What is the most common primary malignant bone tumor in children?
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
A 15 Yo M presents with a PMH of injury to his L thigh with subsequent:
-Pain and swelling over the metaphyses of the L femur
-"Sunburst" pattern on x-ray
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, a
A 15 Yo Caucasian M presents with:
-Primary lytic lesion with "onion skinning" of the diaphysis of the R femur on x-ray
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
What is the translocation seen in a majority of patients with Ewing sarcoma?
A majority of the patients with Ewing sarcoma have a t(11;22) translocation, while the rest have a t(21;22) translocation.
A 10 Yo M 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
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 t
A 10 Yo M presents with:
-Unremitting and worsening pain of his proximal L femur
-Pain is worse at night
-Palpation and range of motion do not worsen the pain
-On xray, a round metaphyseal lucency surrounded by sclerotic bone
-ASA relieves the pain
Osteoid osteomas are benign tumors that generally occur between 5 and 20 years of age in boys. About 25% cannot be seen on plain xray but can be seen on CT scan. Treat by excising the lesion.
What syndrome presents with these findings?
-Maternal breast cancer
-Sarcoma in children
-Germline mutations in the p53 gene
-CNS tumors: gliomas, ependymomas, and choroid plexus carcinomas
Li-Fraumeni Syndrome is a familial cancer syndrome associated with an increased risk of gliomas, epndymomas, and choroid plexus carcinomas.
Name the disease associated with hemagioblastomas in the cerebellum, medulla, and spinal cord.
von Hippel-Lindau Disease
VHL 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.
What is the syndrome associated with this triad?
-impaired upward gaze
-dilated pupils with better reactivity to accommodation than to light
-retraction or conversion nystagmus with lid retraction
Parinaud syndrome is caused by compression or infiltration of the midbrain tectum, particularly with pineal tumors.
What is the most common type of malignant CNS tumor in childhood?
Primitive Neuroectodermal Tumors
Primitive neuroectodermal tumors are the most common type of malignant CNS tumor in childhood; medulloblastomas account for most of these.
What is the most common type of CNS tumor in childhood?
Gliomas make up 50-60% of all brain tumors and are the most common primary childhood CNS tumors. They can be astrocytomas or gliomas.
Which is the most common posterior fossa tumor of childhood?
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 astrocytoma
What type of brain tumor is benign, but because of its invasive growth, commonly impinges on many structures, including those listed above?
-CN 3 (oculomotor nerve)
Craniopharyngiomas are benign tumors that are derived from squamous epithelial cells and arise in the suprasellar region. More thatn 50% of children with craniopharyngioma have visual changes due to optic involvement. Because the pituita
Which is the most common malignant childhood germ cell tumor?
Endodermal Sinus (Yolk Sac) Tumor
Yolk sac tumor most often occurs in the infantile testes, ovary, or sacrococcyx. AFP is a very reliable tumor marker whereas b-hCG is absent.
Which is the most common benign tumor of the liver in childhood?
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.
What histologic feature(s) is/are required for the diagnosis of Langerhans cell histiocytosis (LCH)?
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.
What major side effect do you worry about with use of vincristine?
Closely monitor patients on the chemotherapy drug vincristine, and be on the lookout for foot drop, constipation, poor fine motor control, and neuropathic pain.
What is the most common malignancy in infants?
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 at <18 months of age.
A 20 month old presents with ataxia and myoclonic jerking with random eye movements.
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.
What is the most common primary malignant tumor of the kidney in childhood?
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.
hemihyperplasia, GU anomlaies, sporadic
What is the most common congenital renal disorder?
Mesoblastic nephroma presents as a firm, solitary mass of the kidney. It looks like a leiomyoma. It is benign and resection is curative.
What is the most common soft tissue tumor of childhood?
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 loc
benign bone tumor associated with dull persistent pain worse at night
what improves pain of osteoid osteoma
salicylates and NSAIDS, NOT acetaminophen
most common location for osteoid osteoma
proximal femur, but also proximal tibia, humerus, posterior spine
osteoid osteoma on plain film
radiolucent, sharp, rounded lesion < 2 cm surrounded by a reactive sclerosis in the cortex of the bone
how does osteochondroma present clinically
hard, immobile, painless mass
where on bone does a chrondroblastoma usually affect
epiphyses of long bones
sclerotic destruction of bone resembling a sunburst appearance describes
intracytoplasmic inclusion bodies that appear as rod or tennis racquet shaped structures describe
birbeck granules, seen in langerhans cell histiocytosis
unilateral or bilateral proptosis and and ecchymosis is seen in metastasis of what cancer to the orbit
pituitary dysfunction in langerhans cell histiocytosis leads to
diabetes insipidus, inability to concentrate urine leading to decreased urine osmolarity
radiographs showing a broad, stalk-like projection from the involved bone that angled away from adjacent joint describes which bone tumor
sunburst pattern and codman triangle describe which bone tumor
bone tumor characterized by periosteal elevation or onion skinning on plain radiograph
bone tumor with radiograph showing well circumscribed metaphyseal or diaphyseal lucency surrounded by sclerotic bone
what ages does osteochondroma typically present
between 5 and 15 years
mass composed of cartilage cap on a sessile or pedunculated bony base that protrudes from cortex of the bone
what do you expect with children who receive cranial radiation at a young age (<3)
profound neurocognitive problems, unable to live independently
next best step after solid testicular mass concerning for testicular cancer is found
obtain tumor markers (then CT/MRI to look for mets, then surgery to remove affected testicle)
beta HCG is elevated in which testicular tumors
choriocarcinoma, mixed germ cell tumors
alpha-fetoprotein is elevated in which testicular tumors
yolk sac tumors, embryonal carcinoma
calcifications within a tumor on CT is typically associated with which tumor: wilms or neuroblastoma
tumor that sometimes secretes vasoactive intestinal peptide (VIP) and causes significant diarrhea
tumor with finding on CT scan of a mass arising from the right kidney (claw sign) and extending into the right renal vein
most common sites of involvement for langerhans cell histiocytosis
skull, femur, ribs, vertebra, humerus
cortical and medullary bone growth with cauliflower like appearance
exostosis, usually osteochondroma
30% of hodgkin lymphoma survivors are diagnosed with what later in life
pulmonary fibrosis is associated with which chemotherapeutic treatments
bleomycin and busulfan
oval metaphyseal lucency surrounded by sclerotic bone describes radiographic findings associated with
what presents as a sharply marginated eccentric lucency in the metaphyseal cortex on radiograph
metaphyseal fibrous defect/fibroma
what presents as sharply marginated radiolucencies within the epiphysis on radiograph
a radiolucent sharply marginated lesion noted on XR of the hand describes
enchondroma - benign bone tumor arising from cartilage
eccentric lytic destruction and expansion of the metaphysis of the tibia bordered by a thin sclerotic border describes
aneurysmal bone cyst
a lytic ground glass appearing expansile lesion of the metaphysis of the femur describes
where will wilms tumor frequently metastasize to
where will ewing sarcoma most likely metastasize to
how does osteosarcoma commonly present as metastatic disease
to the lung
what malignancy is associated with wiskott-aldrich syndrome, ataxia-telangiectasia, HIV and other immunosuppressive diseases
non-hodgkin lymphoma (like Burkitts)
where do must cases of burkitt lymphoma originate
peyer patches in GI tract, at ileocecal junction
how does hodgkin lymphoma typically present
B symptoms at presentation - unexplained weight loss greater than 10%, drenching night sweats, fever greater than 38
biopsy results revealing osteoid production by spindle cells is a pathological feature of
next step for back pain in a child with radicular symptoms - tingling, change in sensation, weakness, bowel/bladder dysfuction
considered an emergency and pathologic - get emergent spinal MRI
tumor associated with prematurity, beckwith-wiedemann, and hemihypertrophy
Patients treated with intrathecal methotrexate are at increased risk for developing