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Question 281

Topic: Bone Tumors

Figure 15a shows the radiograph of a patient who has a chondrosarcoma of the acetabulum. Bone scans are shown in Figures 15b and 15c. Numerous soft subcutaneous masses are present. A clinical photograph of the hand is shown in Figure 15d. What is the most likely diagnosis?

. Multiple hereditary exostosis
. Ollier's disease
. Maffucci's syndrome
. McCune-Albright syndrome
. Neurofibromatosis

Correct Answer & Explanation

. Maffucci's syndrome


Explanation

Chondrosarcomas associated with diffuse bone lesions (enchondromas) are characteristic of Ollier's disease. When accompanied by subcutaneous masses (hemangiomas), the condition is called Maffucci's syndrome. Multiple hereditary exostosis is characterized by diffuse osteochondromas. McCune-Albright syndrome is characterized by polyostotic fibrous dysplasia with cafe-au-lait spots and precocious puberty. Neurofibromatosis can have associated bone lesions but is not associated with chondrosarcomas. Sun TC, Swee TC: Chondrosarcoma in Maffucci's syndrome. J Bone Joint Surg Am 1985;67:1214-1219. Schwartz HS, Zimmerman NB, Simon MA, et al: The malignant potential of enchondromatosis. J Bone Joint Surg Am 1987;69:269-274.

Question 282

Topic: Bone Tumors

A 40-year-old woman has a symptomatic mass on the anterior aspect of the ankle. She reports no constitutional symptoms. An MRI scan is shown in Figure 12. What is the most likely diagnosis?

Foot & Ankle 2000 Practice Questions: Set 1 (Solved) - Figure 28

. Ganglion
. Osteosarcoma
. Aneurysmal bone cyst
. Unicameral bone cyst
. Gouty tophi

Correct Answer & Explanation

. Ganglion


Explanation

The MRI scan reveals a lobular mass that is below the vitamin E tablet marker taped to the skin. This is juxtaposed to the tibialis anterior tendon. It is slightly more enhanced than the surrounding subcutaneous fat and is consistent with a ganglion. Osteosarcoma, aneurysmal bone cyst, or unicameral bone cyst all would demonstrate enhancement or pathology in the bone. This is clearly a well-defined soft-tissue mass. Gouty tophi show low to intermediate signal on T1- and T2-weighted images. Kransdorf MJ, Jelinek JS, Moser RP Jr, et al: Soft tissue masses: Diagnosis using MR imaging. Am J Roentgenol 1989;153:541-547. Wetzel LH, Levine E: Soft-tissue tumors of the foot: Value of MR imaging for specific diagnosis. Am J Roentgenol 1990;155:1025-1030.

Question 283

Topic: Bone Tumors

A 23-year-old man has had right posterolateral knee pain and occasional lateral calf dysesthesias for the past 8 months. A radiograph, CT scan, MRI scans, and a biopsy specimen are shown in Figures 62a through 62e. What is the most likely diagnosis?

. Osteoid osteoma
. Brodie's abscess
. Osteoblastoma
. Chondroblastoma
. Osteosarcoma

Correct Answer & Explanation

. Osteoid osteoma


Explanation

The radiograph shows an eccentric, cortically based lytic lesion in the proximal fibula. The CT and MRI scans confirm that it is well circumscribed and cortically based with significant surrounding edema. The radiographic differential diagnosis would be a Brodie's abscess or osteoid osteoma. An osteoblastoma would have to be greater than 2 cm in size. A chondroblastoma may also have significant edema around it, but it is an epiphyseal-based lesion, not cortically based. The well-circumscribed nature of the lesion is not consistent with osteosarcoma. The pathology shows a very cellular and vascular stroma with plump, but not atypical osteoblast cells making a matrix of immature woven bone. There are no abundant inflammatory cells or dead bone suggestive of osteomyelitis or a Brodie's abscess. Therefore, the clinical and histologic picture is most consistent with an osteoid osteoma. Percutaneous radiofrequency ablation, usually with CT guidance, has become the preferred method for treating most cases of osteoid osteoma. Rosenthal DI: Radiofrequency treatment. Orthop Clin North Am 2006;37:475-484.

Question 284

Topic: Bone Tumors

Figures 4a through 4c show the radiographs, CT scans, and T1-weighted MRI scan of a 19-year old man who has had increasing right hip pain and decreasing range of motion for the past several years. He also reports intermittent "locking" of the hip. What is the most likely diagnosis?

. Chondrosarcoma
. Stress fracture
. Osteochondroma
. Osteosarcoma
. Synovial osteochondromatosis

Correct Answer & Explanation

. Synovial osteochondromatosis


Explanation

The radiographs reveal small ossified masses around the femoral neck. The CT scans also show these masses and suggest that they are separate from the underlying cortex of the femoral neck, although they abut it. The MRI scan does not reveal significant marrow changes in the proximal femur apart from some mild reactive changes immediately adjacent to the nodules. These findings suggest a synovial or joint-based disorder as opposed to a primary bone tumor. The most likely diagnosis is synovial osteochondromatosis, which is consistent with the patient's mechanical symptoms. Crotty JM, Monu JU, Pope TL Jr: Synovial osteochondromatosis. Radiol Clin North Am 1996;34:327-342.

Question 285

Topic: Bone Tumors

A 9-year-old boy has a painless enlarged mass on the dorsum of his hand. Figures 14a through 14d show the clinical photograph, radiographs, and biopsy specimen. What is the most likely diagnosis?

. Fracture callus
. Enchondroma (Ollier's disease)
. Multiple hereditary exostosis
. Osteosarcoma
. Chondrosarcoma

Correct Answer & Explanation

. Multiple hereditary exostosis


Explanation

Multiple hereditary exostosis and enchondroma commonly present as multiple lesions in the hand. Multiple hereditary exostosis consists of cartilage capped bony exostoses arising from the metaphyseal end of rapidly growing bones. Osteosarcoma and chondrosarcoma rarely appear as multiple lesions. Fracture callus can exhibit enchondral ossification that is usually circumferential, but the radiographic findings are not consistent with fracture. Porter DE, Emerton ME, Villanueva-Lopez F, Simpson AH: Clinical and radiographic analysis of osteochondromas and growth disturbance in hereditary multiple exostoses. J Pediatr Orthop 2000;20:246-250.

Question 286

Topic: Bone Tumors

An otherwise healthy 33-year-old man who works in construction reports a 3-month history of knee pain. Radiographs are shown in Figures 9a and 9b. An axial T1-weighted MRI scan with contrast, an angiogram, and histologies are shown in Figures 9c through 9f. What is the most likely diagnosis?

. Conventional osteosarcoma
. Myositis ossificans
. Osteochondroma
. Parosteal osteosarcoma
. Dedifferentiated parosteal osteosarcoma

Correct Answer & Explanation

. Dedifferentiated parosteal osteosarcoma


Explanation

Dedifferentiated parosteal osteosarcoma designates high-grade transformation of conventional low-grade parosteal osteosarcoma. Unlike conventional parosteal osteosarcoma, where wide surgical excision alone is considered adequate treatment, patients with dedifferentiated osteosarcoma are treated with neoadjuvant chemotherapy and wide local resection. Recognition of dedifferentiated areas with angiography can localize the area that should be biopsied and thus render an accurate diagnosis. Percutaneous biopsy of hypervascular areas should prompt the administration of chemotherapy and wide local excision to optimize patient outcome. Sheth DS, Yasko AW, Raymond AK, et al: Conventional and dedifferentiated parosteal osteosarcoma: Diagnosis, treatment, and outcome. Cancer 1996;78:2136-2145.

Question 287

Topic: Bone Tumors

A 62-year-old woman reports diffuse aches and pains of the hip and pelvis. She denies any significant trauma but does have a history of chronic anemia. Figure 17a shows a radiograph of the pelvis, and Figures 17b and 17c show T2-weighted MRI scans. What is the most likely diagnosis?

. Chondrosarcoma
. Diffuse fibrous dysplasia
. Multiple myeloma
. Osteoporosis
. Bone infarcts

Correct Answer & Explanation

. Multiple myeloma


Explanation

The radiograph reveals diffuse osteopenia and areas in the proximal femora that are moth-eaten in appearance. The extent of the marrow-replacing process is evident on the MRI scans, which reveal signal abnormality throughout the entire pelvis and both proximal femora. This represents a marrow-packing process, of which multiple myeloma is the best choice. This diagnosis is also supported by the anemia noted on the patient's history. Metastatic carcinoma and lymphoma also may have a similar presentation.

Question 288

Topic: Bone Tumors

Figure 12 shows a lateral radiograph of the elbow. What is the most likely diagnosis?

Anatomy 2002 Practice Questions: Set 1 (Solved) - Figure 29

. Osteochondroma
. Old fracture fragment
. Heterotopic ossification
. Normal anatomic variant
. Osteosarcoma

Correct Answer & Explanation

. Normal anatomic variant


Explanation

The figure shows a supracondylar process, which is a normal anatomic variant. An osteochondroma tends to occur more toward the end of bones, and the medullary space of the underlying bone extends into the base of the osteochondroma. The presence of a supracondylar process is usually asymptomatic. However, the ligament of Struthers that always extends from the supracondylar process to the medial epicondyle can result in median nerve entrapment secondary to trauma. Last RJ: Anatomy: Regional and Applied, ed 6. London, England, Churchill Livingstone, 1978, pp 132-133.

Question 289

Topic: Bone Tumors

A previously asymptomatic 12-year-old girl sustained a direct blow to the right lateral knee from a baseball bat. Examination reveals an area of ecchymosis and tenderness over the lateral thigh. The patient can walk without pain, but range of motion of the knee causes discomfort. Plain radiographs of the knee are shown in Figures 11a and 11b. To address the bone lesion, management should consist of

. a three-phase bone scan.
. CT.
. MRI.
. a repeat examination in 6 weeks.
. a biopsy.

Correct Answer & Explanation

. a repeat examination in 6 weeks.


Explanation

The plain radiographs reveal a pedunculated osteochondroma with a fracture. There is a bony growth in the metaphysis of a long bone, on a stalk that is directed away from the nearby epiphysis. On the AP view, the host cortical and medullary bone are shown as "blending" with lesional bone. There is also a fracture through the lesion. Based on these radiographic findings, the diagnosis is an osteochondroma; therefore, initial management of an acute fracture of an osteochondroma is symptomatic treatment alone. Additional imaging studies are not indicated in this patient. At times it may be difficult to distinguish a sessile osteochondroma from a parosteal osteosarcoma. In the latter case, the host medullary bone and lesion bone are not confluent. A CT scan may be helpful to distinguish if the host medullary and cortical bone are confluent with the lesion.

Question 290

Topic: Bone Tumors

Figure 40 shows the radiograph of a 30-year-old woman who has a painful elbow. Examination reveals a deformed skull, multiple cafe-au-lait spots, and bone deformities. What is the most likely diagnosis?

Basic Science Board Review 2002: High-Yield MCQs (Set 4) - Figure 9

. McCune-Albright syndrome
. Neurofibromatosis, type I (NF-1)
. Neurofibromatosis, type II (NF-2)
. Paget's disease
. Ollier's disease

Correct Answer & Explanation

. McCune-Albright syndrome


Explanation

Findings in patients with McCune-Albright syndrome include polyostotic fibrous dysplasia, multiple cafe-au-lait spots, and precocious puberty. The bone changes in NF-1 resemble nonossifying fibromas, not fibrous dysplasia. NF-2 has little bony change with typical ocular abnormalities. Paget's disease occurs in older individuals and does not present with cafe-au-lait spots. Ollier's disease (multiple enchondromatosis) may show bone changes but not the other findings. Albright F, Butler AM, Hampton AO, et al: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females. N Engl J Med 1937;216:727-746. Danon M, Robboy SJ, Kim S, Scully R, Crawford JD: Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancy. J Pediatr 1975;87:917-921.

Question 291

Topic: Bone Tumors

A 10-year-old child has leg discomfort with activity. A radiograph, bone scan, and biopsy specimen are shown in Figures 1a through 1c. What is the most likely diagnosis?

. Parosteal osteosarcoma
. Unicameral bone cyst
. Aneurysmal bone cyst
. Eosinophilic granuloma
. Fibrous dysplasia

Correct Answer & Explanation

. Fibrous dysplasia


Explanation

The ground glass appearance on the radiograph, the hot bone scan, and histologic findings of bony spicules without osteoblastic rimming in a background of bland fibrous tissue all suggest fibrous dysplasia. Stress-related pain is common with activity because of the dysplastic bone. Parosteal osteosarcomas are surface lesions. Simple cysts, aneurysmal bone cysts, and eosinophilic granuloma are all possible radiographically; however, the histology is most consistent with fibrous dysplasia. Harris WH, Dudley HR Jr, Barry RS: The natural history of fibrous dysplasia: An orthopaedic, pathological and roentgenographic study. J Bone Joint Surg Am 1962;44:207.

Question 292

Topic: Bone Tumors
A 16-year-old girl has had anterior leg pain and a mass for the past 8 months. Figures 2a and 2b show a radiograph and an H&E histologic specimen. Which of the following disorders is believed to be a precursor of this lesion?
. Nonossifying fibroma
. Fibrous dysplasia
. Unicameral bone cyst
. Osteogenesis imperfecta
. Osteofibrous dysplasia

Correct Answer & Explanation

. Osteofibrous dysplasia


Explanation

The radiograph and pathology are consistent with adamantinoma. While the mechanism underlying adamantinoma has not been identified, it is believed to be closely related to osteofibrous dysplasia, which may represent a precursor. The other diagnoses are not known to give rise to adamantinoma.

Question 293

Topic: Bone Tumors

A 40-year-old man with an acetabular chondrosarcoma has a small soft-tissue mass. Treatment should consist of

Basic Science 2005 Practice Questions: Set 3 (Solved) - Figure 2

. chemotherapy with ifosfamide and doxorubicin hydrochloride.
. external beam radiation.
. wide resection.
. curettage and cementation of the pelvic lesion.
. cemented total hip arthroplasty with acetabular reconstruction.

Correct Answer & Explanation

. wide resection.


Explanation

The treatment of choice for pelvic chondrosarcoma is wide resection via an internal hemipelvectomy. Chondrosarcoma requires surgical resection for control and does not respond to traditional chemotherapy or external beam radiation. Hip arthroplasty with acetabular reconstruction and curettage and cementation of the lesion are intralesional procedures that result in a higher incidence of local recurrence of tumor. Pring M, Weber KL, Unni K, Sim FH: Chondrosarcoma of the pelvis: A review of sixty-four cases. J Bone Joint Surg Am 2001;83:1630-1642.

Question 294

Topic: Bone Tumors

A 23-year-old woman reports right knee pain and fullness. The pain is worse with activity but also present at rest. Radiographs are shown in Figures 20a and 20b. What is the most likely diagnosis?

. Osteosarcoma
. Chondroblastoma
. Stress fracture
. Posttraumatic changes
. Chondrosarcoma

Correct Answer & Explanation

. Osteosarcoma


Explanation

The radiographs reveal a predominantly lytic, destructive lesion of the distal femur, although there is a hint of some blastic change as well. The lesion has violated the cortex, and there is mineralization outside the cortex laterally. The lateral radiograph suggests a soft-tissue density. These aggressive changes on radiographs in this age group are strongly suggestive of osteosarcoma. Sanders TG, Parsons TW: Radiographic imaging of musculoskeletal neoplasia. Cancer Control 2001;8:221-231.

Question 295

Topic: Bone Tumors

Mutations of what gene are associated with the subsequent development of osteosarcoma?

. RB
. EWS-FLI1
. EGF-R
. IGF-R
. VEGF

Correct Answer & Explanation

. RB


Explanation

Patients with a mutation of the retinoblastoma gene (RB) have an increased likelihood for the development of osteosarcoma. The EWS-FLI1 gene is the fusion product of a chromosomal translocation of 11:22. EGF-R and IGF-R are growth factor receptors, but mutations in these genes have not been correlated with osteosarcoma. VEGF is a proangiogenic molecule that is involved in tumor formation in multiple sites but not the development of osteosarcoma. Scholz RB, Kabisch H, Delling G, et al: Homozygous deletion within the retinoblastoma gene in a native osteosarcoma specimen of a patient cured of a retinoblastoma of both eyes. Pediatr Hematol Oncol 1990;7:265-273.

Question 296

Topic: Bone Tumors

A 31-year-old woman has a history of a painful ankle that has failed to respond to conservative management. She has associated night pain that is relieved with nonsteroidal anti-inflammatory drugs. MRI and technetium Tc 99m scans are consistent with an osteoid osteoma. Management should now consist of

. curettage or en bloc resection.
. allograft limb salvage.
. injection with 5% phenol.
. radiation therapy.
. hormonal therapy.

Correct Answer & Explanation

. curettage or en bloc resection.


Explanation

Surgical curettage or en bloc resection is the treatment of choice for osteoid osteoma. Night pain and relief of symptoms with nonsteroidal anti-inflammatory drugs are classic findings for osteoid osteoma. Donley BG, Philbin T, Rosenberg GA, Schils JP, Recht M: Percutaneous CT guided resection of osteoid osteoma of the tibial plafond. Foot Ankle Int 2000;21:596-598. Kenzora JE, Abrams RC: Problems encountered in the diagnosis and treatment of osteoid osteoma of the talus. Foot Ankle 1981;2:172-178.

Question 297

Topic: Bone Tumors

An otherwise healthy 75-year-old man has a painful mass in the popliteal fossa of his right knee. A lateral radiograph of the knee, a CT scan of the distal femur, and a histopathologic specimen are shown in Figures 13a through 13c. Management should consist of

. observation.
. radiation therapy.
. radiation therapy and chemotherapy.
. surgical resection.
. surgical resection and chemotherapy.

Correct Answer & Explanation

. surgical resection.


Explanation

The patient has a parosteal osteosarcoma of the distal femur. The findings of mild knee pain, radiographic evidence of a radiodense mass involving the parosseous space or surface of the distal femur, and histologic findings of a spindle cell lesion forming immature osteoid with little to no necrosis most likely suggest a parosteal osteosarcoma. The treatment of choice is surgical resection. Okada K, Frassica FJ, Sim FH, Beabout JW, Bond JR, Unni KK: Parosteal osteosarcoma: A clinicopathological study. J Bone Joint Surg Am 1994;76:366-378.

Question 298

Topic: Bone Tumors

A 19-year-old woman reports persistent neck pain for 2 years. Pain is relieved with aspirin. A bone scan shows intense uptake in the superior, posterior portion of the C3 vertebral body. A sagittal CT reconstruction is shown in Figure 5. Treatment should consist of

Spine Surgery 2009 Practice Questions: Set 1 (Solved) - Figure 18

. radiation therapy.
. en bloc excision.
. posterior fusion at C2-C3 with instrumentation.
. CT-guided aspiration followed by IV antibiotics.
. radiofrequency ablation.

Correct Answer & Explanation

. en bloc excision.


Explanation

The CT scan shows an osteoblastic nidus pathognomic for an osteoid osteoma. Surgical treatment should include an en bloc excision of the lesion. Surgical treatment is not mandatory because the lesion often becomes asymptomatic over time. This lesion is not amenable to radiofrequency ablation due to its proximity to the spinal cord. A complete corpectomy is not necessary to adequately resect the lesion, as only the nidus needs to be removed. Radiation therapy and antibiotics are not appropriate treatments for an osteoid osteoma. Posterior C2-C3 fusion will not address the pathology. Spivak JM, Connolly PJ (eds): Orthopaedic Knowledge Update: Spine 3. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2006, pp 351-366.

Question 299

Topic: Bone Tumors

What additional percentage of energy expenditure above baseline is required for ambulation after an above-the-knee amputation?

. 0%
. 5%
. 20%
. 65%
. 90%

Correct Answer & Explanation

. 65%


Explanation

Patients with an above-the-knee amputation have a 65% increase in energy expenditure. A patient with a transtibial amputation requires 25% more energy above baseline values; however, bilateral transtibial amputations are associated with a 40% increase in energy expenditure. Otis JC, Lane JM, Kroll MA: Energy cost during gait in osteosarcoma patients after resection and knee replacement and after above-the-knee amputation. J Bone Joint Surg Am 1985;67:606-611.

Question 300

Topic: Bone Tumors

A 15-year-old boy complains of intense, unrelenting right thigh pain that is noticeably worse at night and dramatically relieved within 30 minutes of taking ibuprofen. A CT scan reveals a 7mm radiolucent nidus surrounded by dense, reactive cortical sclerosis in the proximal femur. Which of the following factors produced within the nidus is most directly responsible for this characteristic pain pattern?

. High concentration of interleukins
. Extensive infiltration of eosinophils
. Elevated levels of prostaglandins
. Accumulation of lactic acid
. Overexpression of RANK ligand

Correct Answer & Explanation

. Elevated levels of prostaglandins


Explanation

The nidus of an osteoid osteoma produces extraordinarily high levels of prostaglandins (particularly Prostaglandin E2, or PGE2) due to high expression of cyclooxygenase (COX) enzymes. This localized prostaglandin production causes profound vasodilation and is the direct cause of the intense, nocturnal pain that is classically and rapidly relieved by NSAIDs or salicylates.