Chapter·PediatricsCommon pediatric cancers

OsteosarcomaDownloads

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1

A 62-year-old retired professor comes to the clinic with the complaints of back pain and increasing fatigue over the last 4 months. For the past week, his back pain seems to have worsened. It radiates to his legs and is burning in nature, 6/10 in intensity. There is no associated tingling sensation. He has lost 4.0 kg (8.8 lb) in the past 2 months. There is no history of trauma. He has hypertension which is well controlled with medications. Physical examination is normal. Laboratory studies show normocytic normochromic anemia. Serum calcium is 12.2 mg/dL and Serum total proteins is 8.8 gm/dL. A serum protein electrophoresis shows a monoclonal spike. X-ray of the spine shows osteolytic lesions over L2–L5 and right femur. A bone marrow biopsy reveals plasmacytosis. Which of the following is the most preferred treatment option?

ARenal dialysis

BPalliative care

CChemotherapy and autologous stem cell transplant

DBisphosphonates

EChemotherapy alone

2

An investigator studying targeted therapy in patients with gastrointestinal stromal tumors requires a reliable test to determine the spatial distribution of CD117-positive cells in biopsy specimens. Which of the following is the most appropriate test?

ANorthern blot

BImmunohistochemistry

CFlow cytometry

DFluorescence in-situ hybridization

EWestern blot

3

A 12-year-old Caucasian male presents with his mother to the pediatrician’s office complaining of right thigh pain. He reports that he has noticed slowly progressive pain and swelling over the distal aspect of his right thigh over the past two months. He denies any recent trauma to the area and his temperature is 100.9°F (38.3°C). On exam, there is swelling and tenderness overlying the distal right femoral diaphysis. Laboratory evaluation is notable for an elevated white blood cell (WBC) count and elevated erythrocyte sedimentation rate (ESR). A radiograph of the patient’s right leg is shown. Biopsy of the lesion demonstrates sheets of monotonous small round blue cells with minimal cytoplasm. Which of the following genetic mutations is most likely associated with this patient’s condition?

At(11;22)

BRB1 inactivation

CTP53 inactivation

Dt(8;14)

Et(X;18)

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