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

The radiographs reveal phleboliths on the volar side of the forearm consistent with hemangioma. The MRI scan reveals a rather well-circumscribed in size, irregular in shape, intramuscular soft-tissue mass in the volar aspect of the distal right forearm within the flexor group musculature. The mass demonstrates heterogeneous mixed signal intensity in both T1- and T2-weighted sequences with increased signal intensity on the T1, suggesting fat within the tumor, typical of hemangioma. The postgadolinium-enhanced sequences demonstrate heterogeneous enhancement. The MRI findings are consistent with a soft-tissue hemangioma. Garzon M: Hemangiomas: Update on classification, clinical presentation and associate anomalies. Cutis 2000;66:325-328.

There are a number of important technical details in performing a biopsy. Incisions should always be longitudinal in the extremity. Good hemostasis is important in avoiding contamination from hematoma. The approach should avoid neurovascular structures, and go through a single muscle belly when possible. Although a frozen section should be obtained to ensure adequate viable tissue has been obtained, definitive diagnosis is not necessary at the time of the frozen section. Vaccaro AR (ed): Orthopaedic Knowledge Update 8. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2005, pp 197-215.

This is a case of synovial sarcoma. The radiograph shows some soft-tissue swelling in the upper arm. The MRI scans show a lesion that has increased signal on T2-weighted images and low signal on T1-weighted images. There is a suggestion of a large cystic component to this lesion. The pathology shows a biphasic population of cells, a spindle cell component, and an epithelioid component. Up to 20% of synovial cell sarcomas have areas of cyst formation. The most common cytogenetic translocation with synovial cell sarcoma is X; 18. The 11; 22 translocation is most commonly associated with Ewing's sarcomas; the 12; 22 translocation is most commonly associated with clear cell sarcomas; the 2; 13 translocation is most commonly associated with alveolar rhabdomyosarcomas, and the 12; 16 translocation is most commonly associated with myxoid liposarcomas. Kawai A, Woodruff J, Healey JH, et al: SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. New Engl J Med 1998;338:153-160.

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial sarcoma that is frequently misdiagnosed at presentation. It is frequently excised prior to suspecting that the lesion is a sarcoma and if not appropriately treated with tumor bed resection to obtain wide margins, these lesions have a high incidence of local recurrence. It is recommended that the wide excision include the deep fascia and a 2.5- to 3-cm cuff of normal-appearing skin. Distant disease spread is rare and usually occurs in the face of a multiply recurrent lesion. Despite the apparent gross circumscription of these lesions, the tumor diffusely infiltrates the dermis and subcutaneous tissues. A characteristic histologic finding can be seen in the deep margins of the tumor where it intricately interdigitates with normal fat. Lindner NJ, Scarborough MT, Powell GJ, et al: Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities. Eur J Surg Oncol 1999;25:392-397.

An intramuscular lipoma is a benign soft-tissue lesion that can grow and has a small risk of progressing to a liposarcoma. Radiographs usually show a globular radiolucent mass adjacent to higher-density muscle tissue shadows. When the patient has symptoms and reports an increase in size of the mass, the treatment of choice after appropriate radiographic analysis is complete excision of the mass with marginal resection. Sampling error is a problem with fatty lesions and core or incisional biopsies are frequently unnecessary, especially if an MRI scan of the lesion shows signal intensity that matches subcutaneous fat on all sequences. Damron TA: What to do with deep lipomatous tumors. Instr Course Lect 2004;53:651-655. Gaskin CM, Helms CA: Lipomas, lipoma variants, and well-differentiated liposarcomas (atypical lipomas): Results of MRI evaluations of 126 consecutive fatty masses. Am J Roentgenol 2004;182:733-739.

Giant cell tumor of bone has about a 2% risk of benign pulmonary metastasis in all cases and 6% risk in recurrent cases. The radiograph and MRI scans show a lytic destructive lesion in the distal radius with no matrix mineralization. The lesion extends up to the subchondral bone. In a young woman, the most likely diagnosis is giant cell tumor of bone, which is supported by the pathology results that show monotonous fibrovascular stroma with numerous multinucleated giant cells where the nuclei that make up the giant cells are identical to the nuclei that make up the background stromal cells. Athanasian EA, Wold LE, Amadio PC: Giant cell tumors of the bones in the hand. J Hand Surg Am 1997;22:91-98.

Localized PVNS is a form of the disease in which synovial proliferation is restricted to one area of a joint and causes the formation of a small mass-like lesion. The true incidence of this is unknown but is probably less common than the diffuse form of the disease. PVNS presents as a usually painful discrete mass. The anterior compartment of the knee is the most common location. Tyler WK, Vidal AF, Williams RJ, et al: Pigmented villonodular synovitis. J Am Acad Orthop Surg 2006;14:376-385.

The figures show a lesion of the proximal humerus consistent with an enchondroma. The lesion is calcified on the radiographs. There is no cortical destruction, significant endosteal scalloping, or soft-tissue mass. The bone scan shows mild uptake in the area of the proximal humerus, and the T2-weighted MRI scan shows a lesion with high uptake, suggesting a lesion with high water content. A CT scan could also be obtained to rule out bone destruction or periosteal reaction. Pain with overhead activities is likely related to the rotator cuff. A biopsy is unlikely to add information because of inherent difficulties interpreting low-grade cartilaginous lesions. Curettage and grafting and en bloc resection are excessive treatments for a benign lesion that is apparently asymptomatic. Observation with a follow-up radiograph in 3 to 6 months is appropriate. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, pp 103-111.

Ewing's sarcoma is the second most common bone tumor in children with an incidence of three per one million Caucasian children younger than 21 years of age. Ewing's sarcoma is rare in African Americans. Osteosarcoma is the most common bone tumor in children. Rhabdomyosarcoma is the most common soft-tissue sarcoma in children. Fibrosarcoma is a rare primary bone tumor most commonly seen in adults. Adamantinoma is a rare primary bone malignancy also most commonly seen in adults in the tibia. Menendez LR (ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2002, p 195.

This radiolucent lesion with a "fallen leaf sign" is typical for a unicameral bone cyst(UBC). The most appropriate treatment is to allow the fracture to heal with clinical and radiographic observation. Curettage and bone grafting is not the best initial management for UBC. Wide resection is not indicated for UBC. The proximal humerus is the most common site for UBC. While staging studies consisting of MRI, bone scan, and CT of the chest are appropriate for lesions suspected of being malignant, the classical appearance of this UBC is such that this work-up is not necessary initially. Following fracture healing, aspiration and injection of the cyst may be indicated. Dormans JP, Pill SG: Fractures through bone cysts: Unicameral bone cysts, aneurysmal bone cysts, fibrous cortical defects, and nonossifying fibromas. Instr Course Lect 2002;51:457-467.

This clinical picture is most consistent with periarticular erosions from gout. The patient has multiple periarticular lytic lesions in the hand. The laboratory studies show an elevated serum uric acid level, and the biopsy specimen demonstrates acute and chronic inflammation with prominent clefts. Therefore, the preferred treatment is systemic control of her gout. Radiation therapy, chemotherapy, and/or amputation should be considered for a malignancy; however, the pathology does not demonstrate any evidence of pleomorphism, high nuclear-to-cytoplasmic ratio, nuclear atypia, or mitotic activity. Antibiotics for an infectious process is a consideration, but the minimal elevation in the WBC count and erythrocyte sedimentation rate does not support an infectious process. Wise CM: Crystal-associated arthritis in the elderly. Clin Geriatr Med 2005;21:491-511.

The pathology demonstrates a very cellular chondroid matrix with multinucleated forms, atypia, and myxomatous regions. This is most consistent with a myxoid chondrosarcoma. The radiograph shows a well-circumscribed lesion in the superior and medial aspect of the right acetabulum. The CT and MRI scans confirm these same findings with no evidence of matrix mineralization or significant surrounding edema. Unfortunately, in this location with this appearance, the radiographic differential diagnosis includes all the diagnoses listed. Terek RM: Recent advances in the basic science of chondrosarcoma. Orthop Clin North Am 2006;37:9-14. Donati D, El Ghoneimy A, Bertoni F, et al: Surgical treatment and outcome of conventional pelvic chondrosarcoma. J Bone Joint Surg Br 2005;87:1527-1530.

The radiographs show a bone-producing lesion in the distal femoral metaphysis in this case of classic osteosarcoma presenting in the most common location, the distal femur. The coronal MRI scan reveals a marrow-occupying lesion with extension into the soft tissues. The histology shows osteoid production by pleomorphic cells consistent with an osteosarcoma. Ewing's sarcoma is a bone tumor characterized by uniform small blue cells on histology. Rhabdomyosarcoma is the most common childhood soft-tissue sarcoma. Osteomyelitis has an inflammatory appearance on histology. Malignant fibrous histiocytoma of bone has a lytic radiographic appearance and a pleomorphic storiform pattern without osteoid on histology. Wold LE, Adler CP, Sim FH, et al: Atlas of Orthopedic Pathology, ed 2. Philadelphia, PA, WB Saunders, 2003, p 179.

The radiograph shows a benign-appearing cortically based lesion eroding the underlying cortex, producing a saucer-shaped defect typical of a periosteal chondroma. The histology shows benign-appearing neoplastic cartilage. Although enchondroma would have the same histologic appearance, radiographs generally show a lesion with a central medullary epicenter. The benign-appearing histology does not support chondrosarcoma. Chondromyxoid fibroma will generally show histologic elements of its fibrous and myxoid components. Chondroblastoma typically demonstrates histologic findings of polyhedral cells separated by a chondroid matrix with pericellular, lattice-like "chicken wire" calcification. Schajowicz F: Tumors and Tumorlike Lesions of Bone: Pathology, Radiology, and Treatment, ed 2. Berlin, Springer-Verlag, 1994, pp 147-151.

Celiac sprue results in rapid gastrointestinal transit and fatty stools that impair the absorption of calcium and vitamin D and result in nutritional-deficiency osteomalacia with secondary hyperparathyroidism. The radiographs show marked osteopenia with brown tumors. A pathologic fracture is seen in the proximal humerus through a large brown tumor. Serum findings include low or normal calcium, low phosphate, elevated alkaline phosphatase, low 1,25(OH)2D, and increased PTH levels. Secondary hyperparathyroidism is associated with a variety of conditions including malabsorption syndromes. Potts JT: Parathyroid hormone: Past and present. J Endocrinol 2005;187:311-325. Corazza GR, Di Stefano M, Maurino E, et al: Bones in coeliac disease: Diagnosis and treatment. Best Pract Res Clin Gastroenterol 2005;19:453-465.

The biopsy specimens reveal a high-grade spindle cell lesion adjacent to an area of benign cartilage. This is consistent with a dedifferentiated chondrosarcoma. The radiograph shows a pathologic fracture through a lesion characterized by calcification within the left greater trochanter. Distal to the area of calcification, there is a more osteolytic, destructive appearance. Synovial sarcoma has a biphasic appearance histologically with areas of glandular differentiation that stain positive with keratin. Metastatic prostate cancer, although osteoblastic in appearance, would have a glandular histologic appearance. There is no cartilage in these lesions. Classic low-grade chondrosarcoma does not have an area of high-grade pleomorphic spindle cells within the lesion. A periosteal osteosarcoma is a surface-based lesion with a sunburst radiographic pattern. Although there may be cartilage in the lesion histologically, there are also malignant cells producing osteoid. Dedifferentiated chondrosarcoma is an aggressive, high-grade variant of chondrosarcoma. Wold LE, Adler CP, Sim FH, et al: Atlas of Orthopedic Pathology, ed 2. Philadelphia, PA, WB Saunders, 2003, p 269.

The lesion is a chordoma and the other listed choices can be eliminated based on the histology. Many tumors can occur in the sacrum including chordoma, multiple myeloma, giant cell tumor, aneurysmal bone cyst, and metastatic disease. The histology in this patient shows a lobulated lesion on low power with fibrous septae separating the lobules. At higher magnification, the cells have eosinophilic vacuolated cytoplasm and are called physaliferous cells. Chordoma is a low-grade neoplasm that most commonly occurs in the sacrum and rarely in the base of the skull. The diagnosis is often delayed. Chordoma is thought to originate from notochordal remnants. Chordoma typically occurs in the midline and has an associated soft-tissue mass. Wold LE, Adler CP, Sim FH, et al: Atlas of Orthopedic Pathology, ed 2. Philadelphia, PA, WB Saunders, 2003, p 372. Fuchs B, Dickey ID, Yaszemski MJ, et al: Operative management of sacral chordoma. J Bone Joint Surg Am 2005;87:2211-2216.

This is a conventional chondrosarcoma. The radiograph and the CT scan show a lesion arising from the inferior pubic ramus with a large soft-tissue mass. Abundant punctate, stippled, or "popcorn-like" calcification is present. The photomicrograph demonstrates hypercellular cartilage. Surgical resection is the only effective treatment. Whereas chemotherapy might play a role in the treatment of a dedifferentiated chondrosarcoma, it has no role in the treatment of a conventional chondrosarcoma. Chondrosarcomas are relatively radioresistant. Donati D, El Ghoneimy A, Bertoni F, et al: Surgical treatment and outcome of conventional pelvic chondrosarcoma. J Bone Joint Surg Br 2005;87:1527-1530. 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 findings are consistent with an aneurysmal bone cyst. The MRI scan demonstrates a lesion involving the posterior elements of the vertebrae with fluid-fluid levels and neural compression. Fibrovascular tissue with multinucleated giant cells surrounding a vascular lake is seen on the histology. The most appropriate treatment is a marginal resection of the involved posterior elements. Although the recurrence rate can be as high as 25% to 30%, wide surgical resection could result in permanent neurologic injury and is not necessary. Aspiration and steroid injection have been advocated but would not relieve the nerve compression in this patient. Radiation therapy and chemotherapy are not indicated. Mankin HJ, Hornicek FJ, Ortiz-Cruz E, et al: Aneurysmal bone cyst: A review of 150 patients. J Clin Oncol 2005;23:6756-6762.

The imaging studies show a permeative lytic destructive lesion in the proximal radius with "hair-on-end" periosteal reaction and a large soft-tissue mass most consistent with Ewing's sarcoma. The pathology reveals monotonous sheets of "round blue" cells. This limits the differential diagnosis to primary lymphoma of bone versus Ewing's sarcoma. These are best differentiated by immunohistochemistry, cytogenetics, and flow cytometry. Lymphoma of bone is typically CD34 positive and CD99 negative; whereas, the reverse is true of Ewing's sarcoma, CD34 negative and CD99 positive. The most common cytogenetic translocation with Ewing's sarcoma is 11; 22; 21; 22 and 7; 22 translocations have also been reported in Ewing's sarcomas. The X; 18 translocation is most commonly associated with synovial cell sarcomas; the 12; 22 translocation is most commonly associated with clear cell sarcomas; the 2; 13 translocation is most commonly associated with alveolar rhabdomyosarcomas, and the 12; 16 translocation is most commonly associated with myxoid liposarcomas. Flow cytometry is used to characterize the cell types of lymphomas. Womer R: The cellular biology of bone tumors. Clin Orthop Relat Res 1991;262:12-21. Yamaguchi U, Hasegawa T, Morimoto Y, et al: A practical approach to the clinical diagnosis of Ewing's sarcoma/primitive neuroectodermal tumour and other small round cell tumours sharing EWS rearrangement using new fluorescence in situ hybridisation probes for EWSR1 on formalin fixed, paraffin wax embedded tissue. J Clin Pathol 2005;58:1051-1056.

This classic case of osteosarcoma illustrates the typical radiographic and histologic characteristics of this disease. The radiographs show an aggressive-appearing lesion of the distal femur. The lesion has both lytic and blastic areas. Periosteal reaction is present in the form of a Codman's triangle. The radiographs are highly suggestive of osteosarcoma. The photomicrographs show malignant spindle cells that produce osteoid, thus confirming the diagnosis of osteosarcoma. Treatment of osteosarcoma is multimodal including multi-agent chemotherapy and surgery (wide resection or amputation). Bacci G, Ferrari S, Bertoni F, et al: Long-term outcome for patients with nonmetastatic osteosarcoma of the extremity treated at the Instituto Ortopedico Rizzoli according to the Instituto Ortopedico Rizzoli/osteosarcoma-2 protocol: An updated report. J Clin Oncol 2000;18:4016-4037. Bielack SS, Kempf-Bielack B, Delling G, et al: Prognostic factors in high-grade osteosarcoma of the extremities or trunk: An analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols. J Clin Oncol 2002;20:776-790.

The most significant impact on long-term survival is the presence or absence of identifiable metastatic disease on initial presentation. All of these factors have been shown to be predictive of long-term survival to varying degrees. Enneking WF, Spanier SS, Goodman MA: A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop Relat Res 1980;153:106-120.

The patient has a large deep anterior thigh mass that has imaging characteristics of mature fat. Intramuscular lipomas are effectively treated with marginal resections with very low recurrence rates. Large lipomas often have small amounts of intralesional signal changes frequently representing trapped muscle fibers and do not necessitate more extensive margins. Gaskin CM, Helms CA: Lipomas, lipoma variants, and well-differentiated liposarcomas (atypical lipomas): Results of MRI evaluations of 126 consecutive fatty masses. Am J Roentgenol 2004;182:733-739.

The definitive surgery would be removal of the entire resection bed, and in this case of dedifferentiated chondrosarcoma, a hemipelvectomy was performed. The MRI and CT scans show an aggressive cartilage lesion. The histology, representative of a dedifferentiated chondrosarcoma, shows a bimorphic low-grade cartilage lesion with high-grade spindle cell sarcoma. The cartilage lesion is usually an enchondroma or low-grade chondrosarcoma. The dedifferentiated portion is typically a malignant fibrous histocytoma, osteosarcoma, or fibrosarcoma. Weber KL, Pring ME, Sim FH: Treatment and outcome of recurrent pelvic chondrosarcoma. Clin Orthop Relat Res 2002;397:19-28.

Radiation therapy is commonly used as an adjuvant in the treatment of soft-tissue sarcomas, but a controversy exists whether it should be preoperative or postoperative. Radiation therapy can be given prior to or following resection of the tumor. Postoperative radiation is usually given in a higher dose to a larger treatment field. This commonly results in a higher incidence of fibrosis and lymphedema. There is no statistical difference in local recurrence rate between the two radiation treatment plans. Neuropathy is more commonly a complication of chemotherapy. Preoperative radiation therapy has been shown to have a higher wound complication rate than postoperative radiation. Vaccaro AR (ed): Orthopaedic Knowledge Update 8. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2005, pp 197-215.