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Question 2001

Topic: 10. Pathology and Oncology

A 30-year-old man has had intermittent swelling of his right ankle for the past 6 months. He denies any history of trauma. Radiographs reveal osteolytic changes on both sides of the joint. An axial CT scan and a T2-weighted MRI scan are shown in Figures 40a and 40b. He undergoes surgical excision. An intraoperative photograph and a biopsy specimen are shown in Figures 40c and 40d. What is the most likely diagnosis?

. Synovial sarcoma
. Infection
. Pigmented villonodular synovitis
. Malignant fibrous histiocytoma
. Synovial chondromatosis

Correct Answer & Explanation

. Pigmented villonodular synovitis


Explanation

Pigmented villonodular synovitis often presents with intermittent swelling and minimal pain. It often occurs around joints but may be found around tendon sheaths and bursal linings. Periarticular erosions involving both sides of joints are typical, and multiple joint involvement has been described. Portions of low-signal intensity on T1- and T2-weighted images are characteristic of hemosiderin-laden processes. High-signal content is suggestive of high water content. The combination of low-signal intensity areas in intra-articular lesions with or without osseous destruction is diagnostic of pigmented villonodular synovitis. Aspiration reveals bloody or brownish fluid. The treatment of choice is synovectomy performed arthroscopically or open. Recurrence is common. Walling AK: Soft tissue and bone tumors, in Coughlin MJ, Mann RA (eds): Surgery of the Foot and Ankle, ed 7. St Louis, MO, Mosby, 1999, pp 1007-1032.

Question 2002

Topic: 10. Pathology and Oncology

Figure 10 shows the MRI scan of a 56-year-old woman with metastatic breast cancer who now reports progressive paraparesis. Her general health remains good. Treatment should consist of

Spine Surgery Board Review 2006: High-Yield MCQs (Set 2) - Figure 1

. posterior spinal fusion for stabilization, followed by radiation therapy of the anterior tumor.
. radiation therapy and a brace.
. chemotherapy, then reevaluation.
. anterior corpectomy and stabilization.
. laminectomy.

Correct Answer & Explanation

. anterior corpectomy and stabilization.


Explanation

If the patient's medical condition and prognosis remain good in the presence of significant and progressive neurologic deficit from cord compression, then the most reliable means of restoring function is via surgical decompression and fusion. Decompression should be directed toward the compressing structure (eg, anteriorly if the compression is from the anterior side). This procedure can be done via a posterolateral technique, such as costotransversectomy in some cases.

Question 2003

Topic: 10. Pathology and Oncology

Figures 32a and 32b show the radiographs of an active 13-year-old boy who has persistent left thigh pain and a limp despite a trial of protected weight bearing. Management should consist of

. curettage and bone grafting.
. systemic chemotherapy.
. an intralesional steroid injection.
. en block resection with autograft reconstruction.
. low-dose radiation therapy.

Correct Answer & Explanation

. curettage and bone grafting.


Explanation

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.

Question 2004

Topic: 10. Pathology and Oncology
What type of multiple lesions is associated with Maffucci's syndrome?
. Nonossifying fibromas
. Enchondromas
. Langerhan's cell histiocytosis
. Osteochondromas
. Giant cell tumors

Correct Answer & Explanation

. Enchondromas


Explanation

Maffucci's syndrome is a form of enchondromatosis associated with subcutaneous and deep hemangiomas. Similar to Ollier's disease, the risk of malignant transformation of the enchondromas is much higher than that of a solitary enchondroma. Multifocal nonossifying fibromas associated with other clinical findings such as mental retardation and cafรฉ-au-lait spots is known as Jaffe-Campanacci syndrome. There are two types of multifocal forms of histiocytosis: Letterer-Siwe and Hand-Schรผller-Christian disease.

Question 2005

Topic: 10. Pathology and Oncology

A 58-year-old woman has had a painless periscapular mass for the past year. An MRI scan and biopsy specimen are shown in Figures 4a and 4b. What is the most likely diagnosis?

. Elastofibroma
. Desmoid tumor
. Nodular fasciitis
. Malignant fibrous histiocytoma
. Atrophic serratus anterior

Correct Answer & Explanation

. Elastofibroma


Explanation

Elastofibroma is a rare tumor that most commonly occurs in adults who are older than age 55 years. The lesions usually grow between the chest wall and the scapula, and 10% are bilateral. Histologic analysis shows that they are composed of equal amounts of elastin and collagen with occasional fibroblasts. Briccoli A, Casadei R, Di Renzo M, Favale L, Bacchini P, Bertoni F: Elastofibroma dorsi. Surg Today 2000;30:147-152.

Question 2006

Topic: Bone Tumors

A 9-year-old girl reports progressive right knee pain. Radiographs are shown in Figures 59a and 59b. Work-up reveals no other sites of disease. Low- and high-power photomicrographs are shown in Figures 59c and 59d. What is the most appropriate treatment?

. Chemotherapy alone
. Surgery alone
. Radiation therapy alone
. Chemotherapy and surgery
. Radiation therapy and surgery

Correct Answer & Explanation

. Chemotherapy and surgery


Explanation

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.

Question 2007

Topic: 10. Pathology and Oncology
Figures 30a and 30b show the MRI scans of a 51-year-old woman who has had an enlarging soft-tissue mass in her right thigh for the past 18 months. Examination reveals no inguinal adenopathy. Results of a biopsy show a high-grade sarcoma. A bone scan is unremarkable, and a CT scan of the chest shows no evidence of pulmonary metastasis. According to the Musculoskeletal Tumor Society (MSTS) staging system, the tumor should be classified as what stage?
. 1
. IA
. IB
. IIA
. IIB

Correct Answer & Explanation

. IIA


Explanation

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.

Question 2008

Topic: 10. Pathology and Oncology

A 17-year-old woman seen in the emergency department reports right knee pain and swelling that has progressively worsened over the past several weeks. Radiographs are shown in Figures 31a and 31b. What is the most likely diagnosis?

. Giant cell tumor
. Infection
. Chondrosarcoma
. Osteosarcoma
. Chondroblastoma

Correct Answer & Explanation

. Osteosarcoma


Explanation

The radiographs reveal a blastic lesion of the proximal tibial metaphysis with cortical destruction, mineralization extending into the soft tissue laterally, indistinct margins, and destruction of the normal trabecular pattern. In this age group, with this aggressive appearance, osteosarcoma is the most likely diagnosis. Chondroblastoma and giant cell tumor are generally geographic and lytic. Chondrosarcoma is rare in this age group and would likely be a secondary lesion from an underlying chondroid tumor that is not present here. Whereas infection can have a wide variety of appearances, it tends to be more lytic in the acute presentation. Sanders TG, Parsons TW: Radiographic imaging of musculoskeletal neoplasia. Cancer Control 2001;8:221-231.

Question 2009

Topic: 10. Pathology and Oncology

Figures 1a through 1c show the radiograph and MRI scans of a 16-year-old patient who has a painful hip. Examination reveals a significant limp, limited abduction and internal rotation, and severe pain with internal rotation and adduction. A biopsy specimen is shown in Figure 1d. What is the deposited pigment observed in this condition?

. Hemoglobin
. Myoglobin
. Melanin
. Copper
. Hemosiderin

Correct Answer & Explanation

. Hemosiderin


Explanation

Pigmented villonodular synovitis (PVNS) is a synovial proliferative disorder that remains a diagnostic difficulty. The most common clinical features are mechanical pain and limited joint motion. On radiographs, the classic finding is often a large lesion, associated with multiple lucencies. Other findings may include a normal radiographic appearance, loss of joint space, osteonecrosis of the femoral head, or acetabular protrusion. MRI is the imaging modality of choice and will show the characteristic findings of a joint effusion, synovial proliferation, and bulging of the hip. The synovial lining has a low signal on T1- and T2-weighted images, secondary to hemosiderin deposition. Copper deposition occurs in patients with Wilson's disease, which mainly affects the liver. Bhimani MA, Wenz JF, Frassica FJ: Pigmented villonodular synovitis: Keys to early diagnosis. Clin Orthop 2001;386:197-202.

Question 2010

Topic: Bone Tumors

A 65-year-old man has a painful right hip mass that has been growing for several years. A radiograph, CT scan, and photomicrograph are shown in Figures 56a through 56c. What is the most appropriate treatment?

. Chemotherapy alone
. Surgery alone
. Radiation therapy alone
. Chemotherapy and surgery
. Radiation therapy and surgery

Correct Answer & Explanation

. Surgery alone


Explanation

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.

Question 2011

Topic: 10. Pathology and Oncology

A 20-year-old woman has had wrist pain for the past 5 months. A radiograph, MRI scans, and biopsy specimen are shown in Figures 46a through 46d. The patient is then treated with intralesional surgery. The patient should be counseled that her risk of developing lung metastasis is approximately what percent?

. 0%
. 4%
. 15%
. 25%
. 50%

Correct Answer & Explanation

. 4%


Explanation

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.

Question 2012

Topic: 10. Pathology and Oncology

Figures 37a and 37b show radiographs of a 24-year-old man who has a humeral bone lesion that was found during a screening chest radiograph. He denies any symptoms despite leading a very active lifestyle. What is the most likely diagnosis?

. Fibrous dysplasia
. Enchondroma
. Ewing's sarcoma
. Metastatic carcinoma
. Aneurysmal bone cyst

Correct Answer & Explanation

. Fibrous dysplasia


Explanation

The radiographs reveal a geographic, diaphyseal lesion with very subtle cortical expansion, cortical thinning, relatively sharp demarcation, and angular rather than rounded borders, suggesting a fibrous bone lesion. This lesion demonstrates the classic ground glass appearance of fibrous dysplasia. Ewing's sarcoma, metastases, and aneurysmal bone cyst all typically have a more aggressive appearance. Parsons TW: Benign bone tumors, in Fitzgerald RH, Kaufer H, Malkani AL (eds): Orthopaedics. St Louis, MO, Mosby, 2002, pp 1027-1035.

Question 2013

Topic: 10. Pathology and Oncology

Figures 44a and 44b show the radiographs of a 28-year-old woman who has had progressive hip pain for the past 3 months. What is the most likely diagnosis?

. Enchondroma
. Osteochondroma
. Chondrosarcoma
. Ollier's disease
. Maffucci's syndrome

Correct Answer & Explanation

. Chondrosarcoma


Explanation

The patient has multiple hereditary exostoses and a secondary chondrosarcoma arising from a proximal femoral exostosis. The radiograph of the knee shows multiple osteochondromas typical in a patient with multiple hereditary exostoses. Patients with this diagnosis are at an increased risk for malignant degeneration of an osteochondroma. The lateral radiograph of the hip shows a bony lesion emanating from the anterior aspect of the femoral neck that is not well defined in the surrounding soft tissues. There are punctate calcifications and a large soft-tissue mass. The most likely diagnosis is a secondary chondrosarcoma developing from a benign osteochondroma. An enchondroma is an intramedullary benign cartilage lesion. Ollier's disease and Maffucci's syndrome involve multiple enchondromas. Scarborough M, Moreau G: Benign cartilage tumors. Orthop Clin North Am 1996;27:583-589.

Question 2014

Topic: 10. Pathology and Oncology

A 20-year-old patient has foot pain. A radiograph and T1-weighted MRI scan are shown in Figures 8a and 8b. A biopsy specimen is shown in Figure 8c. Treatment should consist of

. extended curettage and cementation.
. amputation of the first ray.
. wide resection and chemotherapy.
. extended curettage, radiation therapy, and chemotherapy.
. Syme's amputation.

Correct Answer & Explanation

. extended curettage and cementation.


Explanation

Giant cell tumors occur near articular surfaces in young adults. The histology shows abundant giant cells with nuclei resembling the surrounding cells. Although the MRI scan shows soft-tissue involvement, curettage is still the preferred treatment. Chemotherapy is not necessary for benign lesions, and amputation is too aggressive. Cementation, phenol, and cryosurgery (liquid nitrogen) are all acceptable local adjuvants to curettage. Packing the cavity with bone graft rather than cement is also acceptable. Dahlin DC, Unni KK: Bone Tumors: General Aspects and Data on 8,542 Cases. Springfield, IL, Charles C. Thomas, 1986.

Question 2015

Topic: 10. Pathology and Oncology

A 10-year-old boy with a history of retinoblastoma now reports right knee pain. AP and lateral radiographs are shown in Figures 3a and 3b. What is the most likely diagnosis?

. Ewing's sarcoma
. Primitive neuroectodermal tumor
. Osteosarcoma
. Osteonecrosis
. Osteomyelitis

Correct Answer & Explanation

. Osteosarcoma


Explanation

The radiographs show a bone-producing lesion in the femoral diaphysis. The radiographic appearance of small round cell tumors is more permeative with an elevated periosteum and no matrix production. The appearance of this lesion is most consistent with osteosarcoma. Patients who carry the Rb gene are predisposed to osteosarcoma. However, Ewing's sarcoma, primitive neuroectodermal tumor, and osteomyelitis can all occur in this location. 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 2016

Topic: 10. Pathology and Oncology

A 13-year-old boy has pain and a firm mass in his left knee. A radiograph and MRI scan are shown in Figures 2a and 2b, and a biopsy specimen is shown in Figure 2c. Based on these findings, what is the most likely diagnosis?

. Osteosarcoma
. Osteochondroma
. Ewing's sarcoma
. Chondrosarcoma
. Periosteal chondroma

Correct Answer & Explanation

. Osteosarcoma


Explanation

The most likely diagnosis is osteosarcoma. The imaging studies show an aggressive primary tumor of bone, and the histology slide shows a typical chondroblastic osteosarcoma, with osteoid deposited along the surface of bone trabeculae. Ewing's sarcoma histologically consists of small round blue cells. Osteochondroma and periosteal chondroma can occur near the knee but have different radiographic and histologic patterns. Chondrosarcoma rarely occurs in children. Simon M, Springfield D, et al: Osteogenic sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 267.

Question 2017

Topic: 10. Pathology and Oncology

Figures 61a and 61b show the CT and MRI scans of a 40-year-old man who has hip pain. He undergoes total hip arthroplasty and curettage and cementation of the lesion as shown in Figure 61c. Histopathologic photomicrographs of the curettage specimen are shown in Figures 61d and 61e. What is the best course of treatment?

. Observation
. Revision and removal of any remaining gross disease
. Hemipelvectomy
. Exploration and repeat curettage
. Hip disarticulation

Correct Answer & Explanation

. Hemipelvectomy


Explanation

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.

Question 2018

Topic: 10. Pathology and Oncology

A 13-year-old girl has had increasing left hip pain for the past 4 months. A radiograph, bone scan, MRI scan, and photomicrograph are shown in Figures 1a through 1d. Which of the following immunohistochemistry results would confirm the most likely diagnosis?

. Cytokeratin positive
. PAS negative
. Reticulin positive
. MIC-2 positive
. Vimentin negative

Correct Answer & Explanation

. MIC-2 positive


Explanation

The imaging studies show a permeative lesion of the left hemipelvis with a large soft-tissue mass. The photomicrograph demonstrates a small blue cell tumor with pseudorosettes. The most likely diagnosis is primitive neuroectodermal tumor (Ewing's sarcoma family of tumors). MIC-2 is a highly sensitive and specific marker for this family of tumors. Cytokeratin is an epithelial marker. Vimentin is a mesenchymal marker. Thus, Ewing's sarcomas are cytokeratin negative and vimentin positive. Before discovery of the MIC-2 antigen, PAS and reticulin stains were commonly used to help differentiate Ewing's sarcoma from lymphoma. In contrast to lymphoma, Ewing's sarcomas are typically PAS positive and reticulin negative. Halliday BE, Slagel DD, Elsheikh TE, et al: Diagnostic utility of MIC-2 immunocytochemical staining in the differential diagnosis of small blue cell tumors. Diagn Cytopathol 1998;19:410-416.

Question 2019

Topic: 10. Pathology and Oncology

A 57-year-old man reports right hip pain that has been progressive for the past several months. The pain is exacerbated by weight-bearing activities and improves somewhat with rest. A radiograph is shown in Figure 10a and a coronal T1-weighted MRI scan is shown in Figure 10b. What is the most likely diagnosis?

. Osteoarthritis of the hip
. Osteonecrosis of the hip
. Metastatic carcinoma
. Femoral head fracture
. Rheumatoid arthritis of the hip

Correct Answer & Explanation

. Osteonecrosis of the hip


Explanation

These are classic findings of osteonecrosis of the hip. The radiograph reveals the subchondral sclerotic pattern commonly seen in osteonecrosis and is quite extensive in this patient. The MRI scan reveals the typical serpentine-like region of low signal intensity with a central zone where the signal is similar to fat. Resnick D (ed): Diagnosis of Bone and Joint Disorders. Philadelphia, PA, WB Saunders, 2002, pp 3160-3162.

Question 2020

Topic: 10. Pathology and Oncology

A healthy 16-year-old boy has had increasing pain in the right knee for the past 3 months. Examination reveals warmth and swelling around the distal femur. Radiographs and an MRI scan are shown in Figures 51a through 51c, and a biopsy specimen is shown in Figure 51d. What is the most likely diagnosis?

. Ewing's sarcoma
. Osteosarcoma
. Rhabdomyosarcoma
. Osteomyelitis
. Malignant fibrous histiocytoma

Correct Answer & Explanation

. Osteosarcoma


Explanation

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.