Question 76
A 19-year-old girl has had pain and swelling in the right ankle for the past 4 months. She denies any history of trauma. Examination reveals a small soft-tissue mass over the anterior aspect of the ankle and slight pain with range of motion of the ankle joint. The examination is otherwise unremarkable. A radiograph and MRI scan are shown in Figures 45a and 45b, and biopsy specimens are shown in Figures 45c and 45d. What is the most likely diagnosis?
Explanation
45b 45c 45d Giant cell tumors typically occur in a juxta-articular location involving the epiphysis and metaphysis of long bones, usually eccentric in the bone. The radiographs show a destructive process within the distal tibia and an associated soft-tissue mass. The histology shows multinucleated giant cells in a bland matrix with a few scattered mitoses. Osteosarcoma can have a similar destructive appearance but a very different histologic pattern with osteoid production. Ewing's sarcoma also can have a diffuse destructive process in the bone. The histologic pattern of Ewing's sarcoma is diffuse round blue cells. Aneurysmal bone cysts typically are seen as a fluid-filled lesion on imaging studies and have only a scant amount of giant cells histologically. Metastatic adenocarcinoma does not demonstrate the pattern shown in the patient's histology specimen. Wold LA, et al: Atlas of Orthopaedic Pathology. Philadelphia, PA, WB Saunders, 1990, pp 198-199.
Question 77
A 13-year-old boy has had a painless mass in the arm for the past 2 months. An MRI scan and biopsy specimens are shown in Figures 46a through 46c. What is the most likely diagnosis?
Explanation
46b 46c Nodular fasciitis is a benign soft-tissue lesion that usually arises from the fascia and is often misdiagnosed as a sarcoma. Desmoid tumors (aggressive fibromatosis) are also benign tumors with a greater tendency for local recurrence. Desmoid tumors have more spindle-shaped fibroblasts in an abundant collagenous matrix. Malignant fibrous histiocytoma is a hypercellular pleomorphic sarcoma more commonly found in adults. The histology is not consistant with a fatty tumor.
Question 78
A 20-year-old man has a symptomatic lesion of fibrous dysplasia in the femoral neck. Management should consist of
Explanation
Fibrous dysplasia in the femoral neck frequently warrants treatment because of the risk of pathologic fracture. Cortical strut grafts reduce the risk of local recurrence compared with cancellous bone grafting. Because of the consequences associated with fracture in this location, prophylactic fixation is recommended. Radiation therapy and chemotherapy are not used for this benign condition. Simon M, et al: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, pp 197.
Question 79
In addition to radiographs of the primary lesion and chest, MRI of the primary lesion, and CT of the chest, staging studies for Ewing's sarcoma should include which of the following?
Explanation
A bone scan and bone marrow biopsy are part of the staging studies for Ewing's sarcoma. Whole body MRI and PET scans are investigational and show promise of greater sensitivity than a bone scan. Schleiermacher G, Peter M, Oberlin O, Philip T, Rubie H, Mechinaud F, et al: Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized ewing tumor. J Clin Oncol 2003;21:85-91.
Question 80
Which of the following conditions is transmitted by an autosomal dominant trait?
Explanation
Multiple hereditary exostosis is transmitted by an autosomal dominant trait. Li-Fraumeni syndrome and retinoblastoma are autosomal recessive or associated with autosomal recessive mutations. No genetic predisposition to Ollier's disease or Maffucci's syndrome has been identified. Mirra J (ed): Bone Tumors: Clinical, Radiologic and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989, p 1627.
Question 81
A previously healthy 13-year-old girl has had thigh pain for the past 3 weeks. The radiograph shown in Figure 47a reveals a lesion in the right femur. A bone scan and CT scan of the chest show no evidence of other lesions. A biopsy specimen is shown in Figure 47b. What is the most likely diagnosis?
Explanation
47b The patient has Langerhans cell histiocytosis that may be solitary (eosinophilic granuloma) or associated with systemic illness (Hand-Schuller-Christian disease and Letterer-Siwe disease). The solitary form of the disease, eosinophilic granuloma, typically affects patients in the first three decades of life. Radiographically, it is characterized as a well-defined, lytic, "punched out" intramedullary lesion. Histologically, two cell types, eosinophils and Langerhans cells, are seen. The Langerhans cells are seen as mononuclear histiocyte-like cells with oval nuclei with well-defined round or oval cytoplasm. A prominent nuclear groove can be seen in most of the nuclei (coffee bean nuclei). A mixture of inflammatory cells and lipid-laden foam cells with nuclear debris may be present as well. The lack of nuclear atypia and atypical mitoses excludes malignant conditions such as Ewing's sarcoma, lymphoma of bone, and metastatic neuroblastoma. The lack of acute inflammatory cells excludes the diagnosis of osteomyelitis. The eosinophils have bi-lobed nuclei and granular eosinophilic cytoplasm. Dorfman H, Czerniak B: Bone Tumors. St Louis, MO, Mosby, 1988.
Question 82
A 50-year-old man with metastatic renal cell carcinoma has right hip pain. A radiograph and CT scan are shown in Figures 48a and 48b. The first step in management should consist of
Explanation
48b These lesions are extremely vascular and can cause uncontrolled intraoperative bleeding; therefore embolization is the appropriate first treatment. Because the radiograph and CT scan show a lytic lesion in the supra-acetabular region that affects the weight-bearing dome and medial wall, the next step in treatment would most likely be a total hip arthroplasty and acetabular reconstruction. Treatment with bisphosphonates and radiation therapy will not prevent an acetabular fracture. Cementoplasty is an emerging technique in which cement is injected percutaneously into a lesion, but no long-term results have been reported. Radiofrequency ablation of bone metastases is also an emerging technique that provides palliative pain control. Layalle I, Flandroy P, Trotteur G, Dondelinger RF: Arterial embolization of bone metastases: Is it worthwhile? J Belge Radiol 1998;81:223-225.
Question 83
What is the most common benign bone tumor in childhood?
Explanation
The most common benign bone tumor in childhood is a nonossifying fibroma. It is estimated that 30% of children have a nonossifying fibroma. In most patients, the lesion is not identified until a radiograph is obtained for unrelated reasons. Similarly, most identified cases of fibrous cortical defect are not biopsied because the radiographic and clinical presentations are diagnostic. Aboulafia AJ, Kennon RE, Jelinek JS: Benign bone tumors of childhood. J Am Acad Orthop Surg 1999;7:377-388.
Question 84
A 13-year-old patient has foot drop and lateral knee pain. AP and lateral radiographs and an MRI scan are shown in Figures 49a through 49c. A biopsy specimen is shown in Figure 49d. What is the preferred method of treatment?
Explanation
49b 49c 49d The "sunburst" radiographic appearance suggests an osteosarcoma, and the histologic findings confirm the diagnosis with malignant cells surrounded by pink osteoid. MRI scans are not particularly helpful in the diagnosis of osteosarcoma but are mandatory for surgical planning. Osteosarcomas are high-grade sarcomas that are best treated with chemotherapy and wide resection. Even though the peroneal nerve is involved, limb salvage is indicated. Survival after limb salvage is equivalent to amputation, with better function. Goorin AM, Abelson HT, Frei E: Osteosarcoma: Fifteen years later. N Engl J Med 1985;313:1637. Link MP, Goorin AM, Miser AW, et al: The effect of adjuvant chemotherapy on relapse-free survival in patients with osteosarcoma of the extremity. N Engl J Med 1986;314:1600.
Question 85
A 13-year-old girl is diagnosed with a stage IIB osteosarcoma of the proximal tibia. Following neoadjuvant chemotherapy, local control should consist of
Explanation
Local control of osteosarcoma consists of wide resection and reconstruction. Radiation therapy is not recommended except in unresectable lesions or for palliation. Curettage and bone grafting result in intralesional resection with an unacceptable high rate of local recurrence. Chemotherapy alone is not adequate for local control. Simon M, Springfield D, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue. Philadelphia, PA, Lippincott Raven, 1998, p 274.
Question 86
A 23-year-old woman has had vague left knee pain for the past 6 months. A radiograph and CT scan are shown in Figures 50a and 50b. What is the most likely diagnosis?
Explanation
50b The radiographic appearance of the lesion emanating from the posterior cortex of the left distal femur is consistent with a surface bone-producing lesion; therefore, the most likely diagnosis is a parosteal osteosarcoma. In an osteochondroma, the cortex and medullary cavity of the lesion are in continuity with that of the native bone. A dedifferentiated chondrosarcoma has histologic components of a high-grade sarcoma plus a benign or low-grade malignant cartilage tumor. Tumoral calcinosis is characterized by amorphous calcium in the soft tissues and does not emanate from the bone itself. While often confused with parosteal osteosarcoma, myositis ossificans is usually more mature at the periphery of the lesion rather than the center. In addition, myositis ossificans does not involve the underlying cortex but remains separate from the bone. Unni KK: Dahlin's Bone Tumors: General Aspects and Data on 11,087 Cases, ed 5. Philadelphia, PA, Lippincott-Raven, 1996, pp 185-196.
Question 87
What is the preferred treatment of a patient with breast cancer and a pathologic fracture of the clavicle in her dominant arm?
Explanation
Closed management should be attempted for upper extremity pathologic fractures, particularly the clavicle. If nonunion or pain persists, surgery may be indicated. Radiofrequency ablation is not indicated for subcutaneous bones. Early motion is likely to cause increased pain and disability. Weber KC, Lewis VO, Randall RL, Lee AK, Springfield D: An approach to the management of the patient with metastatic bone disease. Instr Course Lect 2004;53:663-676.
Question 88
A 14-year-old boy has an asymptomatic mass on the right arm. MRI scans and biopsy specimens are shown in Figures 51a through 51d. Immunostaining is positive for desmin. Additional staging studies should include
Explanation
51b 51c 51d The patient has rhabdomyosarcoma. Axilliary node and bone marrow biopsy are part of the staging because about 12% of patients with rhabdomyosarcoma of the extremity have evidence of lymph nodes metastases at presentation. Bone marrow metastases have been shown to portend a worse prognosis. Lawrence W, Jr., Hays DM, Heyn R, Tefft M, Crist W, Beltangady M, et al: Lymphatic metastases with childhood rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study. Cancer 1987;60:910-915.
Question 89
A 15-year-old girl has left knee pain and an enlarging mass in the distal thigh. AP and lateral radiographs are shown in Figures 52a and 52b, and a biopsy specimen is shown in Figure 52c. What is the most likely diagnosis?
Explanation
52b 52c A bone-producing lesion in the metaphysis of an adolescent is most likely an osteosarcoma. The radiographs show a distal femoral bone-producing lesion extending into the surrounding soft tissues. The histologic appearance consists of pleomorphic cells producing osteoid. Ewing's sarcoma and metastatic neuroblastoma do not produce a matrix. Chondrosarcoma is a radiographically destructive lesion with calcification and cartilage cells on histologic section. An osteochondroma is a benign cartilage lesion that is continuous with the medullary cavity of the underlying bone and extends into a bony lesion and covered by a cartilage cap. Unni KK: Dahlin's Bone Tumors: General Aspects and Data on 11,087 Cases, ed 5. Philadelphia, PA, Lippincott-Raven, 1996, pp 143-160.
Question 90
Survival rates for children with soft-tissue sarcoma other than rhabdomyosarcoma are best correlated with
Explanation
In review of 154 patients with nonrhabdomyosarcoma, Rao reported that histologic grade, tumor invasiveness, and adequate surgical margin were the most important prognostic factors. Histologic subtype, use of adjuvant chemotherapy, and patient age were not as important. Size related to degree of invasiveness was not statistically significant. Rao BN: Nonrhabdomyosarcoma in children: Prognostic factors influencing survival. Semin Surg Oncol 1993;9:524-531.
References:
- Andrassy R, et al: Non-rhabdomyosarcoma Soft-Tissue Sarcomas: Pediatric Surgical Oncology. Philadelphia, PA, WB Saunders, p 221.
Question 91
A 25-year-old woman has had pain and stiffness in her knee following a motor vehicle accident 9 months ago. The radiograph, CT scan, MRI scan, and biopsy specimen are shown in Figures 53a through 53d. What is the most likely diagnosis?
Explanation
53b 53c 53d Heterotopic ossification may occur spontaneously or following trauma. The imaging studies and histology reveal mature fatty bone marrow and trabecular bone. Osteochondromas are cortically based with the medullary canal extending into the lesion. This is not evident in this patient. Also, no obvious cartilage cap is present. Parosteal osteosarcoma commonly occurs in the posterior distal femoral cortex but is ruled out by the lack of the typical fibrous stromal cells forming the low-grade malignant osteoid. The histology and clinical presentation eliminate osteomyelitis and osteoblastoma. Horne LT, Blue BA: Intra-articular heterotopic ossification in the knee following intramedullary nailing of the fractured femur using a retrograde method. J Orthop Trauma 1999;13:385-388. Stannard JP, Wilson TC, Sheils TM, McGwin G Jr, Volgas DA, Alonso JE: Heterotopic ossification associated with knee dislocation. Arthroscopy 2002;18:835-839.
Question 92
Which of the following lesions most closely resembles Ewing's sarcoma histologically?
Explanation
Ewing's sarcoma is characterized by small round blue cells. Lesions with a similar appearance include lymphoma, primitive neuroectodermal tumor, rhabdomyosarcoma, small cell lung tumor, and metastatic neuroblastoma. Karyotyping, immunohistochemistry, and electron microscopy can help differentiate these lesions.
Question 93
Primary chondrosarcoma of bone most commonly occurs in which of the following locations?
Explanation
The most common location of chondrosarcoma is the pelvis (30%), followed by the proximal femur (20%) and shoulder girdle (15%). Chondrosarcoma rarely affects the spine or hand. Lee FY, Mankin HJ, Fondren G, et al: Chondrosarcoma of bone: An assessment of outcome. J Bone Joint Surg Am 1999;81:326-338.
Question 94
An 18-year-old man has had an enlarging mass in his hand for the past 3 months. Radiographs, an MRI scan, and biopsy specimens are shown in Figures 54a through 54d. What is the most likely diagnosis?
Explanation
54b 54c 54d Also known as Nora's lesion, BPOP is a benign osteocartilaginous tumor that almost always occurs in the hands and feet; one occurrence each in the femur and tibia has been reported. Although local recurrence is common after excision, metastases have not been reported. Abramovici L, Steiner GC: Bizarre parosteal osteochondromatous proliferation (Nora's lesion): A retrospective study of 12 cases, 2 arising in long bones. Hum Pathol 2002;33:1205-1210.
Question 95
A 55-year-old man has had a mass in his right thigh for the past 2 months. An MRI scan and biopsy specimens are shown in Figures 55a through 55c. What is the most likely diagnosis?
Explanation
55b 55c The histology shows extraskeletal myxoid chondrosarcoma, characterized by abundant blue myxoid matrix with cords and nests of small tumor cells. Treatment consists of wide resection. Despite the name, hyaline cartilage is not a common component of these tumors. Adult rhabdomyosarcoma and malignant fibrous histiocytoma are highly pleomorphic sarcomas often containing multinucleated giant cells. Myxoid liposarcoma contains a prominent capillary network and lipoblasts. Myxoma is less cellular than extraskeletal myxoid chondrosarcoma and does not have a cord-like arrangement of tumor cells.
Question 96
Figures 56a through 56c show the radiograph, CT scan, and biopsy specimen of a 44-year-old man who underwent chemotherapy and radiation therapy for lymphoma of the distal femur 20 years ago. His current problem is most likely related to
Explanation
56b 56c The patient has changes consistent with radiation therapy to the femur, including osteopenia and an aggressive appearing neoplasm. The tumor is most likely a radiation-induced sarcoma. This is more likely than recurrent lymphoma at this late date. It is not related to steroid use or a primary lung tumor. Mirra J (ed): Bone Tumors: Clinical, Radiologic and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989, p 353.
Question 97
What is the most common reason an individual with a malignant soft-tissue tumor in the extremities seeks medical attention?
Explanation
Unlike malignant bone tumors, malignant soft-tissue tumors usually are asymptomatic and present with the presence of a mass. Malignant soft-tissue tumors enlarge by centrifugal growth, creating a mass while compressing surrounding tissue. Symptoms may develop as the result of direct compression on neurovascular structures as the tumor enlarges. This is especially true in the pelvis where the tumor can enlarge appreciably without being noticed. However, in the extremities, the tumor is most often apparent before neurologic symptoms develop. An asymptomatic mass is not necessarily benign; therefore, biopsy should not be delayed. It is uncommon for a malignant soft-tissue mass to be discovered incidentally. Soft-tissue tumors are not typically apparent on radiographs; they are best identified with MRI. Brouns F, Stas M, De Wever I: Delay in diagnosis of soft tissue sarcomas. Eur J Surg Oncol 2003;29:440-445. Rougraff B: The diagnosis and management of soft tissue sarcomas of the extremities in the adult. Curr Probl Cancer 1999;23:1-50.
Question 98
Exostoses in which of the following anatomic locations is the most likely to undergo malignant transformation in a patient with multiple hereditary exostosis (MHE)?
Explanation
Although osteochondromas can occur in almost every bone in patients with MHE, proximally located lesions are more likely to undergo malignant transformation. Annual radiographs of the shoulder girdles and pelvis are indicated in patients with MHE. Any enlarging osteochondromas are a concern as possible malignancies. Peterson HA: Multiple hereditary osteochondromata. Clin Orthop 1989;239:222.
Question 99
Initial management of a pathologic fracture of the humerus secondary to a unicameral bone cyst should include
Explanation
Most pathologic humeral fractures secondary to a unicameral bone cyst are minimally displaced and should be immobilized and allowed to heal. Persistent and/or progressive lesions may require treatment. Various treatments of unicameral bone cysts have been described. Acceptable treatment options include curettage and bone grafting, intralesional steroid injection, and percutaneous grafting with bone graft substitutes. MRI is not indicated when the diagnosis of unicameral bone cyst is known. Wilkins RM: Unicameral bone cysts. J Am Acad Orthop Surg 2000;8:217-224.
Question 100
An 11-year-old child has Ewing's sarcoma of the femoral diaphysis with a small soft-tissue mass. Staging studies show no evidence of metastases. Treatment should consist of
Explanation
The use of chemotherapy has dramatically improved survival rates of patients with Ewing's sarcoma. Local disease is best handled with wide resection to decrease local recurrence and to avoid the complications of radiation therapy (ie, secondary sarcomas). Radiation therapy alone is reserved for unresectable lesions or poor surgical margins. Amputation generally is not necessary. Toni A, Neff JR, Sudanese A, et al: The role of surgical therapy in patients with non-metastatic Ewing's sarcoma of the limbs. Clin Orthop 1991;286:225. Picci P, Rougraff BT, Bacci G, et al: Prognostic significance of histopathologic response to chemotherapy in non-metastatic Ewing's sarcoma of the extremities. J Clin Oncol 1993;11:1763.
Finish Exam?
You cannot change answers after submitting.