Bone Sarcomas Indian Medical PG Practice Questions and MCQs
Practice Indian Medical PG questions for Bone Sarcomas. These multiple choice questions (MCQs) cover important concepts and help you prepare for your exams.
Bone Sarcomas Indian Medical PG Question 1: Epiphyseal tumor before fusion of epiphysis:
- A. Chondrosarcoma
- B. Chondroblastoma (Correct Answer)
- C. Giant cell tumor
- D. Ewing's sarcoma
Bone Sarcomas Explanation: ***Chondroblastoma***
- This is primarily an **epiphyseal tumor** that occurs predominantly in adolescents and young adults **before epiphyseal fusion**.
- It is a **benign cartilaginous tumor** that typically presents with pain and swelling around the affected joint.
*Chondrosarcoma*
- This is a **malignant cartilaginous tumor** that typically occurs in older adults, usually after epiphyseal fusion.
- While it can occur in various bones, it is generally found in the **metaphysis or diaphysis**, not primarily the epiphysis before fusion.
*Giant cell tumor*
- This tumor is typically found in the **epiphysis/metaphysis** but primarily occurs in skeletally mature individuals **after epiphyseal closure**.
- It is characterized by its aggressive nature and high recurrence rate, but is less common before epiphyseal fusion.
*Ewing's sarcoma*
- This is a highly **malignant bone tumor** that primarily affects the **diaphysis** of long bones and flat bones.
- It is common in children and young adults but is not characteristically an epiphyseal tumor before fusion.
Bone Sarcomas Indian Medical PG Question 2: All of the following are true about Ewing's sarcoma, EXCEPT
- A. Responds well to radiotherapy
- B. Common in 4th decade (Correct Answer)
- C. Grows in diaphysis of long bones
- D. Has onion peel appearance on radiograph
Bone Sarcomas Explanation: ***Common in 4th decade***
- Ewing's sarcoma is most common in **children and adolescents**, typically presenting between 10 and 20 years of age, making it rare in the fourth decade.
- It is the **second most common primary bone tumor** in children, after osteosarcoma.
*Responds well to radiotherapy*
- Ewing's sarcoma cells are highly **radiosensitive**, making radiotherapy a crucial component of its multimodal treatment.
- Both **local control** and **palliation** can often be achieved with radiation therapy, even in metastatic disease.
*Grows in diaphysis of long bones*
- Ewing's sarcoma characteristically arises in the **diaphysis (shaft)** or metadiaphysis of long bones, such as the femur and tibia.
- The tumor's origin in the diaphysis is a key distinguishing feature from other bone tumors that often originate in the metaphysis.
*Has onion peel appearance on radiograph*
- The classic radiographic sign of Ewing's sarcoma is a **multilaminated periosteal reaction**, which creates a characteristic **"onion peel" appearance** due to new bone formation.
- This appearance reflects the aggressive nature of the tumor, as it repeatedly breaches the cortex, stimulating new layers of periosteal bone.
Bone Sarcomas Indian Medical PG Question 3: 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
Bone Sarcomas 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.
Bone Sarcomas Indian Medical PG Question 4: Which bone tumor involves the epiphysis?
- A. Osteosarcoma
- B. Giant cell tumor (Correct Answer)
- C. Ewing's sarcoma
- D. Multiple myeloma
Bone Sarcomas Explanation: ***Giant cell tumor***
- **Giant cell tumor (GCT)**, also known as osteoclastoma, characteristically arises in the **epiphysis** [1] or **metaphysis** of long bones in adults.
- It is a benign but locally aggressive tumor that often presents with pain, swelling, and reduced range of motion in the affected joint [1].
*Osteosarcoma*
- **Osteosarcoma** typically arises in the **metaphysis** of long bones, particularly around the knee (distal femur, proximal tibia).
- It is a highly malignant primary bone tumor characterized by the production of **osteoid** by tumor cells [2].
*Ewing's sarcoma*
- **Ewing's sarcoma** most commonly affects the **diaphysis** of long bones or flat bones (e.g., pelvis, scapula, ribs).
- It is characterized by small, round, blue cells and often presents with pain, swelling, and systemic symptoms like fever.
*Multiple myeloma*
- **Multiple myeloma** is a malignancy of **plasma cells** that primarily affects the **bone marrow** and can cause widespread osteolytic lesions.
- It typically presents in older adults and affects bones with active marrow, such as the vertebrae, ribs, skull, and pelvis, rather than being localized to the epiphysis as a primary bone tumor.
**References:**
[1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1205-1206.
[2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 673-674.
Bone Sarcomas Indian Medical PG Question 5: Sunburst appearance found in:
- A. Ewing sarcoma
- B. Giant cell tumor
- C. Osteosarcoma (Correct Answer)
- D. Chondrosarcoma
Bone Sarcomas Explanation: ***Osteosarcoma***
- A classic radiographic feature of **osteosarcoma** is the **sunburst appearance**, which represents new bone formation radiating outwards from the cortex.
- This aggressive tumor typically occurs in the **metaphysis of long bones** (e.g., distal femur, proximal tibia).
*Ewing sarcoma*
- Characteristically presents with an **onion-skin appearance** (concentric layers of periosteal reaction) due to rapid bone destruction and new bone formation.
- It often affects the **diaphysis of long bones** and flat bones, unlike the metaphyseal involvement of osteosarcoma.
*Giant cell tumor*
- Known for its characteristic **soap-bubble appearance** on imaging, indicating a lytic lesion with thin septations.
- Typically found in the **epiphysis of long bones** in young adults.
*Chondrosarcoma*
- Radiographically, it often shows a **lobulated lesion** with **calcified cartilage matrix** (popcorn or ring-and-arc calcifications).
- While it can be destructive, it does not typically exhibit the radiating pattern seen in a sunburst appearance, which is primarily an osteoblastic phenomenon.
Bone Sarcomas Indian Medical PG Question 6: Dense cartilage matrix calcification is characteristically seen in
- A. Chondrosarcoma (Correct Answer)
- B. Fibrosarcoma
- C. Chondroblastoma
- D. Osteosarcoma
Bone Sarcomas Explanation: ***Chondrosarcoma***
- This is a **malignant cartilaginous tumor** that characteristically exhibits **dense cartilage matrix calcification** appearing as **ring-and-arc, flocculent, or popcorn-like** calcifications on imaging
- This pattern of calcification is a key diagnostic feature differentiating it from other bone tumors
- The calcification reflects the chondroid (cartilaginous) matrix produced by the tumor
*Fibrosarcoma*
- This is a **malignant tumor derived from fibrous tissue** and typically shows **no calcification** because it does not produce cartilage or bone matrix
- Radiographically appears as a lytic, destructive lesion lacking the characteristic calcifications
*Chondroblastoma*
- This is a **rare benign cartilaginous tumor** usually occurring in the **epiphyses of long bones** in skeletally immature patients
- While it may show calcification in ~50% of cases, it is typically **fine stippled calcification** rather than the dense ring-and-arc pattern of chondrosarcoma
- Usually appears as a well-defined lytic lesion with a sclerotic rim
*Osteosarcoma*
- This is a **malignant bone-forming tumor** that produces **osteoid matrix calcification** appearing as **cloud-like or amorphous dense calcification**
- Also shows characteristic periosteal reactions (**sunburst pattern** or **Codman's triangle**)
- The calcification pattern differs from chondrosarcoma as it reflects osseous rather than cartilaginous matrix
Bone Sarcomas Indian Medical PG Question 7: In Ewing's sarcoma, histology shows small round cells that are filled with which of the following?
- A. Glycogen (Correct Answer)
- B. Iron
- C. Fat
- D. Mucin
Bone Sarcomas Explanation: ***Glycogen***
- Ewing's sarcoma cells are characterized by abundant intracellular **glycogen**, which can be demonstrated by a **PAS (Periodic Acid-Schiff) stain** that is positive and digestable by diastase.
- The presence of glycogen is a key diagnostic feature, especially when differentiating it from other small round blue cell tumors.
*Iron*
- While iron is present in various cells, it is not a characteristic **cytoplasmic inclusion** used for the diagnosis of Ewing's sarcoma.
- Iron accumulation is more typical in conditions like **hemochromatosis** or in hemosiderin-laden macrophages.
*Fat*
- Fat vacuoles are characteristic of **liposarcomas** and other tumors with adipocytic differentiation, not Ewing's sarcoma.
- Ewing's sarcoma cells are typically primitive and undifferentiated, lacking significant lipid accumulation.
*Mucin*
- Mucin production is a hallmark of **adenocarcinomas** and some other epithelial tumors.
- Ewing's sarcoma is a mesenchymal tumor and does not produce mucin.
Bone Sarcomas Indian Medical PG Question 8: A child 10 yrs of age presents with a mass on his left thigh. The mass seems to be arising from the diaphysis of the femur and involving the soft tissue of the thigh. The child is having fever also. Give your most probable diagnosis -
- A. Osteosarcoma
- B. Chondrosarcoma
- C. Ewing's sarcoma (Correct Answer)
- D. Malignant fibrous histiocytoma
Bone Sarcomas Explanation: ***Ewing's sarcoma***
- **Ewing's sarcoma** commonly presents in children and adolescents, often involving the **diaphysis of long bones** like the femur.
- The presence of a **soft tissue mass** and **fever** are characteristic systemic symptoms due to its aggressive nature and rapid growth.
*Osteosarcoma*
- While it is a common pediatric bone tumor, **osteosarcoma** typically arises in the **metaphysis** of long bones, not the diaphysis.
- Systemic symptoms like fever are less common at presentation compared to Ewing's sarcoma unless there's significant metastatic disease.
*Chondrosarcoma*
- **Chondrosarcoma** is a malignant tumor of cartilage that usually affects older adults and rarely occurs in children.
- It typically affects the **pelvis, shoulder, or long bones**, but a fever and involvement of the soft tissue with a diaphyseal origin are less classic presentation.
*Malignant fibrous histiocytoma*
- **Malignant fibrous histiocytoma** (now often termed undifferentiated pleomorphic sarcoma) is a tumor of adulthood, primarily affecting individuals over 40 years of age.
- While it can involve deep soft tissues and bone, it is an extremely rare diagnosis in a 10-year-old child.
Bone Sarcomas Indian Medical PG Question 9: An 8 yr old child is having fever with pain and swelling in mid thigh. On Xray lamellated appearance and Codman's triangle is present. Histopathologic examination shows small round cells positive for MIC-2. What is the most likely diagnosis?
- A. Chondrosarcoma
- B. Chondroblastoma
- C. Ewings sarcoma (Correct Answer)
- D. Osteosarcoma
Bone Sarcomas Explanation: ***Ewings sarcoma***
- The combination of **fever with pain and swelling** in a child, **lamellated (onion-skin) periosteal reaction**, **Codman's triangle** on X-ray, and **small round cells positive for MIC-2 (CD99)** on histopathology is classic for Ewing's sarcoma.
- This highly aggressive bone tumor primarily affects children and young adults, often presenting with systemic symptoms and a diaphyseal or metadiaphyseal location in long bones.
*Chondrosarcoma*
- This tumor is characterized by the production of **cartilage matrix** and typically affects older adults (40-70 years old), not children.
- Radiographically, it often shows **calcifications with rings and arcs** within a lucent lesion, and histopathology reveals chondrocytes, not small round cells positive for MIC-2.
*Chondroblastoma*
- Chondroblastoma is a rare, **benign cartilage tumor** that typically occurs in the **epiphysis of long bones** in adolescents and young adults.
- It usually presents as a well-defined lytic lesion and does not typically show lamellated periosteal reactions or positive MIC-2 staining, nor does it commonly present with fever.
*Osteosarcoma*
- While osteosarcoma is also an aggressive bone tumor affecting children and adolescents, it is characterized by the production of **osteoid (immature bone)**.
- Radiographically, it often presents with a **"sunburst" pattern** due to spiculated periosteal reaction and a **Codman's triangle**, but histopathology shows malignant osteoblasts, not small round cells positive for MIC-2.
Bone Sarcomas Indian Medical PG Question 10: Which condition is characterized by an "onion peel" appearance on X-ray?
- A. Ewing's sarcoma (Correct Answer)
- B. Osteosarcoma
- C. Giant cell tumour
- D. Eosinophilic granuloma
Bone Sarcomas Explanation: ***Ewing's Sarcoma***
- This highly malignant bone tumor classically presents with an **\"onion peel\" appearance** on X-ray, which is caused by reactive bone formation (periosteal new bone formation) in layers.
- The layering is due to intermittent growth of the tumor, prompting repeated periosteal reactions.
*Osteosarcoma*
- Osteosarcoma often shows a **Codman triangle** (elevation of the periosteum creating a triangular shadow) or a **sunburst pattern** on X-ray, but not typically an onion peel appearance.
- It's characterized by the production of **osteoid matrix** by malignant cells.
*Giant cell tumour*
- This tumor is typically described as a **lytic lesion** with a **soap bubble appearance** on X-ray, often located in the epiphysis of long bones.
- It is composed of multinucleated giant cells and is usually benign but can be locally aggressive.
*Eosinophilic granuloma*
- Eosinophilic granuloma presents as a **lytic lesion** with sharply defined borders, sometimes described as a **\"punched-out\" lesion** on X-ray, particularly in the skull.
- It is a benign proliferation of Langerhans cells and eosinophils, usually affecting children and young adults.
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