AAOS & ABOS Basic Science MCQs (Set 3): Orthopedic Biomechanics & Bone Physiology | OITE Review

Paget's disease of bone is a metabolic disorder of bone remodeling. The normally coupled process of bone resorption and deposition is lost, resulting in excessive localized bone resorption and compensatory increased bone formation. Pagetic bone tends to be more brittle; therefore, it is susceptible to pathologic fractures and subsequent deformities. Lander PH, Hadjipavlou AG: A dynamic classification of Paget's disease. J Bone Joint Surg Br 1986;68:431-438.

CT of the abdomen and pelvis is not part of the staging of osteosarcoma. Staging studies should consist of CT of the chest, radiographs of the chest and primary tumor, MRI of the primary tumor, and a bone scan. The MRI should be obtained prior to the biopsy.

The factors that are independently prognostic of patient survival are tumor size, tumor grade, and tumor depth (ie, subfascial versus superficial). These factors are the basis for the American Joint Committee on Cancer staging criteria. Patient age and neurovascular invasion are not prognostic. Surgical margin is prognostic for local recurrence but not conclusively for patient survival or metastasis. Metastatic disease is also predictive of survival. Cheng EY, Thompson RC Jr: New developments in the staging and imaging of soft-tissue sarcomas. Instr Course Lect 2000;49:443-451.

The osseous sites most frequently involved by giant cell tumor of bone are the distal femur, proximal tibia, and distal radius with approximately 10% of giant cell tumors involving the distal radius. The goals of treatment are to remove the tumor completely and to preserve maximum function of the extremity.

Osteosarcoma is the most common primary malignant tumor of bone in childhood, followed by Ewing's sarcoma. Rhabdomyosarcoma is a soft-tissue sarcoma of childhood. Chondrosarcoma rarely occurs in childhood. Osteochondroma is a benign tumor of bone. Simon MA, Springfield DS, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 266.

Rheumatoid arthritis is an inflammatory arthritis that usually involves multiple joints. Radiologic findings of periarticular erosion, osteopenia, and minimal osteophyte formation favor rheumatoid arthritis over osteoarthritis. Pigmented villonodular synovitis and Charcot arthropathy are more often considered monoarticular diseases. There are no radiographic findings of Paget's disease.

While guidelines for predicting fracture risk are at best imprecise, the scoring system by Mirels (pain, anatomic location, and pattern of bony destruction) has been shown to be most predictive of fracture risk. Functional pain, peritrochanteric location, and lytic bone destruction are the greatest risk factors for pathologic fracture. The factors of patient weight, age, soft-tissue mass, and location within bone are all of lesser importance. Frassica FJ, Frassica DA, McCarthy EF, Riley LH III: Metastatic bone disease: Evaluation, clinicopathologic features, biopsy, fracture risk, nonsurgical treatment, and supportive management. Instr Course Lect 2000;49:453-459.

The median survival of patients after discovery of bone metastasis from primary lung carcinoma is shorter compared with other primary sites.

Although many hematologic and electrolyte abnormalities may be present in a patient with advanced metastatic cancer, an elevated serum calcium level is most commonly associated with confusion. Treatment with hydration, diuretics, and bisphosphonates is recommended. Clohishy D: Management of skeletal metastasis in clinical orthopaedics, in Craig E (ed): Operative Orthopaedics. Philadelphia, PA, Lippincott Williams & Wilkins, 1999, pp 994-997.

Because the MRI scans show marked dilation and proliferation of lymphatic channels that completely involve all the leg muscles and the clinical photograph shows the severe swelling associated with this disease, the most likely diagnosis is lymphangiomatosis. Poliomyelitis affects the anterior horn cells and manifests as muscle atrophy. Neurofibromatosis can have a similar clinical appearance but usually is associated with other systemic and cutaneous findings. Congenital band syndrome results in amputated or shortened extremities. Chronic venous stasis disease usually is not associated with joint contractures, and typically it affects older individuals. Surgical excision is the only known treatment; this patient underwent an above-knee amputation. Berquist TH (ed): MRI of the Musculoskeletal System, ed 3. Philadelphia, PA, Lippincott Raven, 1997, p 771.

According to the MSTS staging system, soft-tissue sarcomas are staged according to the following factors: grade, site, and metastasis. Roman numerals are used to designate malignant tumors, and Arabic numerals are used for benign tumors. Low-grade malignant tumors are staged as I while intermediate- and high-grade tumors are staged as II. Site is defined as intracompartmental (A) or extracompartmental (B). Any metastasis is staged as III. This patient has a high-grade tumor (II), and the MRI scans show that it is confined to a single compartment; therefore, it is staged as IIA. There is no evidence of metastatic disease. Enneking WF, Spanier SS, Goodman MA: A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop 1980;153:106-120. Peabody TD, Monson D, Montag A, Schell MJ, Finn H, Simon MA: A comparison of the prognoses for deep and subcutaneous sarcomas of the extremities. J Bone Joint Surg Am 1994;76:1167-1173. Pisters PW, Leung DH, Woodruff J, Shi W, Brennan MF: Analysis of prognostic factors in 1,041 patients with localized soft tissue sarcomas of the extremities. J Clin Oncol 1996;14:1679-1689.

Resection of a previously excised soft-tissue sarcoma is recommended. Reoperation is recommended after removal of malignant tumors previously believed to be benign, as approximately one half of the patients will have residual tumor in the re-excised specimen. Observation is not indicated in most patients because local recurrence is likely. Radiation therapy alone may result in long-term local control but is felt to be less effective than reoperation. Chemotherapy and radiation therapy alone are not recommended. Wide excision is the most important factor for local disease control. Radiation therapy after surgical re-excision may also decrease the risk of local recurrence. Peabody TD, Monson D, Montag A, Schell MJ, Finn H, Simon MA: A comparison of the prognoses for deep and subcutaneous sarcomas of the extremities. J Bone Joint Surg Am 1994;76:1167-1173. Giuliano AE, Eilber FR: The rationale for planned reoperation after unplanned total excision of soft-tissue sarcomas. J Clin Oncol 1985;3:1344-1348. Lewis JJ, Leung D, Espat J, Woodruff JM, Brennan MF: Effect of resection in extremity soft tissue sarcoma. Ann Surg 2000;231:655-663.

The patient has osteosarcoma. The radiograph suggests an aggressive primary tumor of bone, and the histology shows malignant cells surrounded by osteoid, classic for osteosarcoma. Ewing's sarcoma histologically consists of small round blue cells. Osteochondroma and periosteal chondroma occur in the shoulder but have a different histologic pattern and a less aggressive radiographic appearance. Chondrosarcomas rarely occur in children. Simon MA, Springfield DS, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 266.

Both myeloma and lymphoma are more responsive to radiation therapy. The other types of tumors are relatively more resistant to radiation. Doses of 25 to 50 Gy (2,500 to 5,000 cGy) are usually sufficient for myeloma and lymphoma, while carcinomas frequently require a higher dosage. Large cell lymphoma of bone is usually of B-cell origin and is treated with chemotherapy and radiation therapy. Simon MA, Springfield DS, et al: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 683.

The plain radiographs show an eccentric metaphyseal lesion involving a long bone in a skeletally immature patient. The lesion is longer than it is wide, with distinctly lobular outer edges that are sclerotic. These findings are characteristic of a nonossifying fibroma. Small asymptomatic lesions may be followed clinically. Larger lesions that occupy greater than two thirds of the width of the shaft and are located in areas of high mechanical stress such as the femur are more prone to fracture than smaller lesions. Pain is often a sign of impending fracture or the presence of a small fracture that may not be apparent on radiographs. The natural history of the lesion is to resolve over a period of years. The procedure that would allow the patient to return to contact sports is curettage and bone grafting. Intralesional steroid injection has been advocated in the treatment of unicameral bone cysts and eosinophilic granuloma but not nonossifying fibromas. En block resection is not indicated for a benign lesion. Low-dose radiation therapy has been used for eosinophilic granuloma but not for nonossifying fibromas. Walker RN, Green NE, Spindler KP: Stress fractures in skeletally immature patients. J Pediatr Orthop 1996;16:578-584.

In 1940, Batson described a valveless plexus of veins that extend from the dural venous sinuses of the skull to the sacrum. This system permits retrograde blood flow and enables tumor cells to enter vertebral bodies at multiple levels. Increased intra-abdominal pressure will enhance this retrograde blood flow.

The diagnosis is eosinophilic granuloma. The plain radiograph and MRI scan show a lesion in the midshaft of the femur. There is no soft-tissue mass. There is reactive bone about the lesion that suggests a less aggressive tumor. The histology reveals eosinophils in an otherwise bland cellular background with no evidence of mitotic figures or malignant cells to suggest sarcoma. The diagnostic elements are the amphophilic (ie, pale purple) histiocytes with cigar-shaped nuclei, some of which have linear longitudinal grooves. There is no histologic evidence of infection. Lymphoma of bone would be an unusual occurrence in this age group, and the histology is not consistent with that diagnosis.

Alcohol abuse, steroids, Caisson disease, sickle cell disease, and radiation therapy are the leading causes of nontraumatic osteonecrosis of the femoral head. Arlet J: Nontraumatic avascular necrosis of the femoral head: Past, present, and future. Clin Orthop 1992;277:12-21.

Synovial sarcoma should always be considered in the differential diagnosis of a foot mass; however, the histopathology shows a typical example of PVNS, with hemosiderin, giant cells, and synovium. Synovial chondromatosis would have metaplastic cartilage in the synovium. The radiograph shows subtle erosion of the lateral cortex of the cuboid, and the MRI scan shows a soft-tissue mass. Ghert MA, Scully SP, Harrelson JM: Pigmented villonodular synovitis of the foot and ankle: A review of six cases. Foot Ankle Int 1999;20:326-330. Jones BC, Sundaram M, Kransdorf MJ: Synovial sarcoma: MR imaging findings in 34 patients. Am J Roentgenol 1993;161:827-830. Sartoris DJ, Resnick D: Magnetic resonance imaging of pediatric foot and ankle disorders. J Foot Surg 1990;29:489-494.

The most common destructive lesions in a patient older than age 40 years are, in decreasing incidence, metastatic carcinoma, multiple myeloma, lymphoma, and chondrosarcoma. Osteosarcoma is found primarily in younger patients.

The patient has lung cancer metastatic to the left humerus that is mildly to moderately symptomatic. Radiographically, the lesion is small and is not associated with significant cortical destruction. Metastatic lesions in the upper extremity have a lower incidence of pathologic fracture than lesions in the lower extremity.

Although considered benign bone lesions, lung metastases can develop in giant cell tumors and chondroblastomas. These often can be treated with multiple thoracotomies, resulting in long-term survival. Roberts PF, Taylor JG: Multifocal benign chondroblastomas: Report of a case. Hum Pathol 1980;11:296-298.

For the fibula (or any bone) to bow, a long-standing process needs to be present. Pressure from a rapid process would cause erosion, not allowing the bone to remodel. The other features are helpful confirmatory findings but also may be associated with aggressive processes. In this patient, the fibular deformity is caused by a sessile osteochondroma of the tibia. Lodwick GS, Wilson AJ, Farrell C, Virtama P, Dittrich F: Determining growth rates of focal lesions of bone from radiographs. Radiology 1980;134:577-583.

Local control of malignant fibrous histiocytoma of bone typically consists of wide excision. Curettage and bone grafting is a procedure with an intralesional surgical margin, with an unacceptable rate of local recurrence. Prophylactic fixation is considered for patients with metastatic disease. Radiation therapy alone is not adequate for local control of this tumor. Neoadjuvant chemotherapy is often used primarily for systemic tumor control.

Surgical margins are classified according to the plane of dissection in relation to the tumor. An intralesional procedure is where the plane of dissection passes through the tumor. A marginal resection involves a resection where the plane of dissection is through the reactive zone of the tumor. A wide resection is through completely normal tissue outside of the reactive zone. A radical resection removes all of the muscles and/or bone of the compartment that are involved by the tumor.