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Question 521

Topic: 10. Pathology and Oncology
Which of the following lesions has the highest risk of malignant degeneration?
. Maffucci syndrome
. Ollier disease
. Solitary enchondroma
. Multiple hereditary osteochondromatosis
. None of the above

Correct Answer & Explanation

. Maffucci syndrome


Explanation

Ollier disease (multiple enchondromatosis) is associated with deformities of the axial skeleton and larger, unilateral lesions. The combination of multiple enchondromatosis and venous-lymphatic vascular malformations (angiomas) is termed Maffucci syndrome. Maffucci syndrome is associated with a higher rate of degeneration into chondrosarcoma than in Ollier disease. Malignant change of solitary lesions is rare, but a sudden increase in pain or size should raise suspicion of degeneration.

Question 522

Topic: 10. Pathology and Oncology

The most common primary bone tumor of the hand is:

. Enchondroma
. Giant cell tumor
. Osteochondroma
. Osteoid osteoma
. None of the above

Correct Answer & Explanation

. Enchondroma


Explanation

Enchondromas account for more than 90% of primary bone tumors of the hand.

Question 523

Topic: Bone Tumors

Prolonged nonsteroidal anti-inflammatory drugs (NSAIDs) cure which of the following lesions:

. Osteosarcoma
. Osteoid osteoma
. Osteoblastoma
. Osteochondroma
. None of the above

Correct Answer & Explanation

. Osteoid osteoma


Explanation

An average 33-month course of treatment with NSAIDs cures osteoid osteoma. The prostaglandin E2 in osteoid osteoma is likely the reason for this response.

Question 524

Topic: 10. Pathology and Oncology

The most common primary bone tumor in the hand is:

. Enchondroma
. Osteochondroma
. Osteosarcoma
. Fibroma
. None of the above

Correct Answer & Explanation

. Enchondroma


Explanation

Enchondroma preferentially occurs in the hand, most commonly in the phalanges and metacarpals, and is the most common primary tumor of bone found in the hand.

Question 525

Topic: 10. Pathology and Oncology
A 16-year-old male undergoes neoadjuvant chemotherapy followed by wide excision of a distal femur osteosarcoma. Histologic analysis of the resected specimen reveals 95% tumor necrosis. What does this histologic finding primarily indicate?
. A higher likelihood of local recurrence
. A favorable prognostic indicator for overall survival
. The need to immediately switch to second-line chemotherapy
. A misdiagnosis of the initial biopsy
. The presence of a skip metastasis

Correct Answer & Explanation

. A favorable prognostic indicator for overall survival


Explanation

Tumor necrosis of 90% or greater (Huvos grade III or IV) following neoadjuvant chemotherapy is the single most important prognostic indicator for overall survival in osteosarcoma. It indicates a strong favorable response to the current chemotherapy regimen.

Question 526

Topic: 10. Pathology and Oncology

Which of the following prognostic factors is the most critical determinant of long-term survival in a patient with high-grade, non-metastatic osteosarcoma of the distal femur?

. Tumor volume at initial presentation
. Percentage of tumor necrosis following neoadjuvant chemotherapy
. Pre-treatment serum alkaline phosphatase levels
. Exact anatomic location of the tumor
. The width of surgical margins achieved during resection

Correct Answer & Explanation

. Percentage of tumor necrosis following neoadjuvant chemotherapy


Explanation

The histologic response to neoadjuvant chemotherapy is the single most important prognostic factor for long-term survival in non-metastatic osteosarcoma. A good response is typically defined as greater than 90% tumor necrosis upon histologic evaluation of the resected specimen.

Question 527

Topic: 10. Pathology and Oncology

A 14-year-old boy is diagnosed with high-grade osteosarcoma of the distal femur. He completes neoadjuvant chemotherapy and undergoes wide surgical resection. Which of the following is the most important prognostic factor for his long-term survival?

. Size of the primary tumor at diagnosis
. Histologic response to neoadjuvant chemotherapy
. Age of the patient at diagnosis
. Specific histologic subtype (e.g., osteoblastic vs. chondroblastic)
. Preoperative serum alkaline phosphatase levels

Correct Answer & Explanation

. Histologic response to neoadjuvant chemotherapy


Explanation

The degree of tumor necrosis (histologic response) following neoadjuvant chemotherapy is the single most important prognostic indicator for overall survival in patients with high-grade, non-metastatic osteosarcoma.

Question 528

Topic: 10. Pathology and Oncology

A 15-year-old girl undergoes neoadjuvant chemotherapy and subsequent wide resection for a conventional high-grade distal femur osteosarcoma. Which of the following is the most significant prognostic factor for her overall survival?

. Initial tumor volume
. Histologic response to chemotherapy (percentage of tumor necrosis)
. Patient age at diagnosis
. Pre-treatment serum alkaline phosphatase level
. Exact anatomic location within the femur

Correct Answer & Explanation

. Histologic response to chemotherapy (percentage of tumor necrosis)


Explanation

The histologic response to neoadjuvant chemotherapy, specifically having greater than 90% tumor necrosis, is the single most important prognostic indicator for overall survival in conventional osteosarcoma.

Question 529

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a destructive diaphyseal lesion in the femur with a permeative pattern and an "onion skin" periosteal reaction. Cytogenetic testing reveals a specific chromosomal translocation. Which translocation is most characteristic of this tumor?

. t(11;22)
. t(9;22)
. t(X;18)
. t(12;16)
. t(2;13)

Correct Answer & Explanation

. t(11;22)


Explanation

The clinical and radiographic presentation is classic for Ewing sarcoma. Over 90% of Ewing sarcomas are characterized by the t(11;22)(q24;q12) translocation, resulting in the EWS-FLI1 fusion protein.

Question 530

Topic: 10. Pathology and Oncology

Which of the following prognostic factors is considered the most significant predictor of overall survival in a patient with localized osteosarcoma of the distal femur treated with neoadjuvant chemotherapy and wide resection?

. Tumor size at presentation
. Histologic subtype
. Percentage of tumor necrosis found in the resected specimen
. Age of the patient
. Preoperative serum alkaline phosphatase levels

Correct Answer & Explanation

. Percentage of tumor necrosis found in the resected specimen


Explanation

The degree of tumor necrosis following neoadjuvant chemotherapy is the single most important prognostic factor for overall survival in patients with localized osteosarcoma. Greater than 90% necrosis indicates a favorable response.

Question 531

Topic: 10. Pathology and Oncology

A 16-year-old male is diagnosed with osteosarcoma of the distal femur. Genetic analysis of the tumor reveals a mutation in a tumor suppressor gene heavily implicated in cell cycle regulation at the G1/S checkpoint. Patients with a germline mutation in this gene have a high incidence of bilateral retinoblastoma. Which gene is this?

. p53
. RB1
. BRCA1
. APC
. PTEN

Correct Answer & Explanation

. RB1


Explanation

The RB1 gene is a tumor suppressor gene that regulates the cell cycle at the G1/S checkpoint. Germline mutations result in hereditary retinoblastoma and significantly increase the lifetime risk of developing secondary primary malignancies, most notably osteosarcoma.

Question 532

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a diaphyseal femur lesion showing an "onion-skin" periosteal reaction. A biopsy reveals small, round blue cells. Which specific chromosomal translocation is most characteristically associated with this diagnosis?

. t(9;22)
. t(11;22)
. t(X;18)
. t(12;16)
. t(2;13)

Correct Answer & Explanation

. t(11;22)


Explanation

Ewing sarcoma is characterized by a diaphyseal location, onion-skin periosteal reaction, and small round blue cells on histology. The classic genetic abnormality is the t(11;22) translocation, resulting in the EWS-FLI1 fusion protein.

Question 533

Topic: 10. Pathology and Oncology

Which of the following is true of interdigital neuromas:

. Interdigital neuromas are a benign neoplasm of the Schwann cell
. Interdigital neuromas most commonly occur in the second web space
. Numbness is a common finding in interdigital neuromas
. Interdigital neuromas most commonly occur in the third web space
. There is an equal incidence of interdigital neuromas in men and women

Correct Answer & Explanation

. Interdigital neuromas most commonly occur in the third web space


Explanation

Discussion Important core knowledge of interdigital neuroma (Morton's syndrome) includes: A. Etiology-neither a neuroma nor a tumor; most likely degenerative from repetitive trauma from the transverse intermetatarsal ligament. 1. Pathology-most commonly perineural fibrosis B. Most common in the third web space (about 80%) 1. Medial branch of the lateral plantar nerve joins the lateral branch of medial plantar curve C . More common in women than men D. Presentation 1. Paresthesias, dysesthesias, burning sensation 2. Diffuse symptoms in forefoot 3. Pain is relieved by massaging the foot 4. Numbness occurs as a late finding E. Physical examination 1. Tenderness to direct pressure in the web space 2. Tenderness with forefoot compression

Question 534

Topic: 10. Pathology and Oncology

Slide 1 Slide 2 Slide 3 Slide 4 A 35-year-old woman has a swollen and painful small toe. The radiographs of her foot are shown in Slides 1 and 2, and biopsy specimens in Slides 3 and 4. The most likely diagnosis is:

. Enchondroma
. Plasmacytoma
. C hondromyxoid fibroma
. C hondroblastoma
. Epidermoid inclusion cyst

Correct Answer & Explanation

. Epidermoid inclusion cyst


Explanation

The radiographs show an expansile, lytic destructive lesion, which has replaced the distal and middle phalanxes of the little toe. The biopsy specimen shows the squamous epithelium of the skin with keratin production extending into the medullary cavity of the bone. The patient has an epidermoid inclusion cyst. These cysts are usually secondary to trauma. The treatment should be curettage and grafting.C orrect Answer: Epidermoid inclusion cyst

Question 535

Topic: 10. Pathology and Oncology

A 35-year-old woman has a swollen and painful small toe. The radiographs of her foot are shown in Slides 1 and 2, and biopsy specimens are shown in Slides 3 and 4. The most appropriate treatment would be:

. C urettage with or without grafting
. Toe amputation
. External beam irradiation
. C urettage and external beam irradiation
. Toe amputation and chemotherapy

Correct Answer & Explanation

. C urettage with or without grafting


Explanation

The radiographs show an expansile, lytic destructive lesion, which has replaced the distal and middle phalanxes of the little toe. The biopsy specimen shows the squamous epithelium of the skin with keratin production extending into the medullary cavity of the bone. The patient has an epidermoid inclusion cyst. These cysts are usually secondary to trauma. The treatment should be curettage and grafting.

Question 536

Topic: 10. Pathology and Oncology

A 40-year-old man has pain in his foot after minor trauma. A radiograph is shown in Slide 1 and biopsy specimens are shown in Slides 2 and 3. The most likely diagnosis is:

. Low-grade chondrosarcoma
. High-grade chondrosarcoma
. Enchondroma
. C hondroblastoma
. C hondromyxoid fibroma

Correct Answer & Explanation

. Enchondroma


Explanation

The radiograph shows a lucent lesion in the proximal phalanx of the second toe and expansion of the proximal phalanx. The bone is expanded with a thin periosteal rim. Faint calcifications are also seen within the medullary cavity. This radiographic appearance is characteristic of an enchondroma. The low-power hematoxylin and eosin stain shows abundant blue hyaline matrix and a paucity of cells. The high-power hematoxylin and eosin stain shows the small dark and uniform nuclei present in enchondromas. No pleomorphism or nuclear atypia are present.

Question 537

Topic: 10. Pathology and Oncology

A 40-year-old man has pain in his foot after minor trauma. A radiograph is shown in Slide 1 and biopsy specimens are shown in Slides 2 and 3. Which of the following best describes this condition:

. Benign bone tumor
. Low-grade malignant bone tumor
. High-grade malignant bone tumor
. Benign reactive lesion (non-neoplastic)
. Metabolic condition

Correct Answer & Explanation

. Benign bone tumor


Explanation

The radiograph shows a lucent lesion in the proximal phalanx of the second toe and expansion of the proximal phalanx. The bone is expanded with a thin periosteal rim. Faint calcifications are also seen within the medullary cavity. This radiographic appearance is fairly characteristic of an enchondroma. The low-power hematoxylin and eosin stain shows abundant blue hyaline matrix and a paucity of cells. The high-power hematoxylin and eosin stain shows the small dark and uniform nuclei present in enchondromas. No pleomorphism or nuclear atypia are present.

Question 538

Topic: 10. Pathology and Oncology

A 35-year-old man has experienced foot pain for 6 months. The anteroposterior and oblique radiographs are shown in Slides 1 and 2, and a biopsy specimen is shown in Slide 3. The most likely diagnosis is:

. Metastatic adenocarcinoma
. Multiple myeloma
. Eosinophilic granuloma (Langerhans cell histiocytosis)
. Aneursymal bone cyst
. Hemangioendothelioma

Correct Answer & Explanation

. Hemangioendothelioma


Explanation

The radiographs show lytic destructive lesions in the first and second metatarsals. Malignant vascular tumors, such as hemangioendothelioma, characteristically manifest as multiple lytic lesions in the same extremity in young patients. The biopsy shows vasoformative cells, which are trying to form blood vessels. The correct diagnosis is hemangioendothelioma of bone, which is a low-grade malignancy with a low risk of pulmonary metastases. Patients are typically treated with external beam irradiation. This patient opted for below-knee amputation. He could have also been treated with irradiation.

Question 539

Topic: 10. Pathology and Oncology

A 35-year-old man has foot pain for 6 months. The anteroposterior and oblique radiographs are shown in Slides 1 and 2, and a biopsy specimen is shown in Slide 3. Which of the following treatments is the most appropriate:

. Preoperative chemotherapy and wide resection
. Wide resection and external beam irradiation
. C hemotherapy and external beam irradiation
. Wide resection alone
. External beam irradiation alone

Correct Answer & Explanation

. External beam irradiation alone


Explanation

The radiographs show lytic destructive lesions in the first and second metatarsals. Malignant vascular tumors, such as hemangioendothelioma, characteristically manifest as multiple lytic lesions in the same extremity in young patients. The biopsy shows vasoformative cells, which are trying to form blood vessels. The correct diagnosis is hemangioendothelioma of bone, which is alow-grade malignancy with a low risk of pulmonary metastases. Patients are typically treated with external beam irradiation. This patient opted for below-knee amputation. He could have also been treated with irradiation.

Question 540

Topic: 10. Pathology and Oncology

A 35-year-old man has foot pain for 6 months. The anteroposterior and oblique radiographs are shown in Slides 1 and 2, and a biopsy specimen is shown in Slide 3. The etiology of this condition is most likely:

. Benign neoplasm
. Low-grade malignant neoplasm
. High-grade malignant neoplasm
. Post-traumatic
. Infectious

Correct Answer & Explanation

. Low-grade malignant neoplasm


Explanation

The radiographs show lytic destructive lesions in the first and second metatarsals. Malignant vascular tumors, such as hemangioendothelioma, characteristically manifest as multiple lytic lesions in the same extremity in young patients. The biopsy shows vasoformative cells, which are trying to form blood vessels. The correct diagnosis is hemangioendothelioma of bone, which is a low-grade malignancy with a low risk of pulmonary metastases. Patients are typically treated with external beam irradiation. This patient opted for below-knee amputation. He could have also been treated with irradiation.