47 Skeletal Malignancies and Related Disorders

Question 1

MC neoplastic mimic of JIA

Answer 1

ALL

Question 2

T/F Bone pain is common in recent-onset JIA

Answer 2

F, unusual

Question 3

(+) ANA can differentiate ALL from JIA

Answer 3

F

Question 4

Full blood exam may be entirely normal in ALL but frequently shows

Answer 4

Mild normocytic anemia

Question 5

Why is it important to perform all appropriate diagnostic investigations for patients suspected of having ALL before initiation of any therapy

Answer 5

Treatment with steroids or MTX for a presumptive JIA may result in amelioration of symptoms in a child with ALL and obscure vital prognostic information (therapy regimens are “risk adapted” in ALL)

Question 6

Bone tumor presenting with pain which is classically nocturnal and reduced by aspirin and NSAIDs

Answer 6

Osteoid osteoma

Question 7

MC malignant bone tumor in the pediatric age group

Answer 7

Osteosarcoma

Question 8

Classic radiological finding in osteosarcoma appearing as a triangle of immature bone

Answer 8

Codman’s sign

Question 9

Second MC primary bone tumor in children and adolescents

Answer 9

Ewing sarcoma

Question 10

Predominant site of metastasis of malignant bone tumors

Answer 10

Lungs

Question 11

Gender predilection of malignant bone tumors

Answer 11

M

Question 12

Classic appearance of Ewing sarcoma in plain radiographs

Answer 12

Onion-skin appearance

Question 13

ES vs OS: Cortical breach

Answer 13

Both

Question 14

Appears as a pedunculated lesion from the bone on plain radiograph

Answer 14

Osteochondroma

Question 15

T/F Prophylactic excision is recommended for osteochondroma

Answer 15

F, malignant transformation is rare and surgery is only indicated if the lesions are causing pain or if significant growth disturbances

Question 16

Benign hyaline cartilage neoplasms of medullary bone, generally affecting adolescents

Answer 16

Enchondroma

Question 17

Enchondroma with associated soft tissue hemangioma

Answer 17

Maffucci syndrome

Question 18

Benign fibrous tissue tumor that are generally asymptomatic, resolve spontaneously, and diagnosis is usually an incidental finding on plain radiograph taken for other reasons

Answer 18

Fibrous cortical defect (nonossifying fibroma)

Question 19

A locally aggressive synovial proliferative disorder of unknown etiology affecting joints, tendon sheaths, and bursae that can present as a painful monoarticular effusion predominantly affecting the knee

Answer 19

Pigmented Villonodular Synovitis

Question 20

PVNS should be considered, particu- larly in adolescents, if (2)

Answer 20

1. A joint aspirate suggests hemarthrosis
   AND/OR
2. Response to intraarticular corticosteroid injection is poor

Question 21

Benign tumor of soft tissue seen histologically as thickened, reddish-brown synovium resulting from hemosiderin deposition, with numerous villous projections

Answer 21

PVNS

Question 22

A serious complication in both vascular malformations and hemangiomata

Answer 22

Development of a consumptive coagulopathy with resultant severe thrombocytopenia (Kasabach–Merritt phenomenon)

Question 22

A serious complication in both vascular malformations and hemangiomata

Answer 22

Development of a consumptive coagulopathy with resultant severe thrombocytopenia (Kasabach–Merritt phenomenon)