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Med 16-17 > Oncology > Flashcards

Oncology Flashcards

1
Q

Name the syndrome:

  • Affects children most commonly between 2 and 5 years of age
  • Skull lesions
  • Diabetes insipidus
  • Exophthalmos
  • MRI shows abnormalities of the posterior pituitary gland and thickening of the pituitary stalk
  • If in an infant, you’ll see:
    • Erosion of the lamina dura of the teeth
    • Premature eruption of teeth
    • Gingival hemorrhage
    • Oral mucosal irritation
    • Seborrheic rash of the scalp and posterior auricular area
A

Multifocal Langerhans Cell Histiocytosis

(Hand-Schuller-Christian Disease)

It is confirmed by finding CD1a by immunophenotyping or Birbeck granules on electron microscopy. There are 3 types of Langerhans cell Histiocytosis: unifocal, multifocal (described here), and systemic (formerly known as Letterer-Siwe disease).

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2
Q

What is the most common type of lymphoma to occur in children?

A

Non-Hodgkin Lymphoma (NHL)

Males outnumber females 3:1. There is a high rate of NHL in children with ataxia-telangiectasia, Wiskott-Aldrich syndrome, HIV, and other immunosuppression diseases.

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3
Q

What is the Philadelphia chromosome translocation?

A

t(9:22)

It occurs in chronic myeloid leukemia (CML) and is a disorder of the pluripotent stem cell defined by the t(9:22) translocation. This results in the juxtaposition of the bcr gene on chromosome 22 with the abl gene on chromosome 9, causing a fusion gene that encodes for a bcr-abl abnormal protein.

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4
Q

Which type of non-Hodgkin lymphoma presents with lymphadenopathy that is tender?

A

Large Cell Non-Hodgkin Lymphoma

It is usually associated with tender lymphadenopathy and can commonly be seen with constitutional symptoms (e.g., fever, sweats, weight loss).

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5
Q

What is the most common childhood malignancy?

A

Acute Lymphoblastic Leukemia (ALL)

Luckily it is one of the most curable cancers today. It’s peak incidence is between 2 and 5 years of age.

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6
Q

What major side effect do you worry about with use of vincristine?

A

Neuropathy

Monitor patients on the chemotherapy drug vincristine closely, and be on the lookout for foot drop, constipation, poor fine motor control, and neuropathic pain.

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7
Q

A 15-year-old Caucasian boy presents with:

  • Fever
  • Weight loss
  • Rib pain
  • X-ray shows a primary lytic lesion with “onion-skinning” of the diaphysis of the right femur.

What is the most likely diagnosis?

A

Ewing Sarcoma

Ewing sarcoma is an undifferentiated sarcoma of bone. It is more likely to affect the diaphyses of long bones and flat bones (ribs, pelvis). Be sure to differentiate this from osteosarcoma (metaphyseal involvement and “sunburst” pattern on x-ray). Ewing’s is more commonly seen in Caucasians.

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8
Q
  • Impaired upward gaze
  • Dilated pupils with better reactivity to accommodation than to light
  • Retraction or conversion nystagmus with lid retraction

What is the syndrome associated with this triad?

A

Parinaud Syndrome

Parinaud syndrome is caused by compression or infiltration of the midbrain tectum, particularly with pineal tumors.

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9
Q

Name the leukemia:

  • Manifests before the age of 2 years
  • Markedly enlarged spleen
  • Modest leukocytosis
  • Thrombocytopenia
  • Elevated fetal hemoglobin
  • Xanthoma
  • Cafe-au-lait spots
  • Eczema
  • No Philadelphia chromosome
  • Monosomy 7 in 30% of patients
A

Juvenile Myelomonocytic Leukemia (JMML)

There is no blast crisis, but the 5-year survival rate without bone marrow transplant is < 10%. Children with neurofibromatosis Type 1 are at increased risk for JMML.

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10
Q

For all ages in pediatrics, what is the most common primary malignant bone tumor? What about for children < 10 years of age?

Which is the most common?

A

Osteosarcoma; Ewing Sarcoma

Osteosarcoma is the most common primary malignant tumor in children and adolescents overall; but in children < 10 years of age, Ewing sarcoma is #1. Both commonly present during the 2nd decade.

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11
Q

What is the most common malignant germ cell tumor?

A

Endodermal Sinus (Yolk Sac) Tumor

Endodermal sinus (yolk sac) tumor most often occurs in the infantile testes, ovary, or sacrococcyx. AFP is a very reliable tumor marker, whereas β-hCG is absent.

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12
Q

A 10-year-old boy presents with:

  • Unremitting and worsening pain of his proximal left femur
  • The pain is worse at night
  • Palpation and range of motion do lot worsen the pain.
  • X-ray shows a round metaphyseal lucency surrounded by sclerotic bone.
  • Aspirin relieves the pain.

What is the most likely diagnosis?

A

Osteoid Osteoma

Osteoid osteomas are benign tumors that generally occur between 5 and 20 years of age in boys. About 25% cannot be seen on plain x-ray but can be seen on CT scan. Treat by excising the lesion.

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13
Q

What is the most common congenital renal disorder?

A

Mesoblastic Nephroma

Mesoblastic nephroma presents as a firm, solitary mass of the kidney. It looks like a leiomyoma. It is benign and resection is curative.

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14
Q

What is the most common type of CNS tumor in childhood?

A

Gliomas

Gliomas make up 50–60% of all brain tumors and are the most common primary childhood CNS tumors. They can be astrocytomas or gliomas.

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15
Q

A 17-year-old boy has the following:

  • Asymptomatic enlarged cervical lymph node noticed by his barber
  • No other symptoms
  • He has no cat exposure,
  • 1 week of cephalexin therapy does not result in decrease in the size of the gland and if anything, it is slightly larger.
  • But he had no new symptoms.

What do you recommend at this point?

A

Excisional Biopsy of the Node

He is in the age group that is at higher risk for Hodgkin lymphoma. An isolated enlarging lymph node with no cat exposure or other risk factors unfortunately raises the possibility of Hodgkin’s. Excisional biopsy to examine the architecture of the node is recommended.

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16
Q

What is the most common malignancy in infants?

A

Neuroblastoma

Neuroblastoma makes up 8–10% of all childhood cancers and is the most common malignancy in infants. Median age of diagnosis is 22 months of age. Best prognosis is seen in children diagnosed < 18 months of age. (Acute lymphoblastic leukemia [ALL] is the single most common childhood malignancy, but its incidence peaks between 2 and 5 years of age.)

17
Q

Name the syndrome:

  • Maternal breast cancer
  • Sarcoma in children
  • Adrenocortical carcinoma
  • Germline mutations in the p53 gene
  • CNS tumors: gliomas, ependymomas, and choroid plexus carcinomas
A

Li-Fraumeni Syndrome

Li-Fraumeni syndrome is a familial cancer syndrome associated with an increased risk of gliomas, ependymomas, and choriod plexus carcinomas.

18
Q

An adolescent boy presents with:

  • Anterior mediastinal mass
  • Nontender cervical and supraclavicular nodes
  • Hepatosplenomegaly

You suspect non-Hodgkin lymphoma.

What cell type is this likely originating from?

A

Thymic T-cell Origin

Non-Hodgkin tumors that present as mediastinal masses are almost always lymphoblastic lymphomas and are of thymic T-cell origin. These are commonly seen in adolescent males.

19
Q

A 15-year-old boy presents with history of injury to his left thigh with subsequent:

  • Pain and swelling over the metaphyses of his left femur
  • Nighttime awakening
  • Palpable mass
  • X-ray shows a “sunburst” pattern

What is the most likely diagnosis?

A

Osteosarcoma

Osteosarcoma typically occurs in the metaphyseal region of long bones, and commonly children think they have injured themselves while playing sports. Differentiate this from Ewing’s, which involves the diaphyses of long bones and flat bones, as well as x-ray findings that show lytic lesions or “onion skinning.”

20
Q

A child presents with an orbital chloroma and hepatosplenomegaly.

What is the most likely diagnosis?

A

Acute Myeloid Leukemia (AML)

An orbital or epidural chloroma, which is a localized mass of leukemic cells, may be the 1st clue of AML. Hepatosplenomegaly can occur. Anemia (median Hgb 7 g/dL) and thrombocytopenia are almost always present. Auer rods seen on histology.

21
Q

An adolescent receives mantle radiation for his Hodgkin disease.

Name a long-term side effect of radiation therapy that affects the heart.

A

Early-onset Coronary Artery Disease

Be familiar with the long-term effects of radiation therapy, especially those listed here:

  • growth retardation,
  • early-onset coronary artery disease,
  • pulmonary fibrosis, and
  • increased risk of breast cancer.
22
Q
  • Optic chiasm
  • Carotid arteries
  • CN 3 (oculomotor nerve)
  • Pituitary stalk

What type of brain tumor is benign, but because of its invasive growth, commonly impinges on many structures, including those listed above?

A

Craniopharyngioma

Craniopharyngiomas are benign tumors that are derived from squamous epithelial cells and arise in the suprasellar region. More than 50% of children with craniopharyngioma have visual changes due to optic involvement. Because the pituitary is commonly involved, look for growth failure, short stature, and polydipsia as presenting signs.

23
Q

What major side effect do you worry about with use of doxorubicin?

A

Cardiomyopathy

This is the main concern with doxorubicin or daunorubicin.

24
Q

Which is the most common posterior fossa tumor of childhood?

A

Cerebellar Astrocytoma

Cerebellar astrocytomas make up 12% of all brain tumors in children and are the most common posterior fossa tumors of childhood. They have one of the best prognoses (> 90% 5-year survival). Symptoms of lateral cerebellar astrocytomas include clumsiness and unsteadiness of the arms and legs. Headaches and vomiting also occur.

25
Q

A child presents with:

  • Nausea and vomiting
  • Abdominal mass and pain in the ileocecal junction area
  • Fever
  • CT scan of the abdomen confirms a mass at the ileocecal junction.

What is the most likely diagnosis?

A

Burkitt Lymphoma

It is the most common form of non-Hodgkin lymphoma, and nearly all originate from relatively mature B cells in Peyer patches within the GI tract, most commonly at the ileocecal junction. Jaw involvement is very common in the African form, but only occurs in 15% of U.S. cases.

26
Q

Name the syndrome associated with hemangioblastomas in the cerebellum, medulla, and spinal cord.

A

von Hippel-Lindau (VHL) Disease

VHL disease increases the risk of hemangioblastomas in the CNS and is most often an autosomal-dominant inherited disorder (80%), but 20% of cases have no family connection.

27
Q

What is the most common soft tissue tumor of childhood?

A

Rhabdomyosarcoma

Rhabdomyosarcoma makes up about 5% of all childhood cancers. It arises from the same embryonic mesenchyme as striated skeletal muscle. Mass lesions of the head and neck, including the orbit and parameningeal sites, are the most common location, followed by the genitourinary tract.

28
Q

A 3-year-old presents with:

  • Fatigue
  • Pallor
  • Painful limping

Yes, that is all you get…welcome to the ABP Board-like experience.

What is the diagnosis?

A

Acute Lymphoblastic Leukemia (ALL)

ALL commonly presents insidiously, especially on the Boards! Look out for the “4 Ps”: pallor, pyrexia, Purpura, and pain. I only gave you 2 Ps - sorry, the Boards do that, too. This is in the peak age for ALL; a 3-year-old who is fatigued with pallor, you always think of anemia - add in pain and you should be thinking ALL!

29
Q

A 10-year-old boy who is otherwise completely asymptomatic presents with:

  • Bony, nonpainful mass on his distal femur
  • On x-ray, “stalks” or “broad-based projections” from the surface of the bone, with an associated cartilage “cap” that is ∼ 0.5 cm thick

What is the most likely diagnosis?

A

Osteochondroma

Osteochondroma is a very common benign tumor of children. Most occur in the metaphysis of long bones, particularly the distal femur, proximal humerus, and proximal tibia. Leave these alone unless they cause symptoms, in which case, excise the lesion.

30
Q

What is the most common primary malignant bone tumor in children?

A

Osteosarcoma

Osteosarcoma occurs most often during the adolescent growth spurt, presenting with unilateral pain and swelling, usually in the metaphyseal region of long bones and in the medullary cavity. The knee joint is most typically affected, followed by the shoulder. Osteosarcoma is the most common primary malignant bone tumor in children and adolescents overall.

31
Q

A boy has a suspicious lymph node removed.

What is the classic histologic feature of Hodgkin disease seen on lymph node biopsy?

A

Reed-Sternberg Cell

This is the classic histologic hallmark of Hodgkin disease and is a large cell with multiple or multilobulated nuclei (looks like “owl’s eyes”).

32
Q

What are the most common type of malignant CNS tumors in childhood?

A

Primitive Neuroectodermal Tumors

Primitive neuroectodermal tumors are the most common type of malignant CNS tumor in childhood; medulloblastomas account for most of these.

33
Q

What is the most common primary malignant tumor of the kidney in childhood?

A

Wilms Tumor

Wilms tumor (nephroblastoma) is the most common primary malignant tumor of the kidney in childhood. The mean age of diagnosis is 42–47 months for unilateral tumors and 30–33 months for bilateral tumors.

34
Q

What is the most common benign tumor of the liver in childhood?

A

Hemangioendothelioma

Hemangioendothelioma is the most common benign liver tumor in childhood. Biopsy is not indicated. If there are no symptoms, no therapy is necessary because these lesions eventually regress.

35
Q

A 20-month-old presents with ataxia and myoclonic jerking with random eye movements.

What is the most likely diagnosis?

A

Neuroblastoma

Opsomyoclonus (myoclonic jerking and random eye movements) in association with ataxia occurs in about 5% of patients with neuroblastoma and may be the presenting symptom. The most common presentation, however, is a nontender abdominal mass.

36
Q

What is the translocation seen in a majority of patients with Ewing sarcoma?

A

t(11,22)

A majority of the patients with Ewing sarcoma have a t(11;22) translocation, while the rest have a t(21;22) translocation.

37
Q

What histologic feature(s) is/are required for the diagnosis of Langerhans cell histiocytosis (LCH)?

A

Intra/extracellular Staining with CD207 (Langerin) or

Extracellular Staining with CD1a

Diagnosis is confirmed by finding CD207 (langerin) or CD1a by immunohistochemistry. Birbeck granules are seen on electron microscopy.

Med 16-17 (27 decks)

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