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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
WAGR syndrome
- Wilms' tumor - Aniridia - Genitourinary abnormalities - Mental retardation
26
Clinical features of Wilms' tumor
- Abdominal mass that rarely crosses midline - Abdominal pain with or without vomiting - Hematuria - Hypertension - Nonspecific findings include fever, anorexia, and weight loss
27
Staging of Wilms' tumor
- 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
28
Most common soft tissue sarcoma in childhood
Rhabdomyosarcoma
29
Rhabdomyosarcoma
- 2/3 occur in children younger than 10 | - Patients with neurofibramotsis at higher risk of developing these
30
Clinical features of rhabdomyosarcoma
- 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
Most common malignant bone tumor
1. Osteogenic sarcoma | 2. Ewing's sarcoma
32
Osteogenic sarcoma
- Peak incidence during rapid growth spurts - Males commonly affected - Associated with retinoblastoma, Paget's disease of bone, radiation therapy and fibrous dysplasia
33
Clinical features of osteogenic sarcoma
- 50% at knee - Locations: 1. distal femur, 2. proximal tibia, 3. proximal humerus, 4. proximal femur - Occur in metaphysis of tubular long bones
34
Ewing's sarcoma
- Most commonly occurs during adolescence | - Characterized as a small, round, blue cell tumor (undifferentiated, monomorphous cell appearance)
35
Clinical features of Ewing's sarcoma
- Occurs at flat bones and diaphysis of tubular bones - Occasionally extraosseous - Locations: 1. axial skeleton, 2. humerus, 3. femur
36
Most common liver tumor in childhood
Hepatoblastoma
37
Hepatoblastoma
- Almost always occurs before 3 years | - Associated with Beckwith-Wiedmann syndrome
38
Hepatocellular carinoma
- 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
Clinical features of hepatoblastoma and hepatocellular carcinoma
- Right upper abdominal mass - Loss of appetite - Weight loss - Jaundice generally absent
40
Teratoma
- 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
Most common teratoma during 1st year of life
Sacrococcygeal teratoma
42
Sacrococcygeal teratoma
- 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
Anterior mediastinal teratoma
- Generally benign | - Presents with signs and symptoms of airway obstruction
44
Ovarian teratoma
- Most common ovarian tumor - Generally benign - Suggested by presence of calcium within the tumor
45
Most common testicular tumors
1. Yolk sac 2. Teratomas 3. Seminomas 4. Embryonal carcinoma
46
Disorders of langerhans cell histiocytosis
- Eosinophilic granuloma - Hand-Schuller-Christian disease - Letterer-Siwe disease
47
Clinical features of langerhans cell histiocytosis
- 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
Down syndrome
Leukemia (ALL or AML)
49
Turner syndrome
Gonadoblastoma
50
Trisomy 13
- Leukemia | - Teratoma
51
Trisomy 18
- Wilms' tumor | - Neurogenic tumors
52
Klinefelter syndrome
- Leukemia - Germ cell tumors - Breast cancer
53
Fanconi anemia
Leukemia
54
Xeroderma pigmentosa
- Basal and squamous cell carcinoma | - Melanoma
55
Ataxia telangiectasia
- Hodgkin's and non-Hodgkin's lymphoma - Leukemia - Sarcomas
56
Bloom syndrome
- Leukemia - Lymphomas - Gastrointestinal malignancies - Solid tumors
57
Beckwith-Wiedmann syndrome
- Wilms' tumor - Hepatoblastoma - Rhabdomyosarcoma - Adrenocortical carcinoma
58
Neurofibromatosis I
- Brain tumors - Lymphoma - Leukemia - Malignant schwannoma
59
Neurofibromatosis II
Acoustic neuroma
60
Most common childhood cancer
Acute lymphocytic leukemia (ALL)
61
Classification of ALL
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
Clinical features of ALL
- 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
Diagnosis of ALL
Bone marrow evaluation demonstrating marrow replacement by lymphoblasts. Other normal marrow elements are decreased or absent.
64
Stages of management of ALL
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
Tumor lysis syndrome
- Hyperuricemia: may result in renal insufficiency - Hyperkalemia: may result in cardiac dysrhythmias - Hyperphosphatemia: may result in hypocalcemia with tetany
66
Classification of AML
- 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
Clinical features of AML
- CNS involvement - Fever - Hepatosplenomegaly - Bruising and bleeding - Gingival hypertrophy - Bone pain - Lymphadenopathy and testicular involvement are uncommon
68
What clinical feature is more common in AML than ALL
CNS involvement
69
Clinical features of adult-type CML
- Fatigue, weight loss, and pain - Massive splenomegaly - Extremely high WBC count
70
Clinical features of juvenile CML
- Fever - Chronic eczemalike facial rash - Supportive lymphadenopathy - Petechiae and purpura - Moderate leukocytosis
71
Management of CML
Bone marrow transplant

Pediatrics (11 decks)

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