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
  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)

Question 9
What is the most common malignant solid tumor of children less than 1 year old ?
   a) Alveolar rhabdomyosarcoma.
   b) Embryonal rhabdomyosarcoma.
   c) Neuroblastoma.
   d) Juvenile fibrosarcoma.
   e) Ewing's sarcoma/PNET

Question 10
What is the juvenile form of dermatofibrosarcoma protuberans?
   a) Atypical fibroxanthoma.
   b) Giant cell fibroblastoma
   c) Myofibroma
   d) Hemangiopricytoma
   e) Dermatofibroma
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
Aneurysmal bone cyst (ABC)

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
Non-ossifying fibroma.

Answer to question 8: D

Answer to question 9: C
Neuroblastoma is the most common malignant solid tumors in children less than 1 y/o with the majority of cases (90%) diagnosed in the first 5 years of life. Neuroblastoma belongs to the group of embryonal tumors that originate from the sympathetic nervous system.
Infantile fibrosarcoam  (IFS) accounts for 36%-80% of congenital cases and 36%-100% occurs in first year of life, although is not that common comparing to neuroblatoma
Embryonal rhabdomyosarcoma is most common subtype of rhabdomyosarcoma, occuring in 3.0/milion U.S. children <15 years old. The greatest proportion (46%) of embryonal rhabdomyosarcomas occurs in children less than five year old but is not common in first year of life. Alveolar rhabdomyosarcoma occurs at all ages, more often in adolescent and young adults. They occur less frequently than embryonal rhabdomyosarcoma.
Ewing sarcoma mainly affects adolescents and young adults with nearly 80% younger than 20 y/o, and the peak incidence is during the second decade of life.

Answer to question 10: B
Giant cell fibroblastoma (GCFB) occurs most exclusively in children and represent adult form of dermatofibrosarcoma protuberans (DFSP). The lesion is positive for CD34 like DFSP. The most common location for the lesion is thigh, inguinal region and back of infants and young children, usually less than 5 y/o. It arises as painless nodule or mass in the dermis or subcutaneous fat.
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