This practice set contains high-yield board review questions covering key concepts in 10. Pathology and Oncology. Each clinical scenario is designed to test your diagnostic and management skills relevant to this subspecialty.
Question 2101
Topic: 10. Pathology and Oncology
A 43-year-old woman has an enlarging mass in the left groin. A radiograph, CT scan, and a biopsy specimen are shown in Figures 42a through 42c. Treatment should consist of
Correct Answer & Explanation
. wide resection of the ischiopelvic region inferior to the acetabulum.
Explanation
The patient has a pelvic chondrosarcoma. The radiograph shows a lytic bone lesion emanating from the left inferior pubic ramus and extending into the soft tissues. Punctate calcifications are revealed on the radiograph and CT scan. The histology is consistent with a malignant cartilage lesion. Appropriate treatment for a pelvic chondrosarcoma is wide resection. In this location, wide resection of the ischiopelvic region (type 3 internal hemipelvectomy) is the treatment of choice. A type 2 internal hemipelvectomy involves resection of the periacetabular region. A type 1 internal hemipelvectomy involves resection of the ilium. No reconstruction is required for a type 3 resection. A classic hemipelvectomy is not necessary because the tumor can be removed with an adequate margin while maintaining the neurovascular structures and hip joint. Pring M, Weber KL, Unni K, Sim FH: Chondrosarcoma of the pelvis: A review of sixty-four cases. J Bone Joint Surg Am 2001;83:1630-1642. Sheth DS, Yasko AW, Johnson ME, Ayala AG, Murray JA, Romsdahl MM: Chondrosarcoma of the pelvis: Prognostic factors for 67 patients treated with definitive surgery. Cancer 1996;78:745-750.
Question 2102
Topic: 10. Pathology and Oncology
A 54-year-old man with metastatic renal cell carcinoma has had increasing pain in the left hip for the past 6 weeks. A radiograph is shown in Figure 36. Prophylactic stabilization will most likely result in
Correct Answer & Explanation
. faster postoperative recovery and decreased hospitalization time.
Explanation
Prophylactic stabilization of impending fractures does not directly affect the overall survival rate, but it does improve factors related to intraoperative and postoperative complications and decreased recovery time. Mirels H: Metastatic disease in long bones: A proposed scoring system for diagnosing impending pathologic fractures. Clin Orthop 1989;249:256-264.
Question 2103
Topic: 10. Pathology and Oncology
What is the most common primary malignant bone or cartilage tumor in children?
Correct Answer & Explanation
. Osteosarcoma
Explanation
Osteosarcoma is the most common primary malignant bone tumor (5.6 per 1 million children younger than age 15 years), and Ewing's sarcoma is second (2.1 per 1 million children). Giant cell tumor and chondrosarcoma are rare in children. Osteochondroma is more common than any of the above tumors in children, but it is not malignant. Himelstein BP, Dormans JP: Malignant bone tumors of childhood. Pediatr Clin North Am 1996;43:967-984. Pierz KA, Womer RB, Dormans JP: Pediatric bone tumors: Osteosarcoma, Ewing's sarcoma, and chondrosarcoma associated with multiple hereditary osteochondromatosis. J Pediatr Orthop 2001;21:412-418.
Question 2104
Topic: Bone Tumors
A 62-year-old woman reports diffuse aches and pains of the hip and pelvis. She denies any significant trauma but does have a history of chronic anemia. Figure 17a shows a radiograph of the pelvis, and Figures 17b and 17c show T2-weighted MRI scans. What is the most likely diagnosis?
Correct Answer & Explanation
. Multiple myeloma
Explanation
The radiograph reveals diffuse osteopenia and areas in the proximal femora that are moth-eaten in appearance. The extent of the marrow-replacing process is evident on the MRI scans, which reveal signal abnormality throughout the entire pelvis and both proximal femora. This represents a marrow-packing process, of which multiple myeloma is the best choice. This diagnosis is also supported by the anemia noted on the patient's history. Metastatic carcinoma and lymphoma also may have a similar presentation.
Question 2105
Topic: 10. Pathology and Oncology
A 52-year-old man has had back pain radiating to the left leg for the past 5 weeks. A radiograph, MRI scans, and biopsy specimens are shown in Figures 23a through 23f. What is the most likely diagnosis?
Correct Answer & Explanation
. Chordoma
Explanation
The histology shows cells with bubbly, abundant clear cytoplasm typical of physaliphorous cells; therefore, the most likely diagnosis is chordoma. These tumors arise from notocord rests in the upper and lower spine.
Question 2106
Topic: Bone Tumors
Figure 12 shows a lateral radiograph of the elbow. What is the most likely diagnosis?
Correct Answer & Explanation
. Normal anatomic variant
Explanation
The figure shows a supracondylar process, which is a normal anatomic variant. An osteochondroma tends to occur more toward the end of bones, and the medullary space of the underlying bone extends into the base of the osteochondroma. The presence of a supracondylar process is usually asymptomatic. However, the ligament of Struthers that always extends from the supracondylar process to the medial epicondyle can result in median nerve entrapment secondary to trauma. Last RJ: Anatomy: Regional and Applied, ed 6. London, England, Churchill Livingstone, 1978, pp 132-133.
Question 2107
Topic: Bone Tumors
A previously asymptomatic 12-year-old girl sustained a direct blow to the right lateral knee from a baseball bat. Examination reveals an area of ecchymosis and tenderness over the lateral thigh. The patient can walk without pain, but range of motion of the knee causes discomfort. Plain radiographs of the knee are shown in Figures 11a and 11b. To address the bone lesion, management should consist of
Correct Answer & Explanation
. a repeat examination in 6 weeks.
Explanation
The plain radiographs reveal a pedunculated osteochondroma with a fracture. There is a bony growth in the metaphysis of a long bone, on a stalk that is directed away from the nearby epiphysis. On the AP view, the host cortical and medullary bone are shown as "blending" with lesional bone. There is also a fracture through the lesion. Based on these radiographic findings, the diagnosis is an osteochondroma; therefore, initial management of an acute fracture of an osteochondroma is symptomatic treatment alone. Additional imaging studies are not indicated in this patient. At times it may be difficult to distinguish a sessile osteochondroma from a parosteal osteosarcoma. In the latter case, the host medullary bone and lesion bone are not confluent. A CT scan may be helpful to distinguish if the host medullary and cortical bone are confluent with the lesion.
Question 2108
Topic: 10. Pathology and Oncology
Ewing's sarcoma of bone most commonly occurs in which of the following locations?
Correct Answer & Explanation
. Major long tubular bones
Explanation
Ewing's sarcoma typically occurs in the major long tubular bones, with the femur the most common location. The flat bones of the pelvis are the second most common location. Ewing's sarcoma occurs in the fibula but with a lower incidence than that seen in the major tubular bones. Ewing's sarcoma infrequently occurs in the metacarpals or the vertebral bodies. Simon M, Springfield D, et al: Ewing's Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 287.
Question 2109
Topic: 10. Pathology and Oncology
A 14-year-old girl reports bilateral patellofemoral symptoms. Based on the radiograph and MRI scans shown in Figures 23a through 23d, what is the next most appropriate step in management of the lesion?
Correct Answer & Explanation
. Observation
Explanation
A periosteal desmoid lesion is a tumor simulator. It is characterized by a bone irregularity along the posteromedial aspect of the distal femur at the insertion of the adductor magnus or the origin of the gastrocnemius muscle. It most commonly occurs in patients who are age 10 to 15 years. The lesions are asymptomatic, with no palpable mass, pain, or swelling. They are frequently an incidental finding when radiographs are obtained for nonspecific symptoms or trauma about the knee. Following recognition of the characteristic imaging findings, observation is the management of choice. Dunham WK, Marcus NW, Enneking WF, et al: Developmental defects of the distal femoral metaphysis. J Bone Joint Surg Am 1980;62:801-806.
Question 2110
Topic: Bone Tumors
Figure 40 shows the radiograph of a 30-year-old woman who has a painful elbow. Examination reveals a deformed skull, multiple cafe-au-lait spots, and bone deformities. What is the most likely diagnosis?
Correct Answer & Explanation
. McCune-Albright syndrome
Explanation
Findings in patients with McCune-Albright syndrome include polyostotic fibrous dysplasia, multiple cafe-au-lait spots, and precocious puberty. The bone changes in NF-1 resemble nonossifying fibromas, not fibrous dysplasia. NF-2 has little bony change with typical ocular abnormalities. Paget's disease occurs in older individuals and does not present with cafe-au-lait spots. Ollier's disease (multiple enchondromatosis) may show bone changes but not the other findings. Albright F, Butler AM, Hampton AO, et al: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females. N Engl J Med 1937;216:727-746. Danon M, Robboy SJ, Kim S, Scully R, Crawford JD: Cushing syndrome, sexual precocity, and polyostotic fibrous dysplasia (Albright syndrome) in infancy. J Pediatr 1975;87:917-921.
Question 2111
Topic: 10. Pathology and Oncology
A 13-year-old girl has had a firm mass and pain in her right shoulder for the past several weeks. She denies any history of trauma. A radiograph and MRI scan are shown in Figures 31a and 31b. Biopsy specimens are shown in Figures 31c and 31d. What is the most likely diagnosis?
Correct Answer & Explanation
. Osteosarcoma
Explanation
The patient has osteosarcoma. The radiograph suggests an aggressive primary tumor of bone, and the histology shows malignant cells surrounded by osteoid, classic for osteosarcoma. Ewing's sarcoma histologically consists of small round blue cells. Osteochondroma and periosteal chondroma occur in the shoulder but have a different histologic pattern and a less aggressive radiographic appearance. Chondrosarcomas rarely occur in children. Simon MA, Springfield DS, et al: Osteogenic Sarcoma: Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA, Lippincott Raven, 1998, p 266.
Question 2112
Topic: 10. Pathology and Oncology
A 10-year-old girl reports activity-related bilateral arm pain. Examination reveals no soft-tissue masses in either arm, and she has full painless range of motion in both shoulders and elbows. The radiograph and bone scan are shown in Figures 20a and 20b, and biopsy specimens are shown in Figures 20c and 20d. What is the most likely diagnosis?
Correct Answer & Explanation
. Fibrous dysplasia
Explanation
Based on these findings, the most likely diagnosis is fibrous dysplasia. Twenty percent of patients with fibrous dysplasia have multifocal disease. The lesions show a typical ground glass appearance. Fibrous dysplasia frequently involves the diaphysis of the long bones. There is no associated soft-tissue mass and no periosteal reactions to these lesions, suggesting a benign lesion. The histology shows proliferating fibroblasts in a dense collagen matrix. Trabeculae are arranged in an irregular or "Chinese letter" appearance. Osteogenic sarcoma and Ewing's sarcoma have a much different radiographic appearance of malignant osteoid and small round blue cells. Periosteal chondroma does occur in the proximal humerus but is not typically multifocal. It appears as a surface lesion with saucerization of the underlying bone and a bony buttress adjacent to the lesion. Some patients with multifocal lesions have associated endocrine abnormalities (McCune-Albright syndrome). Wold LA, et al: Atlas of Orthopaedic Pathology. Philadelphia, PA, WB Saunders, 1990, pp 118-119.
Question 2113
Topic: 10. Pathology and Oncology
A 52-year-old man has a stage IIB malignant fibrous histiocytoma of the distal femur. Local treatment should consist of
Correct Answer & Explanation
. wide excision.
Explanation
Local control of malignant fibrous histiocytoma of bone typically consists of wide excision. Curettage and bone grafting is a procedure with an intralesional surgical margin, with an unacceptable rate of local recurrence. Prophylactic fixation is considered for patients with metastatic disease. Radiation therapy alone is not adequate for local control of this tumor. Neoadjuvant chemotherapy is often used primarily for systemic tumor control.
Question 2114
Topic: 10. Pathology and Oncology
A 6-year-old boy has leg pain. A radiograph, MRI, CT, and bone scans, and a biopsy specimen are shown in Figures 14a through 14e. What is the most likely diagnosis?
Correct Answer & Explanation
. Osteomyelitis
Explanation
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.
Question 2115
Topic: 10. Pathology and Oncology
A patient with a 5-cm synovial sarcoma located in the distal portion of the rectus femoris muscle undergoes excision of the mass. The procedure is performed through a 10-cm longitudinal incision. Only a portion of the rectus femoris is removed; the vast majority of the muscle is preserved. The plane of dissection is beyond the reactive zone, and the pathology reveals that the margins are negative. This procedure is classified as
Correct Answer & Explanation
. wide.
Explanation
The patient underwent a wide resection, which involves excision of the tumor along with a cuff of normal tissue that completely surrounds the tumor. The plane of resection is beyond the reactive zone. A radical resection involves removal of the entire affected muscle from origin to insertion. In a marginal excision, the plane of dissection is through the reactive zone of the tumor. A marginal excision is generally considered inadequate surgery for high-grade sarcomas. In an intralesional resection, the plane of dissection is through the tumor. Excision within the reactive zone but beyond the tumor is the same as a marginal excision. Enneking WF: Staging of musculoskeletal neoplasms, in Current Concepts of Diagnosis and Treatment of Bone and Soft Tissue Tumors. Heidelberg, Germany, Springer-Verlag, 1984.
Question 2116
Topic: 10. Pathology and Oncology
A 12-year-old girl has had pain in her right knee for 1 month that started as activity-related and progressed to night pain. Radiographs are shown in Figures 16a and 16b, and a biopsy specimen is shown in Figure 16c. What is the recommended treatment?
Correct Answer & Explanation
. Preoperative chemotherapy followed by surgical resection of the lesion and postoperative chemotherapy
Explanation
This is a classic appearance for an osteosarcoma. The radiographs reveal a mixed osteolytic and osteoblastic lesion in a skeletally immature patient in the distal right femoral metaphysis. The pain pattern with progressive symptoms leading to the presence of night pain is also typical for this condition. The biopsy specimen reveals pleomorphic cells and the presence of osteoid. The current standard of care in the treatment of osteosarcoma is neoadjuvant chemotherapy followed by surgical resection or amputation followed by additional postoperative chemotherapy. Osteosarcoma is not radiosensitive. Wold LE, Adler CP, Sim FH, et al: Atlas of Orthopedic Pathology, ed 2. Philadelphia, PA, WB Saunders, 2003, p 179.
Question 2117
Topic: 10. Pathology and Oncology
A 68-year-old woman with metastatic breast carcinoma is seen in the emergency department. She appears lethargic, and she reports abdominal pain, nausea, and constipation. An EKG reveals a shortened QT interval. The only physical finding on examination is diffuse hyporeflexia. What is the most appropriate step in management?
Correct Answer & Explanation
. Intravenous fluid administration
Explanation
Intravenous fluid administration is the best first step to treat the hypercalcemia of malignancy. Many of these patients are dehydrated, and the increased serum calcium impairs the ability of the kidney to concentrate the urine. The decreased glomerular filtration rate secondary to the hypovolemia also leads to increased tubular resorption of calcium. The establishment of normovolemia will help promote increased urinary excretion of calcium. Lasix can also be used to help promote calciuria. Mithramycin is an antibiotic derived from Streptomyces plicatus. It is part of a group of drugs referred to as chromomycin antibiotics and is the only one of this group used clinically in the United States. It is rarely used in cancer chemotherapy because of its toxicity. A number of drug-related deaths have occurred from the use of mithramycin. Its use is now limited to the treatment of hypercalcemia associated with malignancy where it is used in lower dosage than that used for the treatment of tumors. Methotrexate has no role in the treatment of hypercalcemia of malignancy. While intravenous bisphosphonates are helpful in slowing progression of metastases and may help lower cerum calcium, they are not appropriate in the emergent treatment of hypercalcemia in the metastatic cancer patient.
Question 2118
Topic: Bone Tumors
A 10-year-old child has leg discomfort with activity. A radiograph, bone scan, and biopsy specimen are shown in Figures 1a through 1c. What is the most likely diagnosis?
Correct Answer & Explanation
. Fibrous dysplasia
Explanation
The ground glass appearance on the radiograph, the hot bone scan, and histologic findings of bony spicules without osteoblastic rimming in a background of bland fibrous tissue all suggest fibrous dysplasia. Stress-related pain is common with activity because of the dysplastic bone. Parosteal osteosarcomas are surface lesions. Simple cysts, aneurysmal bone cysts, and eosinophilic granuloma are all possible radiographically; however, the histology is most consistent with fibrous dysplasia. Harris WH, Dudley HR Jr, Barry RS: The natural history of fibrous dysplasia: An orthopaedic, pathological and roentgenographic study. J Bone Joint Surg Am 1962;44:207.
Question 2119
Topic: 10. Pathology and Oncology
A 10-year-boy has had thigh pain for the past several months. He denies any history of trauma. Examination reveals no soft-tissue mass, and mild tenderness. Figures 33a and 33b show the plain radiograph and MRI scan, and the biopsy specimens are shown in Figures 33c and 33d. What is the most likely diagnosis?
Correct Answer & Explanation
. Eosinophilic granuloma
Explanation
The diagnosis is eosinophilic granuloma. The plain radiograph and MRI scan show a lesion in the midshaft of the femur. There is no soft-tissue mass. There is reactive bone about the lesion that suggests a less aggressive tumor. The histology reveals eosinophils in an otherwise bland cellular background with no evidence of mitotic figures or malignant cells to suggest sarcoma. The diagnostic elements are the amphophilic (ie, pale purple) histiocytes with cigar-shaped nuclei, some of which have linear longitudinal grooves. There is no histologic evidence of infection. Lymphoma of bone would be an unusual occurrence in this age group, and the histology is not consistent with that diagnosis.
Question 2120
Topic: Bone Tumors
A 16-year-old girl has had anterior leg pain and a mass for the past 8 months. Figures 2a and 2b show a radiograph and an H&E histologic specimen. Which of the following disorders is believed to be a precursor of this lesion?
Correct Answer & Explanation
. Osteofibrous dysplasia
Explanation
The radiograph and pathology are consistent with adamantinoma. While the mechanism underlying adamantinoma has not been identified, it is believed to be closely related to osteofibrous dysplasia, which may represent a precursor. The other diagnoses are not known to give rise to adamantinoma.
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