Paget's Disease of Bone — MCQs
Which of the following conditions is not typically associated with an underlying malignancy?
Which is the drug of choice in Paget's disease?
Osteonecrosis is seen in all except
Brittle bone disease is -
Avascular necrosis of bone is LEAST likely to be associated with?
A 50-year-old male presents with backache, morning stiffness, red eye, and ankle swelling. Based on the X-ray provided, what is the most likely diagnosis?
All of the following are true regarding Paget's Disease except -
In which condition is the 'Picture frame vertebra' seen?
A patient undergoing chest x-ray following an automobile accident is found to have an enlarged mediastinum with bilateral hilar and right paratracheal adenopathy. The patient has been asymptomatic, but careful examination demonstrates an enlarged cervical lymph node. This node is biopsied and demonstrates involvement by small, non-caseating granulomas. Occasional giant cells with stellate inclusions are seen within the granulomas. These are most likely which of the following?
Spine MRI shows 'pencil-sharpened' vertebral bodies and 'H-shaped' vertebrae on T1-weighted images. Most likely diagnosis?
Paget's Disease of Bone Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following conditions is not typically associated with an underlying malignancy?
- A. Paget disease of vulva
- B. Paget disease of anal region
- C. Paget disease of bone (Correct Answer)
- D. Paget disease of nipple
Explanation: ***Paget disease of bone*** - While Paget disease of bone can rarely undergo **malignant transformation** into **osteosarcoma**, it is itself a disorder of excessive bone remodeling and **not directly an underlying malignancy** like the other Paget diseases [2]. - The primary concern in Paget disease of bone is metabolic bone changes and potential complications like fractures, rather than being a superficial manifestation of internal cancer [2]. *Paget disease of nipple* - This condition is almost always associated with an **underlying ductal carcinoma in situ** or invasive adenocarcinoma of the breast [1]. - The epidermal changes are a manifestation of malignant cells migrating from the underlying breast tissue [1]. *Paget disease of vulva* - While it can occur as a primary intraepithelial neoplasm (adenocarcinoma in situ), approximately 20-30% of cases are associated with an **underlying invasive adenocarcinoma**, either vulvar or originating from other sites such as the colon, bladder, or urethra. - Its presence necessitates a thorough search for associated malignancies. *Paget disease of anal region* - Similar to vulvar Paget disease, it can be a primary intraepithelial adenocarcinoma, but it frequently is associated with an **underlying adenocarcinoma** of the colon, rectum, or anal glands. - Evaluation for an internal malignancy is crucial when this diagnosis is made. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Breast, pp. 1061-1062. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1191-1194.
Question 2: Which is the drug of choice in Paget's disease?
- A. Allopurinol
- B. Calcitonin
- C. Alendronate (Correct Answer)
- D. Steroids
Explanation: ***Alendronate*** - **Bisphosphonates** like alendronate are the **first-line treatment** for Paget's disease due to their potent antiresorptive inhibitory effect on **osteoclasts**. - They reduce bone turnover, bone pain, and the risk of complications such as **fractures** and **bone deformities**. *Allopurinol* - This drug is used to treat **gout** by inhibiting **xanthine oxidase** and reducing uric acid production. - It has no role in the management of Paget's disease, which is a disorder of abnormal bone remodeling. *Calcitonin* - Historically, calcitonin was used for Paget's disease, but its effectiveness is **less than bisphosphonates** and it is associated with more side effects. - It is now generally reserved for patients who **cannot tolerate bisphosphonates** or have severe renal impairment. *Steroids* - **Corticosteroids** are potent anti-inflammatory and immunosuppressive agents. - They are primarily used in conditions like **autoimmune disorders** or severe inflammatory diseases, and are **not indicated** for the treatment of Paget's disease.
Question 3: Osteonecrosis is seen in all except
- A. Fracture neck femur
- B. Paget's disease (Correct Answer)
- C. Perthe's disease
- D. Sickle cell anemia
Explanation: ***Paget's disease*** - **Paget's disease of bone (osteitis deformans)** is a localized disorder of bone remodeling, characterized by excessive and disorganized bone formation, leading to enlarged, softened, and misshapen bones, but not directly causing osteonecrosis. - While complications like **pathological fractures** and **osteosarcoma** can occur, primary osteonecrosis is not a typical feature of Paget's disease itself. *Fracture neck femur* - **Fractures of the femoral neck** can disrupt the blood supply to the femoral head, particularly the medial circumflex femoral artery, leading to **avascular necrosis** (osteonecrosis) of the femoral head. - This is a well-known and common complication, especially in displaced fractures. *Sickle cell anemia* - **Sickle cell anemia** causes sickling of red blood cells, leading to **vaso-occlusion** and impaired blood flow to bones, resulting in **bone infarcts** (osteonecrosis). - This can affect various bones, including the femoral head, humeral head, and vertebrae. *Perthe's disease* - **Perthe's disease** (Legg-Calvé-Perthes disease) is a childhood condition characterized by **idiopathic osteonecrosis** of the femoral head. - It involves the collapse and subsequent re-ossification of the femoral epiphysis due to an interruption of its blood supply.
Question 4: Brittle bone disease is -
- A. Osteoporosis
- B. Pagets disease
- C. Osteopetrosis
- D. Osteogenesis imperfecta (Correct Answer)
Explanation: ***Osteogenesis imperfecta*** - **Osteogenesis imperfecta** is an inherited disorder characterized by **brittle bones** that fracture easily, due to a defect in **collagen type I** synthesis. - Patients often present with **blue sclera**, **dentinogenesis imperfecta**, and **hearing loss**, in addition to frequent fractures. *Osteoporosis* - **Osteoporosis** is a condition of **decreased bone density**, making bones fragile and prone to fracture, but it is not typically referred to as "brittle bone disease" in the same congenital sense. - It is more common in older adults and is often related to **hormonal changes** (e.g., post-menopause) or lifestyle factors. *Paget's disease* - **Paget's disease of bone** involves abnormal bone remodeling with excessive bone resorption followed by disorganized and expanded bone formation, leading to **enlarged, weakened bones**. - It typically affects older individuals and can lead to bone pain, deformities, and fractures, but it's not the primary condition associated with "brittle bone disease." *Osteopetrosis* - **Osteopetrosis** is characterized by **abnormally dense bones** due to impaired osteoclast function, leading to a buildup of bone. - While bones are dense, they are also **brittle** and prone to fracture, and the condition is also known as "marble bone disease" rather than "brittle bone disease."
Question 5: Avascular necrosis of bone is LEAST likely to be associated with?
- A. Osgood -Schlatter disease (Correct Answer)
- B. Long-term use of corticosteroids
- C. Sickle-cell disease
- D. Legg-Perthes disease
Explanation: ***Osgood-Schlatter disease*** - This condition is characterized by **inflammation of the patellar ligament** at its insertion into the tibial tuberosity, primarily due to repetitive stress in adolescents. - While it involves pain and swelling around the knee, it is a **traction apophysitis** and not a form of avascular necrosis. *Long-term use of corticosteroids* - **Corticosteroids** are a well-established risk factor for avascular necrosis, particularly in the femoral head, by affecting lipid metabolism and blood flow. - They can lead to **fat embolism** and increased intraosseous pressure, compromising blood supply to the bone. *Sickle-cell disease* - **Sickle cell disease** significantly increases the risk of avascular necrosis due to **vaso-occlusive crises**, where sickled red blood cells block small blood vessels. - This leads to **ischemia and infarction** in bone marrow, commonly affecting the femoral and humeral heads. *Legg-Perthes disease* - This is a specific type of **avascular necrosis of the femoral head** in children, causing a temporary interruption of blood supply to the epiphysis. - It results in the collapse of the femoral head and subsequent repair processes, consistent with the pathology of avascular necrosis.
Question 6: A 50-year-old male presents with backache, morning stiffness, red eye, and ankle swelling. Based on the X-ray provided, what is the most likely diagnosis?
- A. Paget's Disease
- B. Healed TB spine
- C. Osteopetrosis
- D. Ankylosing Spondylitis (Correct Answer)
Explanation: ***Ankylosing Spondylitis*** - The X-ray likely shows features of **sacroiliitis** (joint space narrowing, sclerosis, erosion) and possibly **vertebral fusion**, consistent with ankylosing spondylitis. The clinical presentation of **backache, morning stiffness, red eye (uveitis), and ankle swelling (enthesitis)** are classic extra-articular manifestations and peripheral arthritis of ankylosing spondylitis. - X-ray findings like **bamboo spine** (late stage), syndesmophytes, and erosions of the sacroiliac joints are characteristic of this seronegative spondyloarthropathy. *Paget's Disease* - Paget's disease is characterized by disorganized bone remodeling, leading to bone enlargement, deformity, and increased density or lysis. It typically does not present with **sacroiliitis** or widespread **ankylosis**. - Clinical features usually include **bone pain**, **bone deformities**, and potentially **nerve compression**, which are not the primary symptoms described. *Healed TB spine* - Healed TB spine (Pott's disease) would typically show bone destruction, collapse of vertebral bodies, **kyphosis**, and often calcified abscesses. - While backache can occur, the presence of **red eye** and **ankle swelling** are not characteristic of spinal tuberculosis. *Osteopetrosis* - Osteopetrosis is a rare genetic disorder characterized by **increased bone density** due to defective osteoclast function, leading to abnormally thick and brittle bones. - The X-ray would show **generalized sclerosis** (diffuse increased bone density) throughout the skeleton, which is not the primary finding indicated by the clinical context of sacroiliitis and joint fusion.
Question 7: All of the following are true regarding Paget's Disease except -
- A. Pelvis is the most common site
- B. It may progress to a secondary chondrosarcoma (Correct Answer)
- C. High output cardiac failure is one of the complications
- D. Cranial nerve involvement may be seen
Explanation: ***It may progress to a secondary chondrosarcoma*** - While Paget's disease does have a risk of malignant transformation, it most commonly progresses to **osteosarcoma**, not chondrosarcoma [1]. - The malignant transformation occurs in a very small percentage of patients, estimated between 0.7% and 0.9%, primarily affecting older individuals [1]. *Pelvis is the most common site* - The **pelvis** is indeed the most common site affected by Paget's disease, occurring in about 75% of cases [1]. - Other frequently involved bones include the skull, spine, and long bones (femur, tibia) [1]. *High output cardiac failure is one of the complications* - **High-output cardiac failure** can be a complication of widespread Paget's disease due to increased vascularity in affected bone [1]. - The body attempts to compensate for the extensive bone remodeling by increasing blood flow, leading to increased cardiac workload. *Cranial nerve involvement may be seen* - Enlargement of the skull bones in Paget's disease can lead to **compression of cranial nerves**, resulting in symptoms such as hearing loss (compression of the vestibulocochlear nerve) [1]. - This nerve impingement is due to the abnormal bone growth narrowing the foramina through which the nerves pass [1].
Question 8: In which condition is the 'Picture frame vertebra' seen?
- A. Paget disease (Correct Answer)
- B. Osteopetrosis (marble bone disease)
- C. Ankylosing spondylitis (AS)
- D. Osteoporosis
Explanation: ***Paget disease*** - The "picture frame vertebra" sign is a classic radiographic finding in **Paget disease**, characterized by **cortical thickening** and sclerosis around the vertebral body circumference, resembling a picture frame. - This appearance is due to the disordered bone remodeling processes (increased osteoclastic bone resorption followed by disorganized osteoblastic new bone formation) characteristic of Paget disease. *Osteopetrosis (marble bone disease)* - Osteopetrosis is characterized by **increased bone density** due to defective osteoclast function, leading to bones that are dense but brittle. - It does not typically present with the specific "picture frame" appearance of individual vertebrae, but rather with diffuse sclerosis of bones. *Ankylosing spondylitis (AS)* - Ankylosing spondylitis primarily affects the **axial skeleton**, causing inflammation and eventual fusion of the vertebrae (leading to a "bamboo spine" appearance). - While it involves the spine, it does not produce the "picture frame" vertebral sign seen in Paget disease. *Osteoporosis* - Osteoporosis is characterized by **reduced bone mass** and microstructural deterioration of bone tissue, leading to increased bone fragility and fracture risk. - Radiographically, it shows **decreased bone density** and possible vertebral compression fractures, which is the opposite of the increased bone density and cortical thickening seen in the "picture frame" sign.
Question 9: A patient undergoing chest x-ray following an automobile accident is found to have an enlarged mediastinum with bilateral hilar and right paratracheal adenopathy. The patient has been asymptomatic, but careful examination demonstrates an enlarged cervical lymph node. This node is biopsied and demonstrates involvement by small, non-caseating granulomas. Occasional giant cells with stellate inclusions are seen within the granulomas. These are most likely which of the following?
- A. Aschoff bodies
- B. Anitschkow cells
- C. Paget's cells
- D. Asteroid bodies (Correct Answer)
Explanation: ***Asteroid bodies*** - The presence of **non-caseating granulomas** with giant cells containing **stellate inclusions** (asteroid bodies) is characteristic of **sarcoidosis** [1]. - **Sarcoidosis** often presents with **hilar** and **paratracheal lymphadenopathy**, as well as constitutional symptoms which can be subtle or absent [2]. *Aschoff bodies* - **Aschoff bodies** are pathognomonic for **rheumatic fever** and consist of fibrinoid necrosis surrounded by lymphocytes, plasma cells, and Anitschkow cells. - They are typically found in the **myocardium** and other connective tissues in the heart, not in lymph nodes with sarcoidosis-like features. *Anitschkow cells* - **Anitschkow cells** are large, activated macrophages, often described as "caterpillar cells" due to their wavy chromatin, characteristic of **Aschoff bodies** in **rheumatic fever**. - They are primarily seen in the context of **myocardial inflammation** due to rheumatic fever and are not associated with granulomatous lymphadenopathy. *Paget's cells* - **Paget's cells** are large, malignant epithelial cells with abundant pale cytoplasm and prominent nucleoli, associated with **Paget's disease of the nipple** (intraepidermal adenocarcinoma). - They are not found in granulomas within lymph nodes and are unrelated to the described clinical and histological findings. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 198-200. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, pp. 700-701.
Question 10: Spine MRI shows 'pencil-sharpened' vertebral bodies and 'H-shaped' vertebrae on T1-weighted images. Most likely diagnosis?
- A. Sickle cell disease (Correct Answer)
- B. Paget's disease
- C. Thalassemia
- D. Osteopetrosis
Explanation: ***Sickle cell disease*** - **'Pencil-sharpened' vertebral bodies** and **'H-shaped' vertebrae** are characteristic findings in sickle cell disease due to chronic **ischemic necrosis** of the vertebral endplates. - This vertebral deformity arises from the collapse of the central portion of the vertebral body, often associated with episodes of **vaso-occlusion**. *Paget's disease* - Characterized by abnormal bone remodeling, leading to **bone enlargement** and thickening, not vertebral notching or collapse. - Common radiological findings include **cotton wool appearance** of the skull and **sclerosis** of the vertebrae, distinct from the described MRI findings. *Thalassemia* - Causes significant **bone marrow expansion** due to ineffective erythropoiesis, leading to cortical thinning and widened medullary spaces. - While it can affect bone morphology, it typically does not produce the specific 'H-shaped' or 'pencil-sharpened' vertebral body deformities. *Osteopetrosis* - Known as **'marble bone disease'**, characterized by abnormally dense bones due to defective osteoclast function. - Radiologically, it presents with **generalized increased bone density** and obliterated marrow spaces, which is opposite to the changes seen in sickle cell disease.
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