What is your diagnosis?
a) Metaphyseal dysplasia.
b) Osteogenesis Imperfecta Type II
c) Achondrogenesis Type I
d) Thanatophoric dwarfism
e) Osteogenesis Imperfecta Type I
Answer:
b. Osteogenesis Imperfecta Type II
Sunday, March 21, 2010
X-ray of the day
What is your diagnosis?
a) Metaphyseal dysplasia.
b) Osteogenesis Imperfecta Type II
c) Achondrogenesis Type I
d) Thanatophoric dwarfism
e) Osteogenesis Imperfecta Type I
Answer:
b. Osteogenesis Imperfecta Type II
MCQs
1. Regarding achondroplasia:
a) Achondroplasia is the most common type of short limb skeletal dysplasia which shortens stature
b) Achondroplasia is an autosomal recessive defect.
c) The short pedicles of achondroplasia lead to spinal stenosis and paraplegia more than 15% of patients
d) Hydrocephalus is an uncommon complication in patients with achondroplasia
e) About 90% of the cases of achondroplasia occur from a spontaneous mutation in patients with normal parents
.
2. Which is the most severe form of short-limbed dwarfism? Please pick the BEST answer.
a) Hypochondroplasia
b) Achondroplasia
c) Thanatophoric dwarfism
d) Homozygous achondroplasia
Answers:
1. a, c, e
Achondroplasia is the most common type of short limb skeletal dysplasia which shortens stature. It is an autosomal dominant defect in endochondral bone formation (osteochondrodysplasia) which causes skeletal growth derived from cartilage to be shortened. The most significant shortening occurs in long tubular bones, cranial base, and spinal pedicles. The short pedicles of achondroplasia lead to spinal stenosis and paraplegia more than 15% of patients. Hydrocephalus is a concern in 60% of patients with achondroplasia. About 90% of the cases of achondroplasia occur from a spontaneous mutation in patients with normal parents. However, it's mode of inheritance is autosomal dominant.
2. c
Thanatophoric dwarfism is the most common cause of fatal neonatal dwarfism. Features include cloverleaf skull, short ribs with small thoracic volume, small pedicles, and square iliac bones
a) Achondroplasia is the most common type of short limb skeletal dysplasia which shortens stature
b) Achondroplasia is an autosomal recessive defect.
c) The short pedicles of achondroplasia lead to spinal stenosis and paraplegia more than 15% of patients
d) Hydrocephalus is an uncommon complication in patients with achondroplasia
e) About 90% of the cases of achondroplasia occur from a spontaneous mutation in patients with normal parents
.
2. Which is the most severe form of short-limbed dwarfism? Please pick the BEST answer.
a) Hypochondroplasia
b) Achondroplasia
c) Thanatophoric dwarfism
d) Homozygous achondroplasia
Answers:
1. a, c, e
Achondroplasia is the most common type of short limb skeletal dysplasia which shortens stature. It is an autosomal dominant defect in endochondral bone formation (osteochondrodysplasia) which causes skeletal growth derived from cartilage to be shortened. The most significant shortening occurs in long tubular bones, cranial base, and spinal pedicles. The short pedicles of achondroplasia lead to spinal stenosis and paraplegia more than 15% of patients. Hydrocephalus is a concern in 60% of patients with achondroplasia. About 90% of the cases of achondroplasia occur from a spontaneous mutation in patients with normal parents. However, it's mode of inheritance is autosomal dominant.
2. c
Thanatophoric dwarfism is the most common cause of fatal neonatal dwarfism. Features include cloverleaf skull, short ribs with small thoracic volume, small pedicles, and square iliac bones
MCQs: Ortopaedic Oncology
1. Regarding desmoplastic fibroma (Choose a single answer):
a) Desmoplastic fibroma is a malignant neoplasm.
b) The most common site of occurence is the ilium.
c) Curretage is the treatment of choice.
d) "Pseudo-trabeculae" are a classic feature of desmoplastic fibroma
2. Regarding Ewings sarcoma (Choose a single answer):
a) Ewings sarcoma is typically a tumor of middle-age and beyond.
b) Tubular bones are most commonly affected in Ewings sarcoma in patients under 20 years old, with flat bones most commonly affected in those older than 20.
c) In Ewings sarcoma, bone metastases are uncommon and unlike the primary tumor, are usually blastic when they do occur.
d) The vast majority of occurrences of Ewings sarcoma in the long bones are of the central diaphyseal type.
3. Regarding osteochondroma
a) An osteochondroma is a bony projection with a cartilaginous cap arising from a bone preformed in cartilage
b) Malignant degeneration of osteochondroma to chondrosarcoma occurs in up to 20% of patients with hereditary multiple exostoses.
c) The cortex and medullary cavity of the host bone are not continuous with the osteochondroma.
d) The most common sites for osteochondroma are the hands and feet.
e) Almost all pseudoanerysms complicating osteochondromas arise from the popliteal artery
f) Benign osteochondromas generally do not demonstrate continued growth after skeletal maturity
Answers:
1. d
Desmoplastic fibroma is a locally aggressive, benign neoplasm of bone. It can be considered the intraosseous counterpart of the soft tissue desmoid tumor. The most common site of occurence is the mandible. Broad local resection is the treatment of choice. The lesion is prone to recur with curretage. "Pseudo-trabeculae" are a classic feature of desmoplastic fibroma.
2. b
Ewings sarcoma is a tumor of young people, with the peak age of incidence being 15 years old. Tubular bones are most commonly affected in Ewings sarcoma in patients under 20 years old, with flat bones most commonly affected in those older than 20. This is thought to reflect the red marrow distribution of the two age groups. In Ewings sarcoma, bone metastases are common, and usually ARE a lytic destructive process similar in appearance to the primary tumor. The vast majority of occurrences of Ewings sarcoma in the long bones are of the central diaphyseal type.
3. a, b, e, f
An osteochondroma is a bony projection with a cartilaginous cap arising from a bone preformed in cartilage. Malignant degeneration of osteochondroma to chondrosarcoma occurs in up to 20% of patients with hereditary multiple exostoses. The cortex and medullary cavity of the host bone ARE continuous with the osteochondroma. The most common sites of involvement include the distal femur, the proximal tibia, and the humerus. Almost all pseudoanerysms complicating osteochondromas arise from the popliteal artery. Benign osteochondromas generally do not demonstrate continued growth after skeletal maturity. In the presence of a large soft tissue mass or enlargement of a previously stable lesion chondrosarcomatous transformation must be considered.
a) Desmoplastic fibroma is a malignant neoplasm.
b) The most common site of occurence is the ilium.
c) Curretage is the treatment of choice.
d) "Pseudo-trabeculae" are a classic feature of desmoplastic fibroma
2. Regarding Ewings sarcoma (Choose a single answer):
a) Ewings sarcoma is typically a tumor of middle-age and beyond.
b) Tubular bones are most commonly affected in Ewings sarcoma in patients under 20 years old, with flat bones most commonly affected in those older than 20.
c) In Ewings sarcoma, bone metastases are uncommon and unlike the primary tumor, are usually blastic when they do occur.
d) The vast majority of occurrences of Ewings sarcoma in the long bones are of the central diaphyseal type.
3. Regarding osteochondroma
a) An osteochondroma is a bony projection with a cartilaginous cap arising from a bone preformed in cartilage
b) Malignant degeneration of osteochondroma to chondrosarcoma occurs in up to 20% of patients with hereditary multiple exostoses.
c) The cortex and medullary cavity of the host bone are not continuous with the osteochondroma.
d) The most common sites for osteochondroma are the hands and feet.
e) Almost all pseudoanerysms complicating osteochondromas arise from the popliteal artery
f) Benign osteochondromas generally do not demonstrate continued growth after skeletal maturity
Answers:
1. d
Desmoplastic fibroma is a locally aggressive, benign neoplasm of bone. It can be considered the intraosseous counterpart of the soft tissue desmoid tumor. The most common site of occurence is the mandible. Broad local resection is the treatment of choice. The lesion is prone to recur with curretage. "Pseudo-trabeculae" are a classic feature of desmoplastic fibroma.
2. b
Ewings sarcoma is a tumor of young people, with the peak age of incidence being 15 years old. Tubular bones are most commonly affected in Ewings sarcoma in patients under 20 years old, with flat bones most commonly affected in those older than 20. This is thought to reflect the red marrow distribution of the two age groups. In Ewings sarcoma, bone metastases are common, and usually ARE a lytic destructive process similar in appearance to the primary tumor. The vast majority of occurrences of Ewings sarcoma in the long bones are of the central diaphyseal type.
3. a, b, e, f
An osteochondroma is a bony projection with a cartilaginous cap arising from a bone preformed in cartilage. Malignant degeneration of osteochondroma to chondrosarcoma occurs in up to 20% of patients with hereditary multiple exostoses. The cortex and medullary cavity of the host bone ARE continuous with the osteochondroma. The most common sites of involvement include the distal femur, the proximal tibia, and the humerus. Almost all pseudoanerysms complicating osteochondromas arise from the popliteal artery. Benign osteochondromas generally do not demonstrate continued growth after skeletal maturity. In the presence of a large soft tissue mass or enlargement of a previously stable lesion chondrosarcomatous transformation must be considered.
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