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Question 2181

Topic: 10. Pathology and Oncology

A 31-year-old woman has increasing pain and tightness in her right knee, with occasional stiffness and recurrent hemorrhagic effusions. MRI scans are shown in Figures 2a and 2b. What is the most likely diagnosis?

. Rheumatoid arthritis
. Pigmented villonodular synovitis (PVNS)
. Synovial sarcoma
. Synovial chondromatosis
. Fibromatosis

Correct Answer & Explanation

. Pigmented villonodular synovitis (PVNS)


Explanation

PVNS is a rare inflammatory granulomatous condition of unknown etiology, and causes proliferation of the synovium of joints, tendon sheaths, or bursa. The disorder occurs most commonly in the third and fourth decades but can occur at any age. MRI provides excellent delineation of the synovial disease. Characteristic features of PVNS on MRI include the presence of intra-articular nodular masses of low signal intensity on T1- and T2-weighted images and proton density-weighted images. Synovial biopsy should be performed if there is any doubt of the diagnosis. Total synovectomy (open or arthroscopic) is required for the diffuse form, although recurrence is common. Rheumatoid arthritis and synovial chondromatosis are not typically associated with hemorrhagic effusions. De Ponti A, Sansone V, Malchere M: Result of arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthroscopy 2003;19:602-607. Chin KR, Barr SJ, Winalski C, et al: Treatment of advanced primary and recurrent diffuse pigmented villonodular synovitis of the knee. J Bone Joint Surg Am 2002;84:2192-2202.

Question 2182

Topic: Bone Tumors

A 19-year-old woman reports persistent neck pain for 2 years. Pain is relieved with aspirin. A bone scan shows intense uptake in the superior, posterior portion of the C3 vertebral body. A sagittal CT reconstruction is shown in Figure 5. Treatment should consist of

Spine Surgery 2009 Practice Questions: Set 1 (Solved) - Figure 18

. radiation therapy.
. en bloc excision.
. posterior fusion at C2-C3 with instrumentation.
. CT-guided aspiration followed by IV antibiotics.
. radiofrequency ablation.

Correct Answer & Explanation

. en bloc excision.


Explanation

The CT scan shows an osteoblastic nidus pathognomic for an osteoid osteoma. Surgical treatment should include an en bloc excision of the lesion. Surgical treatment is not mandatory because the lesion often becomes asymptomatic over time. This lesion is not amenable to radiofrequency ablation due to its proximity to the spinal cord. A complete corpectomy is not necessary to adequately resect the lesion, as only the nidus needs to be removed. Radiation therapy and antibiotics are not appropriate treatments for an osteoid osteoma. Posterior C2-C3 fusion will not address the pathology. Spivak JM, Connolly PJ (eds): Orthopaedic Knowledge Update: Spine 3. Rosemont, IL, American Academy of Orthopaedic Surgeons, 2006, pp 351-366.

Question 2183

Topic: 10. Pathology and Oncology

A 30-year-old woman has had pain in her right leg for the past 6 months. A lytic lesion is noted in the anterior cortex of the midtibia, extending 5 cm in length without a soft-tissue mass. A radiograph and a biopsy specimen are shown in Figures 35a and 35b. What is the preferred treatment?

. Debridement and IV antibiotics
. Wide resection of the lesion
. Chemotherapy alone
. Observation
. Amputation

Correct Answer & Explanation

. Wide resection of the lesion


Explanation

In an adult with an anterior cortical tibial lesion, this is the classic histologic appearance and anatomic location for an adamantinoma. The histology reveals areas of epithelial cells (in a glandular pattern) within a fibrous stroma. The epithelial cells are shown in nests. They would stain positively for keratin. Adamantinoma is a rare malignant bone tumor with a propensity for late metastasis. It has a high incidence of local recurrence unless resected with a wide margin. Chemotherapy and radiation therapy are not helpful in the treatment of this disease. Amputation generally is not necessary because a diaphyseal resection is usually possible. McCarthy EF, Frassica FJ: Pathology of Bone and Joint Disorders with Clinical and Radiographic Correlation. Philadelphia, PA, WB Saunders, 1998, p 263.

Question 2184

Topic: 10. Pathology and Oncology

A 63-year-woman has an elbow flexion contracture. History reveals that she underwent three previous surgeries to remove a malignant fibrous histiocytoma of the forearm. An MRI scan reveals a locally recurrent tumor at the site of the previous surgery. Which of the following is considered the most predictive factor for local recurrence?

Basic Science Board Review 2002: High-Yield MCQs (Set 2) - Figure 41

. Tumor grade
. Tumor size
. Surgical margin
. Anatomic site of the tumor
. Histologic subtype of the tumor

Correct Answer & Explanation

. Surgical margin


Explanation

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.

Question 2185

Topic: Bone Tumors

What additional percentage of energy expenditure above baseline is required for ambulation after an above-the-knee amputation?

. 0%
. 5%
. 20%
. 65%
. 90%

Correct Answer & Explanation

. 65%


Explanation

Patients with an above-the-knee amputation have a 65% increase in energy expenditure. A patient with a transtibial amputation requires 25% more energy above baseline values; however, bilateral transtibial amputations are associated with a 40% increase in energy expenditure. Otis JC, Lane JM, Kroll MA: Energy cost during gait in osteosarcoma patients after resection and knee replacement and after above-the-knee amputation. J Bone Joint Surg Am 1985;67:606-611.

Question 2186

Topic: 10. Pathology and Oncology

Which of the following tumors have characteristic chromosomal translocations?

Basic Science 2005 Practice Questions: Set 1 (Solved) - Figure 9

. Ewing's sarcoma and osteosarcoma
. Ewing's sarcoma and conventional chondrosarcoma
. Ewing's sarcoma and synovial sarcoma
. Osteosarcoma and conventional chondrosarcoma
. Myeloma and malignant fibrous histiocytoma

Correct Answer & Explanation

. Ewing's sarcoma and osteosarcoma


Explanation

Ewing's sarcoma has an 11;22 translocation that creates the EWS/FLI1 fusion gene, and synovial sarcoma has an X;18 translocation that creates the STT/SSX fusion gene. The other tumors do not have consistent translocations. Sandberg AA: Cytogenetics and molecular genetics of bone and soft-tissue tumors. Am J Med Genet 2002;115:189-193.

Question 2187

Topic: 10. Pathology and Oncology

An axial T1-weighted MRI scan of the pelvis is shown in Figure 13. The arrow is pointing to what muscle?

Anatomy 2005 Practice Questions: Set 1 (Solved) - Figure 34

. Adductor magnus
. Pectineus
. Obturator externus
. Obturator internus
. Levator ani

Correct Answer & Explanation

. Obturator internus


Explanation

The obturator internus muscle originates from the internal pelvic wall and passes laterally through the lesser sciatic foramen, banking around the ischium below the sacrospinous ligament before inserting on the medial aspect of the greater trochanter. Higuchi T: Normal anatomy and magnetic resonance appearance of the pelvis, in Takahashi HE, Morita T, Hotta T, Ogose A (eds): Operative Treatment of Pelvic Tumors. Tokyo, Japan, Springer-Verlag, 2003, pp 4-21.

Question 2188

Topic: 10. Pathology and Oncology

A 35-year-old patient has right hip pain. Figures 34a and 34b show the coronal MRI scan and the biopsy specimen. What is the most likely diagnosis?

. Degenerative cyst with collapse
. Osteonecrosis
. Chondroblastoma
. Metastatic carcinoma
. Clear cell chondrosarcoma

Correct Answer & Explanation

. Osteonecrosis


Explanation

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.

Question 2189

Topic: 10. Pathology and Oncology

A 14-year-old girl has had mild pain and nail deformity of the great toe for the past 4 months. A radiograph is shown in Figure 50. What is the most likely etiology of the lesion?

Foot & Ankle Board Review 2000: High-Yield MCQs (Set 4) - Figure 21

. Fungal infection
. Ingrowth of the medial nail
. Benign exostosis
. Malignant neoplasm
. Reactive pyogenic granuloma

Correct Answer & Explanation

. Benign exostosis


Explanation

The lesion is typical of a subungual exostosis, which is most often found on the medial aspect of the great toe in children and young adults. The diagnosis is confirmed on radiographs and usually requires excision for relief. Lokiec F, Ezra E, Krasin E, Keret D, Wientraub S: A simple and efficient surgical technique for subungual exostosis. J Pediatr Orthop 2001;21:76-79. Letts M, Davidson D, Nizalik E: Subungual exostosis: Diagnosis and treatment in children. J Trauma 1998;44:346-349.

Question 2190

Topic: 10. Pathology and Oncology

A 15-year-old boy has had pain in the right shoulder for the past 3 months. He denies any history of trauma and has no constitutional symptoms. Examination reveals a large firm mass in the proximal arm. A radiograph and MRI scan are shown in Figures 27a and 27b. Biopsy specimens are shown in Figures 27c and 27d. Management should consist of

. observation.
. steroid injection.
. curettage and bone grafting.
. wide resection with neoadjuvant chemotherapy.
. debridement, irrigation, and intravenous antibiotics.

Correct Answer & Explanation

. curettage and bone grafting.


Explanation

The patient has an aneurysmal bone cyst. The fluid-fluid levels seen on the MRI scan are typical for aneurysmal bone cyst, and the histology is consistent with a cystic lining. Vascular lakes, multinucleated giant cells, reactive bone, fibrovascular tissue, and an absence of atypical cells or numerous mitoses are seen histologically. Aneurysmal bone cysts will typically continue to grow and cause further bone destruction; therefore, observation is not recommended. Steroid injections are not effective. A thorough curettage of the cyst lining and bone grafting are required. Wide resection and chemotherapy are reserved for more aggressive tumors. There is no evidence of infection radiographically or histologically. Telangiectatic osteosarcoma should also be considered in the differential diagnosis; therefore, biopsy is an important part of the work-up. Wold LA, et al: Atlas of Orthopaedic Pathology. Philadelphia, PA, WB Saunders, 1990, pp 232-233.

Question 2191

Topic: 10. Pathology and Oncology

A 58-year-old woman has had a slowly progressing mass over the distal interphalangeal (DIP) joint of her dominant hand with a worsening deformity of her nail. She has no significant medical history but underwent bilateral knee arthroplasties 1 year ago. Radiographs reveal a small osteophyte at the DIP joint dorsally. A clinical photograph and a biopsy specimen are shown in Figures 76a and 76b. What is the most likely diagnosis?

. Metastatic lung carcinoma
. Mucous cyst
. Synovial sarcoma
. Inclusion cyst
. Felon abscess

Correct Answer & Explanation

. Mucous cyst


Explanation

A mucous cyst is thought to be a ganglion arising from the DIP joint in patients with osteoarthritis. They are frequently associated with nail deformities. Treatment involves removal of the cyst with debridement of DIP joint osteophytes. Fritz GR, Stern PJ, Dickey M: Complications following mucous cyst excision. J Hand Surg Br 1997;22:222-225.

Question 2192

Topic: 10. Pathology and Oncology

A 12-year-old girl has painless bowing of the tibia. Radiographs and a biopsy specimen are shown in Figures 35a through 35c. What is the most likely diagnosis?

. Osteofibrous dysplasia
. Adamantinoma
. Osteosarcoma
. Ewing's sarcoma
. Fibrous dysplasia

Correct Answer & Explanation

. Osteofibrous dysplasia


Explanation

The patient has osteofibrous dysplasia. The radiographic differential diagnosis includes osteofibrous dysplasia, fibrous dysplasia, and adamantinoma. Histology shows a fibro-osseous lesion with prominent osteoblastic rimming but a lack of epithelial nests. Adamantinoma is a low-grade malignancy that typically is located in the anterior tibial cortex and has a soap bubble appearance. Histologically, it is similar to osteofibrous dysplasia but includes epithelial nests of cells. Treatment requires resection. Fibrous dysplasia usually does not require biopsy; however, in this patient the radiographs do not distinguish it from adamantinoma. The radiographic findings are not typical of Ewing's sarcoma or osteosarcoma. Repeat biopsy should be considered if clinical or radiographic features change.

Question 2193

Topic: 10. Pathology and Oncology

What is the second most common primary bone malignancy in children?

Basic Science 2008 Practice Questions: Set 3 (Solved) - Figure 25

. Rhabdomyosarcoma
. Osteosarcoma
. Ewing's sarcoma
. Fibrosarcoma
. Adamantinoma

Correct Answer & Explanation

. Ewing's sarcoma


Explanation

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.

Question 2194

Topic: 10. Pathology and Oncology

A 60-year-old man has pain at the tip of the index finger. A radiograph and biopsy specimen are shown in Figures 40a and 40b. Management should consist of

. surgical debridement and antibiotics.
. curettage and bone graft.
. amputation through the distal interphalangeal joint.
. amputation of the second ray.
. radiation therapy.

Correct Answer & Explanation

. amputation through the distal interphalangeal joint.


Explanation

The radiograph and histology findings are most consistent with squamous cell carcinoma. This tumor is best treated with wide surgical resection margins alone in the absence of metastasis; in this patient, management should consist of amputation through the distal interphalangeal joint. The other treatments are not indicated. Soltani K, Krunic A: Non melanoma skin neoplasms, in Vokes E, Golomb H (eds): Oncologic Therapies, ed 2. Berlin, Germany, Springer, pp 646-647.

Question 2195

Topic: 10. Pathology and Oncology
An otherwise healthy 65-year-old man reports thigh pain of insidious onset. He states that the pain is increased with weight bearing and also occurs at night. He denies any history of cancer. Radiographs are shown in Figures 22a and 22b. A bone scan shows an isolated lesion. CT scans of the chest and abdomen are negative for any other lesions. Initial management should consist of
. stabilization with a locked intramedullary rod.
. stabilization with a compression plate.
. chemotherapy.
. radiation therapy.
. performing a biopsy.

Correct Answer & Explanation

. performing a biopsy.


Explanation

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.

Question 2196

Topic: 10. Pathology and Oncology

A 19-year-old man has had pain and swelling in his left forearm for the past 8 months. Laboratory studies show a mildly elevated WBC count and erythrocyte sedimentation rate. Radiographs are shown in Figures 58a and 58b, a CT scan is shown in Figure 58c, and T1- and T2-weighted MRI scans are shown in Figures 58d and 58e, respectively. A biopsy specimen is shown in Figure 58f. Immunohistochemistry demonstrates that the lesion is negative for leukocyte common antigen (CD34). What is the most common cytogenetic translocation associated with this lesion?

. (11; 22) (q24; q12)
. (X; 18) (p11; q11)
. (12; 22) (q13; q12)
. (2; 13) (q35; q14)
. (12; 16) (q13; p11)

Correct Answer & Explanation

. (11; 22) (q24; q12)


Explanation

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.

Question 2197

Topic: 10. Pathology and Oncology

What common cytologic abnormality is associated with Ewing's sarcoma?

. t (2, 13)
. t (x, 18)
. t (11, 22)
. t (12, 16)
. t (12, 22)

Correct Answer & Explanation

. t (11, 22)


Explanation

Cytogenetic abnormalities have been well characterized in a number of tumors. Translocation t (2, 13), (x, 18), (12, 16), and (12, 22) have been characterized in rhabdomyosarcoma, synovial cell sarcoma, myxoid liposarcoma, and clear cell sarcoma, respectively. Translocation t(11:22) can be identified in 95% of patients with Ewing's sarcoma. This was first described by Turc-Carel and associates in 1984. Enzinger FM, Weiss SW: Soft Tissue Tumors, ed 3. St Louis, MO, Mosby Year Book, 1995, pp 105-118. Turc-Carel C, Philip I, Berger MP, Philip T, Lenoir GM: Chromosome study of Ewing's sarcoma (ES) cell lines: Consistency of a reciprocal translocation t(11;22) (q24;q12). Cancer Genet Cytogenet 1984;12:1-19.

Question 2198

Topic: 10. Pathology and Oncology

Figure 41a shows the AP radiograph of a 15-year-old boy who reports lateral knee pain. Figures 41b and 41c show a radiograph of the distal femur that was obtained 5 years ago and a current CT scan. The indication for surgery in this patient would be

. activity limitations because of localized pain.
. the likelihood of development of malignant degeneration in adulthood.
. the likelihood of development of a growth deformity.
. ambiguity regarding the diagnosis based on the imaging studies.
. obvious progression over the past 5 years, raising suspicion of malignant degeneration.

Correct Answer & Explanation

. activity limitations because of localized pain.


Explanation

In a young person with solitary osteochondroma, the best surgical indication is symptoms that limit activity. A growth deformity is unlikely to occur at this age. Malignant degeneration is exceptionally rare and noted most commonly in adults. Growth is expected until skeletal maturity. Mirra JM: Bone Tumors: Clinical, Radiologic, and Pathologic Correlations. Philadelphia, PA, Lea and Febiger, 1989, pp 1626-1659.

Question 2199

Topic: 10. Pathology and Oncology

A 55-year-old woman has slowly increasing pain at the distal end of her little finger that is exacerbated by cold temperatures. She denies any history of trauma to her hands and is employed as a school teacher. The histology of the resected specimen is shown in Figure 71. What is the most likely diagnosis?

Basic Science Board Review 2008: High-Yield MCQs (Set 4) - Figure 31

. Inclusion cyst
. Schwannoma
. Scleroderma
. Paranychia
. Glomus tumor

Correct Answer & Explanation

. Glomus tumor


Explanation

Glomus tumors are rare vascular lesions typically occurring about the nail of the distal phalanx of the hand. The diagnostic "triad" of glomus tumors consists of local pain, sensitivity to cold, and paroxysmal pain. They tend to present with pain as the most typical symptom and this can be exacerbated by changes in temperature that is felt to cause a vascular response within the lesion. The biopsy specimen confirms a glomus tumor showing the typical vascular spaces surrounded by glomus epithelioid glomus cells. Zook EG, Brown RE: The perionychium, in Green DP, Hotchkiss RN, Pederson WC (eds): Green's Operative Hand Surgery, ed 4. Philadelphia, PA, Churchill Livingstone, 1999, vol 2, pp 1353-1380.

Question 2200

Topic: 10. Pathology and Oncology

Radiographs of a 15-year-old girl with knee pain reveal a radiopaque lesion of the distal femoral metaphysis and epiphysis with a small associated soft-tissue mass. A biopsy specimen shows osteoid and pleomorphic cells with multiple mitotic figures. Staging studies show no other sites of disease. Treatment should consist of

Basic Science Board Review 2002: High-Yield MCQs (Set 2) - Figure 21

. above-knee amputation.
. preoperative chemotherapy, wide resection of the distal femur, and reconstruction.
. preoperative chemotherapy and above-knee amputation.
. wide resection of the distal femur and subsequent reconstruction, followed by chemotherapy.
. curettage and placement of methylmethacrylate.

Correct Answer & Explanation

. preoperative chemotherapy, wide resection of the distal femur, and reconstruction.


Explanation

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.