Bone Tumors — MCQs

10 questions

Ganga Ram, a 19-year-old male with a short history of pain in the right groin that worsens at night time and has a small circumscribed sclerotic swelling over diaphysis of femur. Likely diagnosis is:

Which of the following is not true about osteosarcoma?

A 13-year-old boy, previously healthy, presents with a 1-month history of right leg pain, without trauma or recent illness. Physical examination reveals warmth, tenderness, and increased circumference of the right thigh compared to the left. His temperature is 39°C. A radiograph shows a 6-cm expansile mass in the diaphyseal region of the right lower femur, extending into soft tissue and covered by reactive bone. Microscopic examination of the mass biopsy reveals sheets of primitive cells with small, uniform nuclei and scant cytoplasm. Karyotypic analysis shows a t(11;22) translocation. What is the most likely diagnosis?

Which statement is incorrect about the pathology of the bone tumor?

Most common benign tumor of bone?

A 12-year-old Caucasian male presents with his mother to the pediatrician’s office complaining of right thigh pain. He reports that he has noticed slowly progressive pain and swelling over the distal aspect of his right thigh over the past two months. He denies any recent trauma to the area and his temperature is 100.9°F (38.3°C). On exam, there is swelling and tenderness overlying the distal right femoral diaphysis. Laboratory evaluation is notable for an elevated white blood cell (WBC) count and elevated erythrocyte sedimentation rate (ESR). A radiograph of the patient’s right leg is shown. Biopsy of the lesion demonstrates sheets of monotonous small round blue cells with minimal cytoplasm. Which of the following genetic mutations is most likely associated with this patient’s condition?

Amount of ORS to be given in the first 4 hours to a child with some dehydration is

A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

A 6 year old child presents with an abdominal mass, fever, bone pain and IVC thrombosis, the diagnosis could be -

Q10

A 2 year old child is seen on a routine visit in the pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month's visit for immunisation. Physical examination is otherwise unremarkable. If a CT guided biopsy of the mass were performed, which of the following histological patterns would be most suggestive of the likely diagnosis?

Bone Tumors Indian Medical PG Practice Questions and MCQs

Question 1: Ganga Ram, a 19-year-old male with a short history of pain in the right groin that worsens at night time and has a small circumscribed sclerotic swelling over diaphysis of femur. Likely diagnosis is:

A. Osteosarcoma
B. Ewing's sarcoma
C. Osteoclastoma
D. Osteoid osteoma (Correct Answer)

Explanation: ***Osteoid osteoma*** - This benign bone tumor classically presents with **nocturnal pain** that is relieved by **NSAIDs**, a key differentiating feature not mentioned but highly characteristic. - The description of a **small, circumscribed sclerotic swelling** over the femoral diaphysis is consistent with the typical radiographic appearance of an osteoid osteoma. *Osteosarcoma* - This is a highly malignant primary bone tumor that typically presents with **progressive, severe pain** and a rapidly growing mass, often in the metaphysis of long bones. - Radiographically, it often shows a **sunburst appearance** or **Codman's triangle**, and is not typically described as a small, circumscribed sclerotic lesion. *Ewing's sarcoma* - This aggressive bone tumor commonly affects children and young adults, presenting with severe pain, swelling, and systemic symptoms like fever. - Radiographically, it often exhibits an **onion-skin periosteal reaction** and lytic lesions, which differs from a small, sclerotic swelling. *Osteoclastoma* - Also known as a **giant cell tumor of bone**, this tumor typically affects the **epiphysis** of long bones in young adults, causing pain and swelling. - Radiographically, it appears as a **lytic, expansile lesion** without significant sclerosis, making it inconsistent with the described circumscribed sclerotic swelling.

Question 2: Which of the following is not true about osteosarcoma?

A. Seen in the metaphyseal region of the long bones
B. Lung metastasis is common
C. Secondary osteosarcoma is seen in older age groups
D. Most commonly arises in the epiphyseal region (Correct Answer)

Explanation: ***Most commonly arises in the epiphyseal region*** - This statement is **FALSE** - osteosarcoma most commonly arises in the **metaphyseal region** of long bones, particularly around the knee (distal femur, proximal tibia) and proximal humerus [1]. - The metaphysis is the region where bone growth is most active, which explains why osteosarcoma preferentially occurs there. - The epiphysis (growth plate region) is **not** the typical location for osteosarcoma. *Seen in the metaphyseal region of the long bones* - This is **TRUE** - osteosarcoma characteristically arises in the **metaphyseal regions** of long bones, especially around the knee and proximal humerus where growth is most active [1]. *Lung metastasis is common* - This is **TRUE** - the lungs are the most common site of distant metastasis in osteosarcoma, occurring in up to 80% of patients who develop metastatic disease [1]. - Pulmonary metastasis significantly impacts prognosis and treatment [1]. *Secondary osteosarcoma is seen in older age groups* - This is **TRUE** - while primary osteosarcoma affects children and young adults (peak 10-20 years), **secondary osteosarcoma** occurs in older patients, typically arising in association with Paget's disease, prior radiation therapy, or bone infarcts [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1200-1202.

Question 3: A 13-year-old boy, previously healthy, presents with a 1-month history of right leg pain, without trauma or recent illness. Physical examination reveals warmth, tenderness, and increased circumference of the right thigh compared to the left. His temperature is 39°C. A radiograph shows a 6-cm expansile mass in the diaphyseal region of the right lower femur, extending into soft tissue and covered by reactive bone. Microscopic examination of the mass biopsy reveals sheets of primitive cells with small, uniform nuclei and scant cytoplasm. Karyotypic analysis shows a t(11;22) translocation. What is the most likely diagnosis?

A. Ewing sarcoma (Correct Answer)
B. Chondrosarcoma
C. Giant cell tumor
D. Metastatic carcinoma

Explanation: ***Ewing sarcoma*** - The patient's age (13-year-old), symptoms (bone pain, systemic fever), **diaphyseal location** of the tumor, and especially the characteristic histological finding of **small, uniform cells** (small round blue cell tumor) with **scant cytoplasm** point towards Ewing sarcoma. - The presence of **t(11;22)(q24;q12) translocation** resulting in the **EWSR1-FLI1 fusion gene** is a definitive diagnostic marker for Ewing sarcoma (present in ~85% of cases), distinguishing it from other bone tumors. *Chondrosarcoma* - This tumor typically affects older adults (>40 years) and arises from cartilage, showing cartilaginous matrix on histology. - It lacks the specific t(11;22) translocation seen in this patient. *Giant cell tumor* - Giant cell tumors typically occur in young adults (20-40 years) and are usually found in the **epiphyseal and metaphyseal regions** of long bones. - Histologically, they contain numerous **multinucleated giant cells** interspersed with mononuclear stromal cells, not sheets of primitive cells. *Metastatic carcinoma* - Metastatic carcinoma to bone is rare in children and usually occurs in older adults with a known primary cancer. - The histology of "sheets of primitive cells with small, uniform nuclei and scant cytoplasm" is not typical for metastatic carcinoma.

Question 4: Which statement is incorrect about the pathology of the bone tumor?

A. Tumor has distinct margin
B. Tumor arises from epiphyseal to metaphyseal region
C. Eccentric lesion
D. Chemotherapy is the treatment of choice for all bone tumors. (Correct Answer)

Explanation: ***Tumor has distinct margin*** - A **distinct margin** often indicates a benign tumor, while malignant tumors typically show **infiltrative margins**. - In bone tumors, particularly malignant ones, the lack of clear demarcation is a key pathological feature. *Chemotherapy is the treatment of choice* - While chemotherapy may be used for certain **malignant bone tumors**, it is not the first-line treatment for most bone tumors [1]. - The primary treatment is often **surgical excision**, especially for localized lesions [1]. *Tumor arise from epiphyseal to metaphyseal region* - While some tumors can originate in these areas, many actually arise from the **diaphyseal** region in bone tumors like osteosarcoma. - This option misrepresents the common locations where various tumors develop, as osteochondromas tend to develop near the epiphyses of limb bones [2]. *Eccentric lesion* - Many bone tumors do indeed present as **eccentric lesions**, especially benign ones like **osteochondromas**. - However, this feature does not apply universally, as some malignant tumors can also be **central or infiltrative** in nature. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 673-674. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 672-673.

Question 5: Most common benign tumor of bone?

A. Osteoma
B. Simple bone cyst
C. Osteochondroma (Correct Answer)
D. Osteoid osteoma

Explanation: ***Osteochondroma*** - This is the **most common benign bone tumor**, characterized by a bony spur with a cartilaginous cap. - It typically arises from the **metaphysis of long bones**, especially around the knee. *Osteoma* - Osteomas are **benign, slow-growing tumors** composed of mature compact or cancellous bone. - They are most commonly found in the **skull and facial bones**, not typically in long bones. *Simple bone cyst* - This is a **fluid-filled lesion** of bone, not a true neoplasm, frequently found in the metaphysis of long bones in children. - It is often discovered incidentally or after a **pathological fracture**. *Osteoid osteoma* - Characterized by a **small, benign bone tumor** with a central nidus of osteoid and trabecular bone, surrounded by reactive sclerotic bone. - It classically causes **nocturnal pain** that is relieved by NSAIDs.

Question 6: A 12-year-old Caucasian male presents with his mother to the pediatrician’s office complaining of right thigh pain. He reports that he has noticed slowly progressive pain and swelling over the distal aspect of his right thigh over the past two months. He denies any recent trauma to the area and his temperature is 100.9°F (38.3°C). On exam, there is swelling and tenderness overlying the distal right femoral diaphysis. Laboratory evaluation is notable for an elevated white blood cell (WBC) count and elevated erythrocyte sedimentation rate (ESR). A radiograph of the patient’s right leg is shown. Biopsy of the lesion demonstrates sheets of monotonous small round blue cells with minimal cytoplasm. Which of the following genetic mutations is most likely associated with this patient’s condition?

A. t(11;22) (Correct Answer)
B. RB1 inactivation
C. TP53 inactivation
D. t(8;14)
E. t(X;18)

Explanation: ***t(11;22)*** - The clinical presentation of a **12-year-old male** with progressive **thigh pain and swelling**, fever, elevated WBC and ESR, a radiograph showing a bone lesion [1], and a biopsy revealing **small round blue cells with minimal cytoplasm**, is highly suggestive of **Ewing sarcoma** [2]. - **Ewing sarcoma** is characterized by the **t(11;22)(q24;q12) chromosomal translocation**, which fuses the *EWSR1* gene with the *FLI1* gene, leading to the formation of a chimeric transcription factor. *t(X;18)* - The **t(X;18) translocation** is the characteristic genetic abnormality of **synovial sarcoma**, another soft tissue malignancy. - While synovial sarcoma can also present in young patients, it typically affects older adolescents and young adults, and the histology differs from the small round blue cell pattern seen in Ewing sarcoma [2]. *RB1 inactivation* - **RB1 gene inactivation** is centrally involved in the pathogenesis of **retinoblastoma**, a childhood eye cancer. - It also plays a role in various other cancers, such as **osteosarcoma** [3] and small cell lung cancer, but its primary association is not with Ewing sarcoma. *TP53 inactivation* - **TP53 gene inactivation** is a common event in a wide range of human cancers, as *TP53* is a critical **tumor suppressor gene**. - While *TP53* mutations can be found in some sarcomas, it is not the defining or most likely specific genetic mutation for **Ewing sarcoma**. *t(8;14)* - The **t(8;14)(q24;q32) chromosomal translocation** is the characteristic genetic abnormality found in **Burkitt lymphoma**. - This translocation leads to the **c-MYC proto-oncogene** being placed near the immunoglobulin heavy chain locus, promoting its overexpression, which is unrelated to Ewing sarcoma. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 671-672. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1204-1205. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1200-1202.

Question 7: Amount of ORS to be given in the first 4 hours to a child with some dehydration is

A. 75 ml/kg body wt. (Correct Answer)
B. 50 ml/kg body wt.
C. 100 ml/kg body wt.
D. 200 ml/kg body wt.

Explanation: ***75 ml/kg body wt.*** - For a child with **some dehydration**, the World Health Organization (WHO) and UNICEF recommend administering **75 mL/kg body weight** of ORS over the first 4 hours as part of Plan B. - This volume is calculated to replenish lost fluids and electrolytes, addressing the estimated fluid deficit in **some dehydration**. *50 ml/kg body wt.* - A dose of **50 mL/kg** is generally insufficient for effective rehydration in a child presenting with **some dehydration**. - This amount might be used in milder cases or for maintenance, but not for initial rehydration in the **first 4 hours** with signs of dehydration. *100 ml/kg body wt.* - Administering **100 mL/kg** body weight is typically used for **severe dehydration** (Plan C) when given as **intravenous fluids**. - For **some dehydration** treated with ORS, the recommended dose is 75 mL/kg, not 100 mL/kg. This higher amount could lead to fluid overload if given orally in the first 4 hours. *200 ml/kg body wt.* - A dose of **200 mL/kg** body weight is excessive and potentially dangerous for a child with **some dehydration**. - Such a large volume could lead to **fluid overload**, electrolyte imbalances, and other complications, especially in young children.

Question 8: A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

A. Wilms tumor
B. Pheochromocytoma
C. Neuroblastoma (Correct Answer)
D. Medulloblastoma

Explanation: ***Neuroblastoma*** - The combination of a **flank mass** in a 1-year-old child, along with paraneoplastic symptoms like **flushing, diarrhea, and sweating** (due to catecholamine secretion), and **bone pain** (indicating metastatic disease), is highly characteristic of neuroblastoma. - Neuroblastoma is a common extracranial solid tumor of childhood arising from **neural crest cells**, often presenting with abdominal mass and systemic symptoms. *Wilms tumor* - While Wilms tumor also presents as a **flank mass** in young children, it typically does not cause the systemic symptoms of flushing, diarrhea, or sweating, nor significant bone pain from metastasis. - Patients with Wilms tumor often present with **asymptomatic abdominal mass** detected incidentally. *Pheochromocytoma* - **Pheochromocytoma** can cause symptoms like flushing, sweating, and episodic hypertension due to catecholamine release, but it is **rare in children** and typically presents as an adrenal mass, not a flank mass with bone pain. - It usually presents with **hypertension crises**, which are not mentioned here. *Medulloblastoma* - **Medulloblastoma** is a **brain tumor** and would present with neurological symptoms such as headache, vomiting, and ataxia, not a flank mass or systemic symptoms like flushing, diarrhea, and sweating. - It is a **malignant tumor** of the cerebellum and does not cause peripheral masses or bone metastases in this manner.

Question 9: A 6 year old child presents with an abdominal mass, fever, bone pain and IVC thrombosis, the diagnosis could be -

A. Neuroblastoma (Correct Answer)
B. Gastric lymphoma
C. Wilm's tumour
D. Langerhans cell Histiocytosis

Explanation: ***Neuroblastoma*** - **Neuroblastoma** commonly presents with an **abdominal mass** and can metastasize to bones, causing **bone pain** and systemic symptoms like **fever**. - A large abdominal mass, particularly in the retroperitoneum, can compress the **inferior vena cava (IVC)**, leading to **thrombosis**. *Gastric lymphoma* - **Gastric lymphoma** primarily affects the stomach and is less likely to present with widespread **bone pain** or systemic **fever** in a young child as the initial symptoms. - While an abdominal mass can occur, **IVC thrombosis** due to gastric lymphoma compression is rare in children and less typical than with retroperitoneal tumors. *Wilm's tumour* - **Wilms' tumor** is a common pediatric renal tumor causing an **abdominal mass**, but **bone pain** as a primary presenting symptom or widespread metastases contributing to fever is less typical. - While it can extend into the **renal vein** and **IVC**, significant **IVC thrombosis** leading to systemic symptoms is less common compared to neuroblastoma's broad presentation. *Langerhans cell Histiocytosis* - **Langerhans cell Histiocytosis (LCH)** can cause **bone pain** and **fever** due to bone lesions, and it can involve many organs. - However, a discrete, large **abdominal mass** and **IVC thrombosis** are not characteristic primary presentations of LCH.

Question 10: A 2 year old child is seen on a routine visit in the pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month's visit for immunisation. Physical examination is otherwise unremarkable. If a CT guided biopsy of the mass were performed, which of the following histological patterns would be most suggestive of the likely diagnosis?

A. Triphasic pattern with tubule formation, spindle cells, and blastemal elements (Correct Answer)
B. Invasive papillary lesions with delicate connective tissue stalk covered with epithelium resembling that lining the bladder.
C. Cords of clear cells with rounded or polygonal shape and abundant clear cytoplasm
D. Small dark cells embedded in a finely fibrillar matrix with formation of numerous rosettes

Explanation: ***Triphasic pattern with tubule formation, spindle cells, and blastemal elements*** - The presence of a **palpable, non-tender abdominal mass** in a 2-year-old, previously undetected, is highly suggestive of **Wilms tumor (nephroblastoma)**. - Wilms tumor characteristically exhibits a **triphasic histological pattern** consisting of **blastemal**, **stromal (spindle cells)**, and **epithelial (tubule formation)** elements. *Invasive papillary lesions with delicate connective tissue stalk covered with epithelium resembling that lining the bladder.* - This description typically refers to **papillary urothelial carcinoma**, a tumor of the **bladder or urinary tract**, which is rarely seen in young children and presents with hematuria, not primarily an abdominal mass. - The absence of urinary symptoms and the age of the patient make this an unlikely diagnosis for an abdominal mass. *Cords of clear cells with rounded or polygonal shape and abundant clear cytoplasm* - This histological pattern is characteristic of **clear cell renal carcinoma**, an adult kidney malignancy, not a common pediatric abdominal tumor. - While clear cell renal cell carcinoma can present as an abdominal mass, it is exceptionally rare in a 2-year-old child. *Small dark cells embedded in a finely fibrillar matrix with formation of numerous rosettes* - This description is highly suggestive of **neuroblastoma**, a small round blue cell tumor that arises from neural crest cells (e.g., adrenal gland or sympathetic chain). - While **neuroblastoma** is the second most common abdominal mass in children after Wilms tumor, it typically presents with different histological features and may be associated with systemic symptoms like weight loss, fever, or bone pain. - The **triphasic pattern** described in option A is more specific for Wilms tumor.

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