Question 1
Which syndrome is defined as association with Ollier's disease
and hemangiomas?
a) Von Recklinghausen's disease of bone
b) Li-Fraumeni syndrome
c) Maffucci's syndrome
d) Hereditary multiple exostoses
e) Gardner's syndrome
Question 2
Which of the following is associated with high risk of osteosarcoma?
a) germline mutation of p53
b) translocation (X;18)
c) tanslocation (12;16)
d) 1p rearrangements
e) nonrandom translocation (9;22)
Question 3
Which of the following is NOT included in the differential of Ewing's sarcoma?
a) lymphoma
b) metastatic neuroblastoma
c) mesenchymal chondrosarcoma
d) leiomyosarcoma of bone
e) metastatic embryonal rhabdomyosarcoma
Question 4
12 year old boy with pain in his leg for past three months.
a) Non-ossifying fibroma (NOF).
b) Aneurysmal bone cyst (ABC)
c) Unicameral bone cyst (UBC)
d) Giant cell tumor (GCT)
e) Telengiectatic osteosarcoma
Question 5
Which of the following osteosarcomas have better prognosis?
a) osteoblastic osteosarcoma
b) periosteal osteosarcoma
c) parosteal osteosarcoma
d) telangiectatic osteosarcoma
e) small cell osteosarcoma
Question 6
Adamantinoma of long bones is associated with the following features EXEPT:
a) The most common histologic variants are basaloid, spindle, sqamoid and tubular
types
b) The epithelial component expresses cytokeratins 8 and 18
c) Younger age is associated with better prognosis
d) An osteofibrous dysplasia-like type is the least common histologic variant
e) Pain at presentation is associated with less favorable prognosis
Question 7
11 year girl with distal femoral lesion. What is your diagnosis?
a) Giant Cell Tumor (GCT)
b) Non-ossifying fibroma (NOF)
c) Aneurysmal Bone Cyst (ABC)
d) Chondroblastoma.
d) Giant Cell Rich Osteosarcoma.
Question 8
8 year old boy with distal femur epiphyseal lesion. What is your diagnosis?
a) Giant Cell Tumor (GCT)
b) Non-ossyfying fibroma (NOF)
c) Osteosarcoma
d) Chondroblastoma
e) Aneurysmal Bone Cyst (ABC)
Answer to question 1: C
Maffucci's syndrome is characterized by multiple enchondromas (Ollier's disease) and cavernous hemangiomas located in skin and soft tissue. This syndrome carries a greater risk of chondrosarcoma as well as nonskeletal malignancies, particulary of ovaries and brain.
Von Recklinghausen's disease of bone refers to severe bone disease of primary hyperparathyroidism characterized by increased osteoblastic activity, osteoclastic resorption, peritrabecular fibrosis, bone cyst and brown tumor (generalized osteitis fibrosa cystica).
Li-Fraumeni syndrome caused by inherited mutation of p53 is associated with greater risk of sarcomas (osteosarcoma) and other malignancies.
Gardner's syndrome is a combination of intestinal polyposis, multiple osteomas, fibromatosis and epidermal cysts.
Answer to question 2: A
Mutation of p53 (Li-Fraumeni syndrome) is associated with greater risk of sarcomas, particulary 500-fold greater risk of osteosarcoma.
Translocation (X;18) is characteristic of synovial sarcoma.
Transloction (12;16) is characteristic of myxoid liposarcoma.
1p rearrangements are characteristic for conventional chondrosarcoma.
Translocation (9;22) is characteristic for myxoid chondrosarcoma.
Answer to question 3: D
Lymphoma, neuroblastoma, mesenchymal chondrosarcoma and embryonal rhabdomyosarcoma show similar morphological features to Ewings sarcoma, characterized by small blue cell tumors. Leiomyosarcoma of bone is composed of bundles or fascicles of spindle cells.
Answer to question 4: B
Answer to question 5: C
Parosteal sarcoma is low grade (Grade 1) osteosarcoma. Periosteal osteosarcoma Grade 2, and osteoblastic osteosarcoma, telangiectatic osteosarcoma and small cell sarcoma are described as high grade sarcomas (Grade 3).
Answer to question 6: B
The epithelial component of adamantinoma expresses cytokeratins 14 and 19. CK8 and CK18 is expressed in epitheliod component of synovial sarcoma.
Answer to question 7: B
Answer to question 8: D
