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 6841
Topic: 10. Pathology and Oncology
A 45-year-old woman presents with weakness in wrist extension and finger extension. MRI demonstrates a large mass composed uniformly of fat sitting intimately on the periosteum of the proximal radius. What is the most likely diagnosis?
Correct Answer & Explanation
. Parosteal lipoma
Explanation
Parosteal lipomas are rare, benign adipose tumors intimately associated with the periosteum, often presenting in the proximal radius. They frequently cause compressive neuropathy of the posterior interosseous nerve (PIN), leading to extensor weakness.
Question 6842
Topic: 10. Pathology and Oncology
A 30-year-old male with a history of primary synovial chondromatosis of the hip undergoes arthroscopic synovectomy. Ten years later, he presents with sudden, rapidly progressive hip pain and an enlarging mass. Radiographs show aggressive bone destruction. What is the most likely diagnosis?
Correct Answer & Explanation
. Secondary chondrosarcoma
Explanation
Primary synovial chondromatosis has a known, albeit rare (1-5%), risk of malignant transformation into secondary chondrosarcoma. Rapid clinical deterioration, new mass formation, and aggressive bony destruction or marrow invasion on MRI are hallmarks of this transformation.
Question 6843
Topic: Bone Tumors
An osteochondroma is generally considered to stop growing at skeletal maturity. If an osteochondroma-like lesion originates within the epiphysis rather than the metaphysis, leading to joint deformity and restricted motion, what is this condition termed?
Trevor disease, or dysplasia epiphysealis hemimelica, is characterized by an osteochondroma-like growth arising from the epiphysis, typically affecting one side (hemimelic) of a joint in the lower extremity. It leads to joint asymmetry, restricted motion, and premature osteoarthritis.
Question 6844
Topic: 10. Pathology and Oncology
A painless soft tissue mass in the posterior neck of a 55-year-old man is biopsied. Histology reveals mature adipocytes, uniform spindle cells, and thick, rope-like collagen bundles without lipoblasts. Immunohistochemistry is strongly positive for CD34. What is the diagnosis?
Correct Answer & Explanation
. Spindle cell lipoma
Explanation
Spindle cell lipomas classically occur in the posterior neck, shoulder, or upper back of older men. They are characterized by mature fat, CD34-positive spindle cells, and ropey collagen, and they are entirely benign.
Question 6845
Topic: 10. Pathology and Oncology
A 35-year-old male presents with a slowly enlarging, painless mass over the distal femur. Radiographs reveal a sessile bony exostosis pointing away from the joint. An MRI is obtained to evaluate the cartilage cap. Which of the following cartilage cap thicknesses is most indicative of malignant transformation to secondary chondrosarcoma in an adult?
Correct Answer & Explanation
. >20 mm
Explanation
In adults, a cartilage cap thickness greater than 1.5 to 2 cm (>15-20 mm) on MRI is highly suspicious for malignant transformation of an osteochondroma into a secondary chondrosarcoma. In skeletally immature patients, thicker caps are normal and expected during active growth.
Question 6846
Topic: 10. Pathology and Oncology
A 40-year-old female presents with progressive knee pain and mechanical catching. Imaging reveals multiple intra-articular calcified loose bodies with relative preservation of the joint space. A diagnosis of primary synovial chondromatosis is suspected. What is the underlying pathogenesis of this condition?
Correct Answer & Explanation
. Benign neoplastic proliferation of synovium
Explanation
Historically considered a reactive metaplastic process, primary synovial chondromatosis is now recognized as a benign neoplastic proliferation of the synovium. It is often associated with specific clonal chromosomal aberrations, notably FN1-ACVR2A fusions.
Question 6847
Topic: 10. Pathology and Oncology
A 60-year-old male presents with a large, deep-seated fatty mass in the thigh. Biopsy shows mature adipose tissue with focal atypia and hyperchromatic stromal cells. Cytogenetic analysis is ordered to differentiate an Atypical Lipomatous Tumor (ALT) from a benign lipoma. Which of the following genetic markers is characteristic of an ALT?
Correct Answer & Explanation
. MDM2 amplification
Explanation
Atypical Lipomatous Tumors (ALT), also known as well-differentiated liposarcomas, are characterized by supernumerary ring chromosomes leading to the amplification of the MDM2 and CDK4 genes on chromosome 12q. This molecular signature reliably differentiates ALT from benign lipomas.
Question 6848
Topic: 10. Pathology and Oncology
A 10-year-old boy presents with multiple bony bumps around his knees, ankles, and shoulders. He is short in stature and exhibits a mild forearm deformity. A diagnosis of Multiple Hereditary Exostoses (MHE) is made. What is the inheritance pattern and the most common genetic mutation associated with this condition?
Correct Answer & Explanation
. Autosomal dominant, EXT1 and EXT2
Explanation
Multiple Hereditary Exostoses (MHE) is an autosomal dominant condition characterized by mutations in the EXT1 or EXT2 genes. These genes are tumor suppressors involved in heparan sulfate synthesis, and their mutation leads to the formation of multiple osteochondromas.
Question 6849
Topic: 10. Pathology and Oncology
A 60-year-old male presents with a deep intramuscular mass in the thigh. MRI reveals a large lipomatous lesion with thick septations.
Core needle biopsy shows adipocytes with variation in cell size and hyperchromatic stromal cells. Which of the following cytogenetic abnormalities is diagnostic for this tumor?
Correct Answer & Explanation
. Ring chromosome 12 with MDM2 amplification
Explanation
Atypical lipomatous tumors (well-differentiated liposarcomas) are characterized by ring or giant rod chromosomes containing amplified sequences of the 12q13-15 region. This results in the specific amplification of the MDM2 and CDK4 genes.
Question 6850
Topic: 10. Pathology and Oncology
A 45-year-old male complains of chronic knee pain, swelling, and catching. Radiographs and MRI are shown.
What is the primary pathophysiological mechanism underlying this condition?
Correct Answer & Explanation
. Metaplasia of the synovial subintimal connective tissue
Explanation
Primary synovial chondromatosis is a benign neoplastic process involving cartilaginous metaplasia of the synovial membrane. This leads to the formation of multiple, typically uniform, intra-articular loose bodies.
Question 6851
Topic: 10. Pathology and Oncology
A 40-year-old female presents with a painless, slow-growing mass on her proximal forearm. Radiographs demonstrate focal cortical hyperostosis of the proximal radius adjacent to a radiolucent soft-tissue mass. What is the most likely diagnosis?
Correct Answer & Explanation
. Parosteal lipoma
Explanation
Parosteal lipomas often cause a classic reactive cortical hyperostosis or bowing of the adjacent underlying bone. The purely radiolucent mass adjacent to the hyperostosis differentiates it from bone-forming tumors.
Question 6852
Topic: Bone Tumors
A 25-year-old male with a known distal femur osteochondroma presents with a rapidly enlarging, pulsatile popliteal mass and calf pain.
What is the most likely etiology of the new mass?
Correct Answer & Explanation
. Pseudoaneurysm of the popliteal artery
Explanation
Vascular complications, such as pseudoaneurysm formation, can occur due to repetitive friction of an artery over the hard cartilage cap of an osteochondroma. This is most classically described with the popliteal artery adjacent to a distal femoral osteochondroma.
Question 6853
Topic: 10. Pathology and Oncology
A 45-year-old male incidentally undergoes a radiograph of his foot after a mild sprain. The lateral view shows a well-defined lytic lesion in the calcaneus with central calcification creating a target sign. What is the appropriate management?
Correct Answer & Explanation
. Observation and reassurance
Explanation
Intraosseous lipomas are benign tumors commonly found in the calcaneus, often displaying a classic target sign due to central calcified fat necrosis. They are typically asymptomatic and require only observation.
Question 6854
Topic: Bone Tumors
A 15-year-old boy presents with a hard mass on his proximal tibia. Radiographs show a sessile bony exostosis pointing away from the joint space.
Which radiographic finding is required to definitively classify this lesion as an osteochondroma?
Correct Answer & Explanation
. Cortical and medullary continuity with the host bone
Explanation
The pathognomonic radiographic feature of an osteochondroma is direct continuity of its cortex and medullary cavity with that of the native host bone. The lesion classically grows in a direction pointing away from the adjacent joint.
Question 6855
Topic: 10. Pathology and Oncology
A 35-year-old male is evaluated for a large, deep intramuscular thigh mass. Core needle biopsy confirms the presence of well-differentiated adipocytes, but the pathologist requests fluorescence in situ hybridization (FISH) to differentiate between a benign lipoma and an atypical lipomatous tumor (ALT). Amplification of which of the following gene loci is pathognomonic for ALT?
Correct Answer & Explanation
. MDM2 and CDK4
Explanation
Atypical lipomatous tumors (well-differentiated liposarcomas) are characterized by supernumerary ring chromosomes leading to the amplification of the MDM2 and CDK4 genes. Benign lipomas typically lack this amplification.
Question 6856
Topic: 10. Pathology and Oncology
A 45-year-old male presents with recurrent catching and locking in his left knee. Imaging reveals numerous uniformly sized, small calcified bodies confined within the joint capsule.
What is the most definitive histological hallmark distinguishing this condition from secondary osteochondromatosis?
Correct Answer & Explanation
. Metaplastic hyaline cartilage nodules arising directly within the synovial subintima
Explanation
Primary synovial chondromatosis is characterized by metaplastic cartilage formation within the synovial tissue itself, leading to uniformly sized nodules. Secondary chondromatosis results from fragmented intra-articular cartilage/bone (e.g., severe osteoarthritis) and features variably sized, larger bodies.
Question 6857
Topic: Bone Tumors
A 14-year-old girl is evaluated for a painless, hard mass about the medial aspect of the proximal tibia.
Radiographs and MRI confirm the diagnosis of an osteochondroma. Which of the following imaging features is an absolute requirement to confidently make this diagnosis?
Correct Answer & Explanation
. Direct continuation of the medullary canal and cortex of the lesion with the host bone
Explanation
The pathognomonic radiographic and MRI finding for an osteochondroma is the continuity of both the cortex and the medullary canal of the lesion with that of the underlying host bone. A cartilage cap >2 cm in an adult is a warning sign for malignant transformation.
Question 6858
Topic: 10. Pathology and Oncology
A 35-year-old male with long-standing primary synovial chondromatosis of the hip presents with rapid worsening of pain and joint swelling over the last three months. MRI reveals permeative destruction of the femoral neck with marrow replacement and a large soft tissue mass breaking through the joint capsule. What complication has most likely occurred?
Correct Answer & Explanation
. Malignant transformation to secondary chondrosarcoma
Explanation
While primary synovial chondromatosis is benign, it carries a rare risk (approx. 1-5%) of malignant transformation to secondary chondrosarcoma. Rapid clinical deterioration, bone marrow invasion, and cortical destruction on imaging are key indicators of this complication.
Question 6859
Topic: 10. Pathology and Oncology
A 52-year-old male is referred for a painless, slow-growing soft tissue mass in his proximal forearm. MRI reveals a well-defined lipomatous mass located entirely within the belly of the brachioradialis muscle. Biopsy demonstrates mature adipocytes interdigitating with normal skeletal muscle fibers, without cytological atypia or lipoblasts. Which of the following statements regarding this lesion is true?
Correct Answer & Explanation
. It is an intramuscular lipoma, which frequently recur if only enucleated due to its infiltrative borders.
Explanation
Intramuscular lipomas are benign but uniquely exhibit an infiltrative growth pattern where mature adipocytes interdigitate with normal skeletal muscle. Despite this appearance, they lack atypia, though they have a higher recurrence rate than superficial lipomas if incompletely excised.
Question 6860
Topic: 10. Pathology and Oncology
An 18-year-old male presents with a pulsatile mass and a palpable thrill in the popliteal fossa of his right knee. He has a known history of a distal femur osteochondroma in the same leg. What is the most likely etiology of this vascular presentation?
Correct Answer & Explanation
. Popliteal artery pseudoaneurysm caused by mechanical friction from the osteochondroma
Explanation
A classic vascular complication of an osteochondroma, particularly in the distal femur or proximal tibia, is mechanical impingement and friction on the adjacent popliteal artery, leading to the formation of a pseudoaneurysm.
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