AAOS Basic Science MCQs (Set 2): Bone Physiology & Biomechanics | 2002 Board Review

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.

Lung carcinoma most often has a lytic radiographic appearance. Bladder and prostate carcinoma are usually blastic. Breast carcinoma can be both blastic and lytic. Thyroid carcinoma may be difficult to visualize radiographically and may be seen only on MRI scans. Metastatic osteosarcomas typically produce bone. 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.

From an imaging point of view, all of the diagnoses are possible. Biopsy results and cultures are necessary to make the diagnosis. The biopsy specimen shows inflammatory cells and necrotic bone, consistent with osteomyelitis. Fletcher BD, Hanna SL: Pediatric musculoskeletal lesions simulating neoplasms. Magn Reson Imaging Clin N Am 1996;4:721-747.

Fibrous dysplasia frequently occurs in the proximal femur. Microfractures and remodeling can lead to the classic "Shepherd's crook deformity." The lesion has a ground-glass appearance on plain radiographs. The histologic appearance shows proliferating fibroblasts in a loose spindle cell background. Dysplastic metaplastic trabeculae are arranged in an irregular or "Chinese letter" appearance. Enneking WF, Gearen PF: Fibrous dysplasia of the femoral neck: Treatment by cortical bone grafting. J Bone Joint Surg Am 1986;68:1415-1422.

Aneurysmal bone cyst may be either a "pattern" or a "diagnosis." Therefore, aneurysmal bone cyst should be viewed as a diagnosis of exclusion. Hemorrhage into a variety of primary bone lesions (eg, giant cell tumor of bone, chondroblastoma, osteoblastoma, fibrous dysplasia, osteosarcoma, or vascular neoplasms) may result in intralesional, membrane-bone blood-filled cysts. Such secondary changes may be confused with aneurysmal bone cyst, resulting in inappropriate therapy because assessment should be focused on identifying the underlying primary process. The entire specimen should be examined histologically in an effort to locate an underlying primary bone tumor. Bonakdarpour A, Levy WM, Aegerter E: Primary and secondary aneurysmal bone cyst: A radiological study of 75 cases. Radiology 1978;126:75-83. Levy WM, Miller AS, Bonakdarpour A, Aegerter E: Aneurysmal bone cyst secondary to other osseous lesions: Report of 57 cases. Am J Clin Pathol 1975;63:1-8.

The patient has a unicameral bone cyst. Because the subtrochanteric part of the femur is a high-stress region, the treatment of choice is bone curettage and grafting. Azouz EM, Karamitsos C, Reed MH, Baker L, Kozlowski K, Hoeffel JC: Types and complications of femoral neck fractures in children. Pediatr Radiol 1993;23:415-420.

A ganglion cyst arising from the posterior labrum of the shoulder is a known cause of suprascapular nerve impingement. The MRI characteristics of low-signal intensity on T1 and high-signal intensity on T2 are seen in lesions with a high fluid content. The histology shows a cavity with a thin fibrous lining. Ferrick MR, Marzo JM: Suprascapular entrapment neuropathy and ganglion cysts about the shoulder. Orthopedics 1999;22:430-434.

Histologic grade, the presence or absence of metastatic disease, and tumor size are important prognostic factors. Of the available choices, however, the size of the sarcoma is the most important prognostic indicator. A tumor size of greater than 5 cm is a more important prognostic factor than tumor location. Patients with sarcomas that measure 5 cm or less have nearly identical 3-year survival rates regardless of whether the tumor is subcutaneous or deep. Histologic grade (high versus low) is an important factor. However, histologic subtype frequently is not as important a factor as tumor size.

Based on the location and extent of the tumor, most patients are candidates for limb salvage surgery. The patient has an osteosarcoma; therefore, the treatment of choice is preoperative chemotherapy, wide resection, and reconstruction. There is no difference in survival rates between patients who undergo limb salvage surgery and those who undergo amputation. Curettage and placement of methylmethacrylate is used to treat benign lesions of bone and would not be appropriate in this patient.

Plate and screw fixation of allograft is associated with the highest incidence of union but also the highest prevalence of allograft fracture. Conversely, intramedullary fixation is associated with a higher incidence of nonunion but fewer fractures. Step-cut osteotomies are not associated with a higher incidence of union.

In a patient with known metastatic disease, the surgeon must rule out additional lesions throughout the femur prior to surgical management. Lesions located in the diaphysis or in the peritrochanteric region may influence the surgical procedure. Frassica FJ, Gitelis S, Sim FH: Metastatic bone disease: General principles, pathophysiology, evaluation, and biopsy. Instr Course Lect 1992;41:293-300.

It is generally accepted that reconstructions of the proximal tibia are associated with the highest incidence of failure, probably because of poor soft-tissue coverage, the need for extensor mechanism reconstruction, and other anatomic issues. It also may be related to the fact that patients with tumors of the proximal tibia, in general, have a better prognosis and better survival rates than patients with tumors located elsewhere in the body. Reconstructions of the proximal humerus may be more durable because they are not involved in weight-bearing activities.

The presence of metastatic disease at diagnosis is the most significant prognostic factor in a patient with osteosarcoma. Limb-sparing surgery, when compared with amputation, is not noted to be associated with any difference in the rate of survival. The presence of a mutated p53 gene has been noted in a minority of patients with osteosarcoma; however, the prognostic importance on an individual basis is unclear. Tumor location and a relative poor response to chemotherapy would be secondary to the presence of metastatic disease in estimating a patient's prognosis. Weis L: Common malignant bone tumors: Osteosarcoma, in Simon MA, Springfield DS (eds): Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott-Raven, 1998, pp 265-274.

The subtrochanteric femur has been identified as an anatomic site that is particularly prone to pathologic fracture. An avulsion fracture of the lesser trochanter is a sign of impending femoral fracture. While the other anatomic locations are also frequently involved in metastatic bone disease, pathologic fractures occur less commonly. Simon MA, Springfield DS, et al: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 683.

The presence of synchronous bone disease in young patients carries a dismal prognosis, one that is even worse than the presence of resectable pulmonary metastasis. Many osteosarcomas cross the physis; therefore, this has not been shown to be of prognostic importance. Similarly, the presence of the soft-tissue mass has less prognostic significance.

Although the degeneration of an isolated benign cartilaginous lesion into a chondrosarcoma is rare, it occurs in roughly 10% of patients with Ollier's disease. Pain is the most common symptom of chondrosarcoma. The treatment of low-grade chondrosarcoma ranges from intralesional excision to wide amputation. The intent of the surgery is to remove all the disease to decrease the chance of local recurrence. Lee FY, Mankin HJ, Fondren G, et al: Chondrosarcoma of bone: An assessment of outcome. J Bone Joint Surg Am 1999;81:326-338.

The radiographs show increased density and reactive bone formation. A faint nidus can be seen on the radiograph but is obvious on the CT scan. The initial therapy for an osteoid osteoma should be oral anti-inflammatory drugs. A biopsy or SPECT bone scan is not indicated because the osteoid osteoma is clearly seen on the CT scan. If the patient fails to respond to nonsurgical therapy, CT-guided radiofrequency ablation or surgical excision is indicated depending on the anatomic location. Frassica FJ, Waltrip RL, Sponseller PD, Ma LD, McCarthy EF Jr: Clinicopathologic features and treatment of osteoid osteoma and osteoblastoma in children and adolescents. Orthop Clin North Am 1996;27:559-574. Kneisl JS, Simon MA: Medical management compared with operative treatment for osteoid osteoma. J Bone Joint Surg Am 1992;74:179-185.

Current treatment regimens for Ewing's sarcoma typically involve induction chemotherapy followed by local control and further chemotherapy. Local control consists of surgery alone, radiation therapy alone, or a combination of the two. In bones that are easily resectable (or expendable) with wide margins, surgery alone is usually recommended. For areas that are unresectable (ie, large, bulky pelvic tumors), radiation therapy alone is sometimes the preferred method of local control. If surgery is chosen and margins are close, radiation therapy can be used as an adjuvant. Amputation rarely is required for an isolated fibular lesion. Observation without adequate local therapy results in local recurrence. Nesbit ME Jr, Gehan EA, Burgert EO Jr, et al: Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: A long-term follow-up of the First Intergroup study. J Clin Oncol 1990;8:1664-1674.

The patient has a solitary lesion that is at high risk for fracture. While metastatic lesions are most common in this age group, a stabilization procedure is contraindicated until results of a biopsy confirm the presence of a metastasis. Because of the lytic lesion and the associated calcification shown on the radiograph, the most likely diagnosis is a chondrosarcoma. Therefore, any type of stabilization procedure with a rod or plate would compromise a wide surgical excision to remove the entire proximal femur. Radiation therapy and chemotherapy are also contraindicated until biopsy results are obtained. Because of these factors, obtaining a frozen section biopsy specimen is considered the next most appropriate step in management. If a metastatic lesion is confirmed on the frozen section, a stabilization procedure could then be performed under the same anesthetic. Therefore, it is important to have a pathologist available at the time of a biopsy. 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 eccentric metaphyseal location, skeletal maturity, narrow zone of transition, and lack of symptoms suggest a benign process and are consistent with a healed nonossifying fibroma. These lesions typically fill in (ossify) with skeletal maturity, eventually remodeling and disappearing. Radiographic monitoring is indicated. Biopsy is not recommended unless the lesion changes radiographically. Marks KE, Bauer TW: Fibrous tumors of bone. Orthop Clin North Am 1989;20:377-393. Bullough PG, Walley J: Fibrous cortical defect and non-ossifying fibroma. Postgrad Med J 1965;41:672-676.

The greatest risk factor for local recurrence is an inadequate surgical margin. The tumor grade, histologic subtype, and size are predictive of systemic relapse. Sarcomas that arise in some anatomic sites, such as the forearm or retroperitoneum, may be more difficult to completely resect compared with other sites. The optimum margin is generally considered to be a cuff of normal tissue beyond the tumor. Bell RS, O'Sullivan B, Liu FF, et al: The surgical margin in soft-tissue sarcoma. J Bone Joint Surg Am 1989;71:370-375. Sadoski C, Suit HD, Rosenberg A, Mankin H, Efird J: Preoperative radiation, surgical margins, and local control of extremity sarcomas of soft tissues. J Surg Oncol 1993;52:223-230.

The patient has a high-grade osteosarcoma of the distal femur with a skip lesion, and pulmonary metastasis is seen on the CT scan. This corresponds to a stage III lesion according to the Musculoskeletal Tumor Society System as adopted from Enneking.

Calcitonin is a hormone that binds to osteoclasts and acts to decrease both osteoclast activity and number. Calcitonin is most effective in reducing vertebral compression fractures in high-turnover osteoporosis. It is also effective in treating regional osteoporosis. Because of its analgesic effect, it is helpful in treating painful acute compression fractures associated with osteoporosis.

In childhood, the more common soft-tissue sarcomas are rhabdomyosarcoma, synovial sarcoma, and fibrosarcoma. Rhabdomyosarcoma, treated with radiation therapy and chemotherapy, is a round cell tumor and is inconsistent with this patient's histologic findings. Synovial sarcoma can be monophasic or biphasic with both spindle and epithelial-like cells and is associated with the characteristic reciprocal chromosomal translocation of t(x:18)(p11;q11) which is not found in fibrosarcoma. Synovial sarcoma also can be associated with cystic loculated areas best seen in a T2-weighted MRI scan. Nonrhabdomyosarcoma childhood soft-tissue sarcomas are treated with surgical excision in conjunction with chemotherapy and/or radiation therapy. The histology reveals no inflammatory cells to suggest an abscess; therefore, antibiotics and drainage are unnecessary. The MRI scans clearly show a mass of soft tissue and no bone involvement; therefore, proximal femoral resection is not appropriate. Serial observation is not appropriate because of the history of enlargement and insidious onset. Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby Year Book, 1995, p 757.

Histologically, there is metaplastic cartilage arising from the synovium. These lobules of zonates hyaline cartilage are of variable size, are embedded within edematous synovium, and protrude into the joint. The lobules calcify and ossify, leading to the characteristic radiographic appearance. Inflammatory synovitis is not characteristic of synovial chondromatosis. The fluid is clear and serosanguin, not blood tinged. Milgram JM: Synovial osteochondromatosis: A histopathological study of thirty cases. J Bone Joint Surg Am 1977;l59:792-801.