Bone Tumors Practice Questions

Q: Maffucci syndrome:

Multiple enchondromatosis with hemangiomas. ***Multiple enchondromatosis with hemangiomas*** - **Maffucci syndrome** is a rare, non-hereditary disorder characterized by the presence of multiple **enchondromas** (benign cartilaginous tumors) and **hemangiomas** (benign vascular tumors). - The enchondromas typically affect the long bones and can lead to skeletal deformities and fractures, while the hemangiomas can be soft tissue or visceral. *Multiple osteochondromas* - This description typically refers to **multiple hereditary exostoses** (MHE), a distinct genetic condition. - In MHE, the bone lesions are **osteochondromas**, which are bone spurs capped with cartilage, rather than enchondromas that form within the bone. *Multiple osteochondromatosis with hemangiomas* - This accurately describes the presence of **hemangiomas** but identifies the bone lesions as **osteochondromas** instead of enchondromas. - The presence of osteochondromas would point towards a different diagnosis like MHE, even with co-occurring hemangiomas. *Multiple Giant cell tumor* - **Giant cell tumors (GCTs)** are generally solitary, benign, but locally aggressive bone tumors, not typically seen as multiple lesions in the context of a syndrome like Maffucci. - They also differ histologically and clinically from enchondromas.

Q: Most common site of adamantinoma of the long bones is -

Tibia. ***Tibia*** - Adamantinoma is a rare, malignant bone tumor that has a strong predilection for the **tibia**, accounting for approximately 85-90% of cases in long bones. - It most commonly occurs in the **diaphysis of the tibia** but can also be found in the metaphysis. *Ulna* - While adamantinoma can rarely affect other long bones, the **ulna is not a common site** for its occurrence. - The incidence of adamantinoma in the upper extremities, including the ulna, is significantly lower compared to the tibia. *Fibula* - The **fibula is an uncommon site** for adamantinoma, with only a small percentage of cases reported in this bone. - When it does occur in the fibula, it usually involves the mid-diaphysis. *Femur* - The **femur is also a rare location** for adamantinoma, with only a handful of cases documented in medical literature. - The vast majority of adamantinomas consistently originate in the lower leg, particularly the tibia.

Q: A young girl presented with swelling of right thigh, with history of trauma 2 months back. Now she presents with swelling at mid-shaft of femur & low grade fever. ESR is mildly raised. X-ray shows a laminated periosteal reaction. Next line of investigation would be:

MRI. ***MRI*** - An **MRI** is the most appropriate next step as it provides excellent detailed imaging of soft tissues and bone marrow, allowing better characterization of the **periosteal reaction**, identifying abscesses, and assessing the extent of bone involvement, crucial for differentiating between infection and tumor. - The presence of a **laminated periosteal reaction** (like an "onion peel") on X-ray, in conjunction with localized swelling and low-grade fever, is highly suggestive of subacute osteomyelitis or even some bone tumors like Ewing sarcoma, for which MRI is superior for defining the extent. *Bone scan* - A **bone scan** (technetium-99m) is sensitive for detecting increased bone turnover, which occurs in infections and tumors, but it is **non-specific**, meaning it cannot differentiate between these conditions. - While it could show increased uptake in the affected area, it would not provide the anatomical detail needed to characterize the lesion or guide further management as effectively as an MRI. *Blood count & CRP* - A **blood count and CRP** would provide information on systemic inflammation (e.g., leukocytosis, elevated CRP for infection), but these are **non-specific**. - While ESR is already mildly raised, these blood tests would confirm generalized inflammation but **cannot localize or characterize the lesion** in the bone, offering little diagnostic value for the specific problem at this stage without imaging. *Biopsy* - A **biopsy** is an invasive procedure and is typically performed after initial imaging studies like X-ray and MRI have characterized the lesion to guide the biopsy site and help determine the nature of the condition (e.g., infection vs. tumor). - Performing a biopsy as the immediate next step without detailed imaging to assess the extent and nature of the lesion would be premature and potentially less effective in diagnosis.

Q: What is the most common site of osteosarcoma:

Lower end of femur. ***Lower end of femur*** - The **distal femur** is the most common site for osteosarcoma, particularly in the **metaphyseal regions** where rapid growth occurs. - This area, along with the proximal tibia, accounts for a significant majority of all osteosarcoma cases. *Lower end of tibia* - While the **proximal tibia** is a common site, the **distal tibia** is less frequently affected by osteosarcoma compared to the distal femur. - The incidence in the distal tibia is notably lower than in the knee region. *Upper end of femur* - The **proximal femur** is a recognized site for osteosarcoma, but it is less common than the **distal femur**. - Tumors in this location can be challenging to manage due to their proximity to major neurovascular structures. *Upper end of humerus* - The **proximal humerus** is another common location for osteosarcoma, accounting for a notable percentage of cases. - However, its incidence is still lower than that of the **distal femur**.

Q: Which is the most common clinical feature of Ewing's sarcoma?

Pain. ***Pain*** - **Localized pain**, often increasing in intensity over time, is the most consistent and often the initial symptom of **Ewing's sarcoma**. - This pain is typically worse at night and may be mistaken for injury or other benign conditions in its early stages [1]. *Fever* - **Fever** can be a systemic symptom in Ewing's sarcoma, indicating inflammation or metastatic disease. - However, it is less common than pain as the initial presenting symptom and is not present in all cases. *Limp* - A **limp** frequently occurs when the tumor affects skeletal elements of the lower extremities or pelvis, leading to mechanical compromise or pain with weight-bearing. - While common, it is a secondary symptom often resulting from underlying pain or a mass, rather than the primary presenting feature. *Palpable mass* - A **palpable mass** is a significant finding in Ewing's sarcoma, especially as the tumor grows and expands beyond the bone cortex. - However, the mass is often preceded by localized pain and may not be detectable in deeply situated tumors until late stages. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 660-661.

Q: An 11 year old boy presented with the complaints of pain in the right arm near the shoulder. X-ray examination revealed an expansile lytic lesion in the upper third of humerus. The most likely diagnosis is

Unicameral bone cyst. ***Unicameral bone cyst*** - **Unicameral bone cysts (UBCs)** are common in children and typically present as **expansile lytic lesions** in the metaphysis of long bones, most frequently the **proximal humerus** or femur. - The patient's age (11 years old) and the **radiographic finding** of an expansile lytic lesion in the upper humerus are highly characteristic of a UBC, which often causes pain due to microfractures. *Osteochondroma* - **Osteochondromas** are **benign bone tumors** that appear as **bony outgrowths** or exostoses, usually arising from the metaphysis near the growth plate. - They are typically **pedunculated or sessile** and have a cortical and medullary continuity with the underlying bone, which is different from a lytic lesion. *Parosteal osteosarcoma* - **Parosteal osteosarcoma** is a **low-grade malignant bone tumor** that originates from the outer surface of the bone, rather than being purely intramedullary. - It usually presents as a **densely calcified mass** attached to the cortex of long bones and is more common in adolescents and young adults, but less likely to manifest as a purely lytic expansile lesion. *Giant cell tumor* - **Giant cell tumors (GCTs)** are typically found in the **epiphysis and metaphysis of long bones**, particularly around the knee, and are more common in adults (20-40 years old). - While they can be lytic and expansile, GCTs are **rare in pre-adolescents** like an 11-year-old boy and often demonstrate a characteristic "soap bubble" appearance on X-ray in older patients.

Q: Which of the following is an epiphyseal tumor?

Chondroblastoma. ***Chondroblastoma*** - **Chondroblastoma** is a rare, benign cartilaginous tumor that typically originates in the **epiphysis** of long bones before the closure of growth plates. - It is histologically characterized by chondroblast-like cells, multinucleated giant cells, and chondroid matrix, and radiographically appears as a lytic lesion with a sclerotic rim in the epiphysis. *Osteosarcoma* - **Osteosarcoma** is the most common primary malignant bone tumor and typically originates in the **metaphysis** of long bones, particularly around the knee. - It invades the surrounding bone and soft tissues, often presenting with a **Codman triangle** or **sunburst pattern** on imaging studies. *Osteoid osteoma* - **Osteoid osteoma** is a benign bone-forming tumor primarily found in the **cortex** of long bones, although it can occur in other locations, presenting with nocturnal pain relieved by NSAIDs. - It is characterized by a central radiolucent nidus surrounded by reactive sclerotic bone. *Adamantinoma* - **Adamantinoma** is an extremely rare, low-grade malignant tumor that almost exclusively occurs in the **tibia diaphysis**. - It is thought to originate from epithelial cells and presents as a lytic lesion within the cortical bone, often with a polycystic appearance.

Q: A 7-year-old child presents with a lesion in upper tibia. X-ray shows radiolucent area with Codman's triangle and sunray appearance. Diagnosis is

Osteosarcoma. ***Osteosarcoma*** - The classic radiographic features of **Codman's triangle** (periosteal elevation) and **sunray appearance** (spiculated periosteal reaction) are highly characteristic of osteosarcoma. - This tumor commonly affects the **metaphysis of long bones** in children and young adults, with the distal femur and proximal tibia being frequent sites. *Osteoid Osteoma* - This is a **benign bone tumor** characterized radiographically by a small radiolucent **nidus** surrounded by a rim of dense sclerosis. - It does not present with Codman's triangle or sunray appearance and typically causes **nocturnal pain relieved by NSAIDs**. *Ewing sarcoma* - Ewing sarcoma often presents with an **"onion skin" periosteal reaction** (layers of new bone formation) due to its aggressive nature. - While it can be destructive and radiolucent, it typically does not classically exhibit the sunray appearance or a distinct Codman's triangle as prominently as osteosarcoma. *Chondrosarcoma* - Chondrosarcoma is a **malignant cartilaginous tumor** that usually affects older adults more commonly than children. - Radiographically, it often shows a **lobulated appearance** with **punctate or ring-and-arc calcifications** within the lesion, not the sunray or Codman's triangle findings.

Q: All of the following are true about Ewing's sarcoma, EXCEPT

Common in 4th decade. ***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.

Question 1

Maffucci syndrome:

Accepted Answer

Multiple enchondromatosis with hemangiomas

Answer

Multiple osteochondromas

Answer

Multiple osteochondromatosis with hemangiomas

Answer

Multiple Giant cell tumor

Question 2

Most common site of adamantinoma of the long bones is -

Accepted Answer

Tibia

Answer

Ulna

Answer

Fibula

Answer

Femur

Question 3

A young girl presented with swelling of right thigh, with history of trauma 2 months back. Now she presents with swelling at mid-shaft of femur & low grade fever. ESR is mildly raised. X-ray shows a laminated periosteal reaction. Next line of investigation would be:

Accepted Answer

MRI

Answer

Bone scan

Answer

Blood count & CRP

Answer

Biopsy

Question 4

What is the most common site of osteosarcoma:

Accepted Answer

Lower end of femur

Answer

Lower end of tibia

Answer

Upper end of femur

Answer

Upper end of humerus

Question 5

Which is the most common clinical feature of Ewing's sarcoma?

Accepted Answer

Pain

Answer

Fever

Answer

Limp

Answer

Palpable mass

Question 6

An 11 year old boy presented with the complaints of pain in the right arm near the shoulder. X-ray examination revealed an expansile lytic lesion in the upper third of humerus. The most likely diagnosis is

Accepted Answer

Unicameral bone cyst

Answer

Osteochondroma

Answer

Parosteal osteosarcoma

Answer

Giant cell tumor

Question 7

Which of the following is an epiphyseal tumor?

Accepted Answer

Chondroblastoma

Answer

Osteosarcoma

Answer

Osteoid osteoma

Answer

Adamantinoma

Question 8

A 7-year-old child presents with a lesion in upper tibia. X-ray shows radiolucent area with Codman's triangle and sunray appearance. Diagnosis is

Accepted Answer

Osteosarcoma

Answer

Osteoid Osteoma

Answer

Ewing sarcoma

Answer

Chondrosarcoma

Question 9

All of the following are true about Ewing's sarcoma, EXCEPT

Accepted Answer

Common in 4th decade

Answer

Responds well to radiotherapy

Answer

Grows in diaphysis of long bones

Answer

Has onion peel appearance on radiograph

Question 10

Ewing's sarcoma is a PNET (Primitive Neuroectodermal Tumor) that develops in the diaphysis of a long bone. A child with Ewing's sarcoma is on radiotherapy and chemotherapy. Which of the following is a poor prognostic factor in Ewing's sarcoma?

Accepted Answer

Large tumor size (>8cm)

Answer

Age >15 years

Answer

Younger age

Answer

Axial/pelvic location

Bone Tumors — MCQs

Bone Tumors — MCQs

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