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Question 481

Topic: 10. Pathology and Oncology

Which of the following cell types is not typically found in giant cell tumors of tendon sheath:

. Multinucleated giant cells
. Histiocytes
. Monocytes
. Polymorphonuclear lymphocytes
. Fibroblasts

Correct Answer & Explanation

. Polymorphonuclear lymphocytes


Explanation

Multinucleated giant cells, histiocytes, monocytes, and fibroblasts are commonly found in pathologic giant cell tumor of tendon sheath specimens. Polymorphonuclear lymphocytes are typically associated with bacterial infections.

Question 482

Topic: Bone Tumors

A 19-year-old male presents with severe, aching pain in his mid-tibia that classically worsens at night. He reports complete relief of symptoms within 30 minutes of taking aspirin. Radiographs reveal a cortical lucency surrounded by sclerosis. The central nidus of this lesion predominantly secretes high levels of which biochemical mediator?

. Interleukin-6
. Tumor Necrosis Factor-alpha
. Prostaglandin E2
. Leukotriene B4
. Substance P

Correct Answer & Explanation

. Prostaglandin E2


Explanation

The clinical presentation is classic for an osteoid osteoma. The central nidus produces a high concentration of Prostaglandin E2 (PGE2), which causes severe pain that is exquisitely sensitive to COX inhibitors like aspirin and NSAIDs.

Question 483

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a destructive metaphyseal lesion of the distal femur with a sunburst periosteal reaction. Biopsy confirms conventional osteosarcoma. Following neoadjuvant chemotherapy, what is the most significant prognostic factor for long-term survival?

. Histologic subtype of the tumor
. Absolute tumor volume at initial presentation
. Percentage of tumor necrosis found in the resected specimen
. Serum alkaline phosphatase levels post-resection
. The specific type of surgical resection performed (amputation vs. limb salvage)

Correct Answer & Explanation

. Percentage of tumor necrosis found in the resected specimen


Explanation

The histological response to neoadjuvant chemotherapy, defined by the percentage of tumor necrosis in the resected specimen, is the single most important prognostic indicator for survival in localized osteosarcoma (>90% necrosis indicates a good responder).

Question 484

Topic: 10. Pathology and Oncology

A 32-year-old woman presents with knee pain. Radiographs show an eccentric, lytic epiphyseal lesion in the distal femur. Biopsy reveals mononuclear cells and multinucleated giant cells.

What is the most appropriate definitive management?

. Wide resection and endoprosthesis
. Extended intralesional curettage with local adjuvant and bone grafting/cementing
. Radiation therapy
. Chemotherapy followed by resection
. Amputation

Correct Answer & Explanation

. Extended intralesional curettage with local adjuvant and bone grafting/cementing


Explanation

The clinical and radiographic picture suggests a Giant Cell Tumor of bone. Extended intralesional curettage using a high-speed burr and adjuvants (like phenol or liquid nitrogen) followed by PMMA or bone graft is the standard treatment.

Question 485

Topic: 10. Pathology and Oncology

A 12-year-old boy presents with progressive knee pain and swelling. Radiographs show a destructive, permeative metaphyseal lesion in the distal femur with a "sunburst" periosteal reaction and Codman's triangle. Histological examination reveals malignant spindle cells producing osteoid matrix. What is the most likely diagnosis?

. Ewing sarcoma
. Osteosarcoma
. Chondrosarcoma
. Osteomyelitis
. Non-ossifying fibroma

Correct Answer & Explanation

. Osteosarcoma


Explanation

Osteosarcoma classically presents in the metaphysis of long bones in adolescents with a sunburst periosteal reaction and Codman's triangle. The histological hallmark is the production of malignant osteoid by the tumor cells.

Question 486

Topic: 10. Pathology and Oncology

A 60-year-old man presents with progressive deep pelvic pain. Radiographs reveal a large, lobulated lytic lesion in the ilium with "popcorn" stippled calcifications. Biopsy demonstrates a hyaline cartilage matrix with atypical chondrocytes. What is the mainstay of treatment for conventional low-grade chondrosarcoma of the pelvis?

. Neoadjuvant chemotherapy followed by wide surgical excision
. Radiation therapy alone
. Wide surgical excision alone
. Intralesional curettage and bone grafting
. Observation with serial MRI

Correct Answer & Explanation

. Wide surgical excision alone


Explanation

Conventional chondrosarcoma is notoriously resistant to both chemotherapy and radiation. Wide surgical excision with negative margins is the definitive treatment and offers the only chance for a cure.

Question 487

Topic: 10. Pathology and Oncology

The most common bone tumor of the upper extremity is:

. Enchondroma
. Osteoblastoma
. Osteochondroma
. Giant cell tumor
. C hondromyxoid tumor

Correct Answer & Explanation

. Osteochondroma


Explanation

Osteochondromas are the most common primary benign bony tumors.

Question 488

Topic: 10. Pathology and Oncology

The most common benign bone tumor of the hand is:

. Enchondroma
. Osteoblastoma
. Osteochondroma
. Giant cell tumor
. C hondromyxoid tumor

Correct Answer & Explanation

. Enchondroma


Explanation

Unlike the entire upper extremity, enchondromas are the most common tumors of the hand.

Question 489

Topic: 10. Pathology and Oncology
Osteochondromas are benign but can have a malignant transformation in which of the following cases:
. Diaphyseal achalasia
. Ollier's disease
. Osteochondromatosis malignant transformans
. Osteochondroma larger than 5 cm
. Maffucci's syndrome

Correct Answer & Explanation

. Diaphyseal achalasia


Explanation

Diaphyseal achalasia, also known as multiple hereditary exostoses, has a risk of malignant degeneration in up to 25% of patients. Ollier's disease and Maffucci's syndrome are associated with enchondromas. There is no lesion called an osteochondromatosis malignant transformans.

Question 490

Topic: 10. Pathology and Oncology

The risk of malignant transformation in patients with multiple hereditary exostoses is:

. 0%
. Less than 1%
. 1% to 2%
. Greater than 5%
. 0.5% to 25%

Correct Answer & Explanation

. 0.5% to 25%


Explanation

The rate of malignant transformation in patients with multiple hereditary exostoses is variable and is generally reported between 0.5% to 25%.

Question 491

Topic: 10. Pathology and Oncology

Recurrence of osteochondroma is likely if:

. The cartilage cap is incompletely excised
. The overlying bursa is incompletely excised
. The bony stalk is incompletely excised
. The tumor is incompletely excised
. Its connection with the medullary canal is not obliterated

Correct Answer & Explanation

. The cartilage cap is incompletely excised


Explanation

The cartilaginous portion of an osteochondroma is the neoplastic part; its complete excision is essential to avoid recurrences.

Question 492

Topic: 10. Pathology and Oncology

Malignant transformation of osteochondroma commonly occurs to:

. High-grade osteosarcoma
. High-grade chondrosarcoma
. Low-grade chondrosarcoma
. Low-grade osteosarcoma
. Parosteal osteosarcoma

Correct Answer & Explanation

. Low-grade chondrosarcoma


Explanation

Osteochondroma is a cartilaginous tumor and malignant transformation is to a low-grade chondrosarcoma.

Question 493

Topic: 10. Pathology and Oncology

All of the following suggest a possibility of malignant transformation in multiple hereditary exostoses except:

. Recent onset of pain
. Growth after skeletal maturity
. C artilaginous cap thickness greater than 3 cm
. Soft tissue extension
. C alcific stippling in the cap on radiograph

Correct Answer & Explanation

. C alcific stippling in the cap on radiograph


Explanation

Stippling on radiographs in the cap is due to calcification and is a common characteristic of cartilaginous tumors.

Question 494

Topic: Bone Tumors
A 12-year-old boy is brought to the clinic by his concerned parents. The boy's forearm is bowed, and his parents are confused as to the possible diagnosis and treatment options. You notice that the right forearm of the child is bowed ulnarwards and is shorter compared to the left forearm. The pronosupination is markedly decreased on the right side but is also limited on the left side. The patient has a good grip, pinch, and grasp. He is neurologically intact as well. The parents say that they first noticed the deformity around 6 or 7 years ago, and the mother informs you that she had noticed a hard bump on the forearm. She has recently noticed another bump on his right leg. The child does not complain of pain and is using both of his hands quite well. The parents were informed by a previous physician that the child has Madelung's deformity and are concerned that the disease is now involving other areas of his body. You order a radiograph of the forearm. The anteroposterior radiograph is shown (Slide 1). The child's skeletal radiograph survey is also presented (Slide 2 and Slide 3). Which of the following areas is unlikely to be involved:
. Phalanges
. Pelvis
. Clavicle
. Femur
. Talus

Correct Answer & Explanation

. Clavicle


Explanation

The clavicle is a membranous bone, and osteochondromas do not arise in membranous bones.

Question 495

Topic: 10. Pathology and Oncology
A 12-year-old boy is brought to the clinic by his concerned parents. The boy's forearm is bowed, and his parents are confused as to the possible diagnosis and treatment options. You notice that the right forearm of the child is bowed ulnarwards and is shorter compared to the left forearm. The pronosupination is markedly decreased on the right side but is also limited on the left side. The patient has a good grip, pinch, and grasp. He is neurologically intact as well. The parents say that they first noticed the deformity around 6 or 7 years ago, and the mother informs you that she had noticed a hard bump on the forearm. She has recently noticed another bump on his right leg. The child does not complain of pain and is using both of his hands quite well. The parents were informed by a previous physician that the child has Madelung's deformity and are concerned that the disease is now involving other areas of his body. You order a radiograph of the forearm. The anteroposterior radiograph is shown (Slide 1). The child's skeletal radiograph survey is also presented (Slide 2 and Slide 3). The most likely complication in this child is:
. Malignant degeneration
. Carpal translocation
. Posterior interosseous neuropathy (PIN) palsy
. Peroneal nerve palsy
. Elbow dislocation

Correct Answer & Explanation

. Carpal translocation


Explanation

Ulnar carpal translocation occurs due to the steep radial articular angulation that occurs due to the tethering effect of a shortened ulna and is already apparent in early stages in the first radiograph. While peroneal palsy is possible due to a proximal fibula lesion, it is less common. Malignant transformation occurs, risk varies with families.

Question 496

Topic: 10. Pathology and Oncology
A 12-year-old boy is brought to the clinic by his concerned parents. The boy's forearm is bowed, and his parents are confused as to the possible diagnosis and treatment options. You notice that the right forearm of the child is bowed ulnarwards and is shorter compared to the left forearm. The pronosupination is markedly decreased on the right side but is also limited on the left side. The patient has a good grip, pinch, and grasp. He is neurologically intact as well. The parents say that they first noticed the deformity around 6 or 7 years ago, and the mother informs you that she had noticed a hard bump on the forearm. She has recently noticed another bump on his right leg. The child does not complain of pain and is using both of his hands quite well. The parents were informed by a previous physician that the child has Madelung's deformity and are concerned that the disease is now involving other areas of his body. You order a radiograph of the forearm. The anteroposterior radiograph is shown (Slide 1). The child's skeletal radiograph survey is also presented (Slide 2 and Slide 3). Which of the following is not true regarding the possibility of malignant degeneration in this child:
. There is a risk of up to 25%
. Bone scan can differentiate
. Most common secondary malignancy is chondrosarcoma
. Risk of malignancy varies between families
. Malignant change occurs in adulthood

Correct Answer & Explanation

. Bone scan can differentiate


Explanation

Bone scan cannot differentiate between an active lesion and a malignant transformation.

Question 497

Topic: 10. Pathology and Oncology

A 25-year-old male presents with knee pain. Radiographs show an eccentric, expansile, purely lytic lesion in the distal femoral epiphysis. Biopsy reveals multinucleated giant cells. Which genetic mutation is highly sensitive and specific for this tumor?

. EXT1
. GNAS
. H3F3A
. t(11;22) EWSR1-FLI1

Correct Answer & Explanation

. H3F3A


Explanation

Giant Cell Tumor of bone is classically an epiphyseal/metaphyseal lesion in skeletally mature patients. It is characterized molecularly by a mutation in the H3F3A (Histone H3.3) gene in over 90% of cases.

Question 498

Topic: 10. Pathology and Oncology

Which of the following clinical or pathologic findings represents the most significant adverse prognostic factor for overall survival in a 16-year-old patient diagnosed with high-grade intramedullary osteosarcoma of the distal femur?

. Tumor size greater than 5 cm at presentation
. Elevated serum alkaline phosphatase levels
. Presence of skip lesions in the femur
. Less than 90% tumor necrosis following neoadjuvant chemotherapy
. Presence of macroscopic pulmonary metastases at presentation

Correct Answer & Explanation

. Presence of macroscopic pulmonary metastases at presentation


Explanation

While poor histologic response to chemotherapy (<90% necrosis) is a very strong prognostic factor, the presence of macroscopic metastatic disease (typically pulmonary) at presentation is the single most significant adverse prognostic indicator for overall survival.

Question 499

Topic: 10. Pathology and Oncology

Which of the following bone tumors is characterized histologically by a proliferation of mononuclear cells and multinucleated giant cells, frequently harboring an H3F3A gene mutation?

. Osteosarcoma
. Chondroblastoma
. Giant cell tumor of bone
. Aneurysmal bone cyst
. Ewing sarcoma

Correct Answer & Explanation

. Giant cell tumor of bone


Explanation

Giant cell tumor of bone is a locally aggressive benign neoplasm characterized by multinucleated giant cells. Up to 95% of these tumors harbor a specific mutation in the H3F3A histone gene.

Question 500

Topic: 10. Pathology and Oncology

A 30-year-old black woman presents with complaints of pain in the tip of her right index finger. The pain started approximately 6 months ago and becomes intense in cold weather. She also states that her nail on the index finger does not look as good as the others despite regular manicures. You notice a bluish discoloration and ridging of the nail. The nail is not split, but it appears clubbed. The patient does not have a history of respiratory or hemodynamic disease and appears healthy. The nail is exquisitely tender on pressure, but no mass is palpable. Two-point discrimination is intact and capillary refill is good. Radiographs do not reveal bony destruction, but you notice pressure indentation over the distal phalanx. The joint space is preserved, and the patient has full range of motion. The most likely diagnosis is:

. Glomus tumor
. Acute perinychia
. Paronychia
. Giant cell tumor
. Turret exostosis

Correct Answer & Explanation

. Glomus tumor


Explanation

This patient presents with the classic triad of glomus tumor: sharp lancinating pain, point tenderness, and cold sensitivity. Localized bluish discoloration is also strongly suggestive of a glomus tumor. Giant cell tumors can be found on the fingertip, however, a presentation of a giant cell tumor with these symptoms would be unusual. Mucous cysts would be part of the differential but does not cause a bluish discoloration or cold insensitivity. An acute paronychia would be painful and erythematous is infectious. A mucous cyst is not infectious. This classic triad does not describe a turret exotosis.