47 Skeletal Malignancies and Related Disorders Flashcards
MC neoplastic mimic of JIA
ALL
T/F Bone pain is common in recent-onset JIA
F, unusual
(+) ANA can differentiate ALL from JIA
F
Full blood exam may be entirely normal in ALL but frequently shows
Mild normocytic anemia
Why is it important to perform all appropriate diagnostic investigations for patients suspected of having ALL before initiation of any therapy
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)
Bone tumor presenting with pain which is classically nocturnal and reduced by aspirin and NSAIDs
Osteoid osteoma
MC malignant bone tumor in the pediatric age group
Osteosarcoma
Classic radiological finding in osteosarcoma appearing as a triangle of immature bone
Codman’s sign
Second MC primary bone tumor in children and adolescents
Ewing sarcoma
Predominant site of metastasis of malignant bone tumors
Lungs
Gender predilection of malignant bone tumors
M
Classic appearance of Ewing sarcoma in plain radiographs
Onion-skin appearance
ES vs OS: Cortical breach
Both
Appears as a pedunculated lesion from the bone on plain radiograph
Osteochondroma
T/F Prophylactic excision is recommended for osteochondroma
F, malignant transformation is rare and surgery is only indicated if the lesions are causing pain or if significant growth disturbances
Benign hyaline cartilage neoplasms of medullary bone, generally affecting adolescents
Enchondroma
Enchondroma with associated soft tissue hemangioma
Maffucci syndrome
Benign fibrous tissue tumor that are generally asymptomatic, resolve spontaneously, and diagnosis is usually an incidental finding on plain radiograph taken for other reasons
Fibrous cortical defect (nonossifying fibroma)
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
Pigmented Villonodular Synovitis
PVNS should be considered, particu- larly in adolescents, if (2)
- A joint aspirate suggests hemarthrosis
AND/OR - Response to intraarticular corticosteroid injection is poor
Benign tumor of soft tissue seen histologically as thickened, reddish-brown synovium resulting from hemosiderin deposition, with numerous villous projections
PVNS
A serious complication in both vascular malformations and hemangiomata
Development of a consumptive coagulopathy with resultant severe thrombocytopenia (Kasabach–Merritt phenomenon)
A serious complication in both vascular malformations and hemangiomata
Development of a consumptive coagulopathy with resultant severe thrombocytopenia (Kasabach–Merritt phenomenon)