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Question 601

Topic: Bone Tumors

A 72-year-old woman presents with severe back pain. Laboratory testing shows anemia, hypercalcemia, and renal insufficiency. Radiographs reveal multiple punched-out lytic lesions in her skull and pelvis. Which of the following laboratory findings is most specific for confirming her likely diagnosis?

. Elevated alkaline phosphatase
. Elevated serum prostate-specific antigen
. Monoclonal spike on serum protein electrophoresis (SPEP)
. Elevated erythrocyte sedimentation rate (ESR)
. Decreased serum parathyroid hormone (PTH)

Correct Answer & Explanation

. Elevated alkaline phosphatase


Explanation

The patient's presentation of back pain, anemia, hypercalcemia, renal issues, and lytic bone lesions is highly characteristic of multiple myeloma (CRAB criteria). A monoclonal spike (M-protein) on Serum Protein Electrophoresis (SPEP) or Urine Protein Electrophoresis (UPEP) is a specific diagnostic hallmark.

Question 602

Topic: Bone Tumors

An 8-year-old boy incidentally undergoes radiography of the humerus, revealing a centrally located, well-circumscribed, radiolucent lesion in the proximal metaphysis with a cortical fragment resting at the dependent aspect of the cyst ('fallen leaf' sign). If aspirated, what is the most likely composition of the fluid contained within this lesion?

. Blood with numerous multi-nucleated giant cells
. Clear, serous fluid with high levels of prostaglandins
. Purulent fluid with abundant polymorphonuclear leukocytes
. Thick, mucin-rich viscous fluid
. Lipid-rich fluid containing visible cholesterol crystals

Correct Answer & Explanation

. Blood with numerous multi-nucleated giant cells


Explanation

The 'fallen leaf' sign and the location are pathognomonic for a Unicameral Bone Cyst (UBC), also known as a simple bone cyst. Aspiration of a UBC typically yields clear, yellow, serous fluid that is biochemically characterized by high levels of prostaglandins, particularly PGE2. Aneurysmal bone cysts (ABCs), in contrast, yield blood.

Question 603

Topic: Bone Tumors

A 16-year-old male complains of severe mid-tibial pain that is characteristically worse at night and awakens him from sleep. The pain dramatically resolves within 30 minutes of taking ibuprofen. Radiographs demonstrate a 7 mm radiolucent nidus surrounded by intense reactive cortical sclerosis. The profound pain relief provided by NSAIDs is primarily mediated by the inhibition of which substance highly produced by the nidus?

. Leukotriene B4
. Interleukin-1 beta
. Prostaglandin E2
. Tumor necrosis factor-alpha
. Substance P

Correct Answer & Explanation

. Leukotriene B4


Explanation

The clinical presentation is classic for an osteoid osteoma. The dramatic pain relief with non-steroidal anti-inflammatory drugs (NSAIDs) is secondary to the profound inhibition of Prostaglandin E2 (PGE2) synthesis. The nidus of an osteoid osteoma inherently produces levels of PGE2 that are up to 1000 times higher than surrounding normal tissue.

Question 604

Topic: Bone Tumors

A 15-year-old girl complains of severe, progressively worsening back pain that is predominantly nocturnal and relieved dramatically by non-steroidal anti-inflammatory drugs. Examination reveals a painful structural scoliotic curve. Imaging demonstrates a small radiolucent nidus with surrounding sclerosis in the posterior elements of L3. Which of the following statements is true regarding her scoliosis?

. The apex of the scoliotic curve points towards the side of the lesion
. The apex of the scoliotic curve points away from the side of the lesion
. Bracing is the most effective initial treatment for the curve
. Spinal fusion is required to prevent progressive deformity
. The curve is rigidly structural and will not resolve with excision of the lesion

Correct Answer & Explanation

. The apex of the scoliotic curve points towards the side of the lesion


Explanation

The patient has an osteoid osteoma of the spine. These lesions provoke severe muscle spasm leading to a painful, non-structural scoliosis. Classically, the apex of the scoliotic curve points away from the side of the lesion (i.e., the lesion is on the concave side of the curve). Resection or ablation of the nidus usually leads to spontaneous resolution of the curve if present for less than 15 months.

Question 605

Topic: Bone Tumors

A 9-year-old boy falls and fractures his proximal humerus. Radiographs reveal a central, purely lytic lesion in the metaphysis with a piece of cortical bone resting dependently within the cavity ('fallen leaf' sign). If aspirated, the fluid within this cyst is most likely to contain high levels of which substance?

. Prostaglandins
. Cholesterol clefts
. Frank, uncoagulated blood
. Purulent exudate
. Eosinophilic granulocytes

Correct Answer & Explanation

. Prostaglandins


Explanation

The clinical and radiographic presentation is classic for a Unicameral Bone Cyst (UBC) complicated by a pathologic fracture (fallen leaf sign). The serous fluid within a UBC is typically high in prostaglandins, interleukins, and other bone-resorbing factors, which contribute to the lytic expansion of the cyst.

Question 606

Topic: Bone Tumors

An 8-year-old boy presents with a symptomatic unicameral bone cyst (UBC) in the proximal humerus. Radiographs show a 'fallen leaf' sign. What is the most widely accepted initial invasive treatment for an active, symptomatic UBC?

. En bloc resection and structural allograft
. Curettage and high-speed burring
. Aspiration and injection of methylprednisolone or bone marrow aspirate
. Prophylactic flexible intramedullary nailing
. Amputation

Correct Answer & Explanation

. En bloc resection and structural allograft


Explanation

The standard first-line surgical/invasive treatment for a symptomatic or fracture-prone unicameral bone cyst is aspiration followed by injection of methylprednisolone, demineralized bone matrix, or bone marrow aspirate. Curettage and grafting are reserved for refractory cases.

Question 607

Topic: Bone Tumors

A 19-year-old male presents with progressive back pain that is worse at night and poorly relieved by NSAIDs. Imaging reveals a 2.5 cm radiolucent nidus with surrounding sclerosis in the posterior elements of L4. What is the most likely diagnosis?

. Osteoid osteoma
. Osteoblastoma
. Aneurysmal bone cyst
. Osteosarcoma
. Eosinophilic granuloma

Correct Answer & Explanation

. Osteoid osteoma


Explanation

While osteoid osteoma and osteoblastoma are histologically similar, osteoblastomas are distinguished by a nidus size greater than 2 cm (often >1.5-2 cm) and pain that is less responsive to NSAIDs. They commonly occur in the posterior elements of the spine.

Question 608

Topic: Bone Tumors

A 30-year-old female presents with a painless mass on the posterior aspect of her distal thigh. Radiographs show a dense, heavily ossified mass attached to the posterior cortex of the distal femur with a 'string sign'. What is the most likely diagnosis?

. Periosteal osteosarcoma
. Parosteal osteosarcoma
. Osteochondroma
. High-grade surface osteosarcoma
. Myositis ossificans

Correct Answer & Explanation

. Periosteal osteosarcoma


Explanation

Parosteal osteosarcoma is a low-grade surface osteosarcoma that typically occurs on the posterior aspect of the distal femur. It presents as a heavily ossified mass, often with a radiolucent cleft (string sign) separating it from the underlying cortex.

Question 609

Topic: Bone Tumors

A 62-year-old man presents with generalized bone pain, fatigue, and renal impairment. Radiographs show multiple 'punched-out' lytic lesions in the skull and pelvis. Which of the following laboratory findings is most specific for confirming the diagnosis?

. Elevated alkaline phosphatase
. Elevated serum prostate-specific antigen
. Monoclonal spike on serum protein electrophoresis
. Positive Philadelphia chromosome
. Elevated serum calcitonin

Correct Answer & Explanation

. Elevated alkaline phosphatase


Explanation

The clinical picture (CRAB criteria: hyperCalcemia, Renal involvement, Anemia, Bone lytic lesions) is classic for Multiple Myeloma. A monoclonal spike (M-protein) on serum or urine protein electrophoresis (SPEP/UPEP) confirms the diagnosis of a plasma cell dyscrasia.

Question 610

Topic: Bone Tumors

A 25-year-old female presents with a painless bowing deformity of her proximal femur. Radiographs reveal a ground-glass intramedullary lesion with cortical thinning and a 'shepherd's crook' deformity. The underlying pathophysiology involves a mutation in which of the following?

. Gs alpha protein (GNAS1)
. Fibroblast growth factor receptor 3 (FGFR3)
. Retinoblastoma gene (RB1)
. EXT1 gene
. Tumor suppressor p53

Correct Answer & Explanation

. Gs alpha protein (GNAS1)


Explanation

The presentation is consistent with fibrous dysplasia, characterized by the 'ground-glass' appearance and bowing deformities (shepherd's crook in the proximal femur). The etiology is a somatic activating mutation in the GNAS1 gene, which encodes the alpha subunit of the stimulatory G protein.

Question 611

Topic: Bone Tumors

An 18-year-old male presents with a painful swelling over the anterior tibia. Radiographs show a radiolucent surface lesion in the diaphysis with crater-like scalloping of the underlying cortex and perpendicular bone spicules ('hair-on-end'). MRI confirms no medullary involvement. Histology shows predominantly intermediate-grade chondroblastic matrix. What is the diagnosis?

. Parosteal osteosarcoma
. Periosteal osteosarcoma
. High-grade surface osteosarcoma
. Periosteal chondroma
. Osteochondroma

Correct Answer & Explanation

. Periosteal osteosarcoma


Explanation

Periosteal osteosarcoma is a rare, intermediate-grade surface osteosarcoma that typically arises on the diaphysis of long bones. Radiographically, it causes a scalloped saucerization of the outer cortex with perpendicular spicules of bone. Histologically, it is predominantly chondroblastic. Parosteal osteosarcoma is low-grade, heavily ossified, fibroblastic, and typically posterior distal femur.

Question 612

Topic: Bone Tumors

An 18-year-old male is undergoing treatment with the MAP protocol (High-dose Methotrexate, Adriamycin/Doxorubicin, and Cisplatin) for high-grade conventional osteosarcoma of the distal femur. Six months into treatment, he presents with progressive dyspnea on exertion, orthopnea, and lower extremity edema. Echocardiography reveals a significantly reduced left ventricular ejection fraction. Which chemotherapeutic agent is most likely responsible?

. Methotrexate
. Cisplatin
. Doxorubicin
. Ifosfamide
. Bleomycin

Correct Answer & Explanation

. Doxorubicin


Explanation

Doxorubicin (Adriamycin) is an anthracycline known for causing dose-dependent, cumulative, and potentially irreversible cardiotoxicity (cardiomyopathy leading to congestive heart failure). Cisplatin is associated with ototoxicity and nephrotoxicity. Methotrexate is associated with hepatotoxicity, mucositis, and myelosuppression. Ifosfamide is known for hemorrhagic cystitis. Bleomycin is associated with pulmonary fibrosis.

Question 613

Topic: Bone Tumors

A 45-year-old female with known polyostotic fibrous dysplasia presents with a newly noticed, painless soft tissue mass in her right anterior thigh. MRI shows a well-circumscribed, T2-hyperintense intramuscular lesion. Biopsy reveals hypocellular, abundant myxoid stroma with rare bland spindle cells and no lipoblasts. The combination of these findings characterizes which of the following syndromes?

. Mazabraud syndrome
. McCune-Albright syndrome
. Maffucci syndrome
. Ollier disease
. Gardner syndrome

Correct Answer & Explanation

. Mazabraud syndrome


Explanation

Mazabraud syndrome is the rare association of single or multiple intramuscular myxomas (the soft tissue mass described) with fibrous dysplasia (which may be monostotic or polyostotic). McCune-Albright syndrome features fibrous dysplasia, precocious puberty, and cafe-au-lait spots. Maffucci syndrome involves multiple enchondromas and soft tissue hemangiomas.

Question 614

Topic: Bone Tumors

A 40-year-old male presents with shoulder pain. Radiographs show a lytic lesion in the proximal humeral epiphysis with central calcifications. Histological examination reveals tumor cells with abundant clear cytoplasm, distinct cell membranes, and scattered areas of conventional chondrosarcoma. What is the most likely diagnosis?

. Chondroblastoma
. Clear cell chondrosarcoma
. Giant cell tumor of bone
. Aneurysmal bone cyst
. Osteosarcoma

Correct Answer & Explanation

. Clear cell chondrosarcoma


Explanation

Clear cell chondrosarcoma typically occurs in the epiphysis of long bones in adults (often older than those with chondroblastoma, which is also epiphyseal). Histology shows sheets of clear cells and may have areas of conventional low-grade chondrosarcoma.

Question 615

Topic: Bone Tumors

In a patient diagnosed with multiple myeloma, which of the following laboratory markers is considered the most powerful independent prognostic indicator of survival and is incorporated into the International Staging System (ISS)?

. Serum calcium level
. Bence Jones proteinuria
. Serum beta-2 microglobulin
. Alkaline phosphatase
. C-reactive protein

Correct Answer & Explanation

. Serum beta-2 microglobulin


Explanation

Serum beta-2 microglobulin (along with serum albumin) forms the basis of the International Staging System (ISS) for multiple myeloma and is the most significant prognostic factor. Higher levels correlate with higher tumor burden and a poorer prognosis.

Question 616

Topic: Bone Tumors

A 9-year-old boy is scheduled to undergo a Van Nes rotationplasty for an osteosarcoma of the distal femur. For this procedure to be mechanically successful and functional, which of the following anatomical structures must be preserved and fully functional?

. Femoral artery
. Saphenous nerve
. Sciatic nerve
. Quadriceps mechanism
. Hamstrings

Correct Answer & Explanation

. Sciatic nerve


Explanation

In a Van Nes rotationplasty, the limb is shortened, rotated 180 degrees, and reattached so that the ankle joint functions as a knee joint. The sciatic nerve MUST be preserved to maintain motor and sensory function to the rotated leg and foot, which will now act as the calf and knee. The femoral vessels are often resected and anastomosed.

Question 617

Topic: Bone Tumors

A 16-year-old male is receiving neoadjuvant chemotherapy for osteosarcoma using the standard MAP regimen (Methotrexate, Doxorubicin, Cisplatin). He subsequently develops irreversible congestive heart failure. Which of the chemotherapeutic agents is most likely responsible for this specific toxicity?

. Methotrexate
. Doxorubicin
. Cisplatin
. Ifosfamide
. Vincristine

Correct Answer & Explanation

. Doxorubicin


Explanation

Doxorubicin (Adriamycin) is an anthracycline known for its dose-dependent, cumulative, and potentially irreversible cardiotoxicity (dilated cardiomyopathy and congestive heart failure). Cisplatin is associated with ototoxicity and nephrotoxicity. Methotrexate is associated with mucositis, hepatotoxicity, and renal toxicity.

Question 618

Topic: Bone Tumors

A patient diagnosed with polyostotic fibrous dysplasia presents with a newly discovered, painless, deep soft tissue mass in the thigh. Excisional biopsy of the mass reveals a well-circumscribed lesion composed of bland spindle cells in an abundant myxoid stroma with decreased vascularity. The presence of this soft tissue lesion in conjunction with fibrous dysplasia is known as:

. McCune-Albright syndrome
. Mazabraud syndrome
. Ollier disease
. Maffucci syndrome
. Jaffe-Campanacci syndrome

Correct Answer & Explanation

. Mazabraud syndrome


Explanation

Mazabraud syndrome is a rare disorder characterized by the association of fibrous dysplasia (usually polyostotic) and one or more intramuscular myxomas. McCune-Albright involves fibrous dysplasia, precocious puberty, and cafe-au-lait spots.

Question 619

Topic: Bone Tumors

An 18-year-old female presents with a rapidly expanding, painful lytic lesion in her distal fibula. MRI shows an expansile lesion with multiple fluid-fluid levels. Genetic analysis reveals a characteristic rearrangement. Which gene is primarily involved?

. EWSR1
. USP6
. GNAS
. EXT1
. RB1

Correct Answer & Explanation

. USP6


Explanation

Primary aneurysmal bone cysts (ABCs) are benign but locally aggressive neoplasms driven by USP6 gene rearrangements. MRI typically shows characteristic fluid-fluid levels indicating blood components of varying ages.

Question 620

Topic: Bone Tumors

A 68-year-old female complains of generalized bone pain and fatigue. Radiographs show multiple "punched-out" lytic lesions in her skull and pelvis. A bone scan shows no increased uptake in these areas. Which of the following laboratory findings is most likely to confirm the diagnosis?

. Elevated serum alkaline phosphatase
. Monoclonal spike on serum protein electrophoresis
. Positive Bence-Jones protein but normal serum calcium
. Elevated prostate-specific antigen
. High titer of antinuclear antibodies

Correct Answer & Explanation

. Monoclonal spike on serum protein electrophoresis


Explanation

Multiple myeloma frequently presents with punched-out lytic bone lesions that are characteristically "cold" on technetium bone scans due to osteoblast suppression. Diagnosis is confirmed by demonstrating a monoclonal paraprotein spike on serum or urine protein electrophoresis.