation demonstrating marrow replacement by lymphoblasts. Other normal marrow elements are decreased or absent.

64
Q

Stages of management of ALL

A
  1. Induction
    - Aims to destroy as many cancer cells as possible to induce remission
    - Steroids, vincristine, L-asparaginase
    - Intrathecal methotrexate
  2. Consolidation
    - Continuation of systemic chemotherapeutic agents and prophylactic regimens to prevent CNS involments
    - Intrathecal methotrexate
    - Cranial irradiation to high risk kids
  3. Maintenance
    - Daily and periodic chemo for up to 3 years
65
Q

Tumor lysis syndrome

A
  • Hyperuricemia: may result in renal insufficiency
  • Hyperkalemia: may result in cardiac dysrhythmias
  • Hyperphosphatemia: may result in hypocalcemia with tetany
66
Q

Classification of AML

A
  • M1: acute myeloblastic leukemia (no maturation)
  • M2: acute myeloblastic leukemia (some maturation)
  • M3: acute promyelocytic leukemia (Auer rods common)
  • M4: acute myelomonocytic leukemia
  • M5: acute monocytic leukemia
  • M6: erythroleukemia
  • M7: acute megakaryocytic leukemia (associated with Down syndrome)
67
Q

Clinical features of AML

A
  • CNS involvement
  • Fever
  • Hepatosplenomegaly
  • Bruising and bleeding
  • Gingival hypertrophy
  • Bone pain
  • Lymphadenopathy and testicular involvement are uncommon
68
Q

What clinical feature is more common in AML than ALL

A

CNS involvement

69
Q

Clinical features of adult-type CML

A
  • Fatigue, weight loss, and pain
  • Massive splenomegaly
  • Extremely high WBC count
70
Q

Clinical features of juvenile CML

A
  • Fever
  • Chronic eczemalike facial rash
  • Supportive lymphadenopathy
  • Petechiae and purpura
  • Moderate leukocytosis
71
Q

Management of CML

A

Bone marrow transplant

Pediatrics (11 decks)

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