Menu

Question 4481

Topic: 10. Pathology and Oncology

A 45-year-old male presents with vague sacral pain and bowel dysfunction. MRI reveals a large, destructive midline sacral mass. A CT-guided biopsy confirms a chordoma. Which of the following statements best characterizes the optimal treatment strategy for this pathology?

. Primary fractionated external beam radiation therapy followed by observation
. Intralesional curettage and cementation
. Wide en bloc surgical resection with negative margins
. Neoadjuvant chemotherapy followed by piecemeal debulking
. Emergent surgical decompression and intralesional excision due to neurologic deficits

Correct Answer & Explanation

. Primary fractionated external beam radiation therapy followed by observation


Explanation

Chordomas are primary malignant bone tumors originating from notochord remnants, commonly found in the sacrum and clivus. They are generally resistant to conventional chemotherapy and radiation. The standard of care for a sacral chordoma to minimize local recurrence and maximize disease-free survival is wide en bloc surgical resection with tumor-free (negative) margins. Intralesional or piecemeal excision has an unacceptably high rate of local recurrence.

Question 4482

Topic: 10. Pathology and Oncology

A 55-year-old female with metastatic renal cell carcinoma to the T8 vertebral body presents with severe back pain and progressive bilateral leg weakness. MRI shows high-grade epidural spinal cord compression. Based on the NOMS framework, what is the optimal management strategy?

. Conventional external beam radiation therapy (cEBRT) alone
. Stereotactic radiosurgery (SRS) alone
. Posterior separation surgery followed by SRS
. Anterior corpectomy and fusion without radiation
. Systemic targeted chemotherapy alone

Correct Answer & Explanation

. Conventional external beam radiation therapy (cEBRT) alone


Explanation

Renal cell carcinoma is a radioresistant tumor. According to the NOMS framework, high-grade epidural spinal cord compression from a radioresistant tumor requires surgical decompression (separation surgery) followed by stereotactic radiosurgery (SRS) to control the local disease.

Question 4483

Topic: 10. Pathology and Oncology

An 80-year-old female presents with severe, worsening back pain 3 months after a minor fall. Initial radiographs at the time of the fall were reported as normal. Current radiographs demonstrate severe vertebral body collapse at T12 with an intravertebral vacuum cleft. What is the most likely diagnosis?

. Multiple myeloma
. Metastatic breast carcinoma
. Pyogenic spondylodiscitis
. Kummell disease
. Tuberculous spondylitis

Correct Answer & Explanation

. Multiple myeloma


Explanation

Kummell disease is delayed, post-traumatic avascular necrosis of a vertebral body. It is classically characterized radiographically by progressive vertebral collapse and the presence of an intravertebral vacuum cleft on extension or traction.

Question 4484

Topic: 10. Pathology and Oncology

A 14-year-old boy presents with a destructive diaphyseal lesion of the femur. Biopsy reveals small round blue cells. Molecular testing demonstrates a t(11;22)(q24;q12) chromosomal translocation. This specific genetic aberration results in the formation of which fusion gene?

. SYT-SSX
. EWS-FLI1
. PAX3-FKHR
. TLS-CHOP
. COL1A1-PDGFB

Correct Answer & Explanation

. SYT-SSX


Explanation

The t(11;22)(q24;q12) translocation is the hallmark of Ewing sarcoma, resulting in the EWS-FLI1 fusion protein, which acts as an aberrant transcription factor. SYT-SSX is synovial sarcoma t(x;18). PAX3-FKHR is alveolar rhabdomyosarcoma. TLS-CHOP is myxoid liposarcoma.

Question 4485

Topic: 10. Pathology and Oncology



A 12-year-old boy presents with an aggressive diaphyseal bone tumor. Cytogenetic analysis of the biopsy specimen reveals a t(11;22)(q24;q12) chromosomal translocation. Which of the following fusion proteins is characteristically produced as a result of this translocation?

. SYT-SSX
. EWS-FLI1
. TLS-CHOP
. PAX3-FKHR
. BCR-ABL

Correct Answer & Explanation

. SYT-SSX


Explanation

The t(11;22)(q24;q12) translocation is the classic cytogenetic hallmark of Ewing sarcoma, resulting in the EWS-FLI1 fusion protein in approximately 85% of cases. SYT-SSX is associated with synovial sarcoma [t(X;18)]. TLS-CHOP is seen in myxoid liposarcoma [t(12;16)]. PAX3-FKHR is seen in alveolar rhabdomyosarcoma [t(2;13)]. BCR-ABL is characteristic of chronic myelogenous leukemia [t(9;22), Philadelphia chromosome].

Question 4486

Topic: 10. Pathology and Oncology

A 45-year-old female presents with a painless, slowly enlarging, firm, lobulated mass on the volar aspect of her index finger. X-rays show soft tissue swelling with no bone involvement.

Histological examination following excision is most likely to show:

. Mucin-filled pseudocyst with no epithelial lining
. Multinucleated giant cells, foam cells, and hemosiderin-laden macrophages
. Proliferation of glomus cells surrounding small blood vessels
. Mature adipocytes separated by thin fibrous septa
. Granulomatous inflammation with caseating necrosis

Correct Answer & Explanation

. Mucin-filled pseudocyst with no epithelial lining


Explanation

The clinical picture describes a giant cell tumor of the tendon sheath, the second most common soft tissue mass in the hand (after ganglion cysts). Histologically, it is characterized by multinucleated giant cells, lipid-laden macrophages (foam cells), and hemosiderin deposits. It is considered a localized form of pigmented villonodular synovitis (PVNS).

Question 4487

Topic: 10. Pathology and Oncology

A 45-year-old female complains of severe, paroxysmal pain in her thumbnail bed, exacerbated by cold temperatures. Exquisite point tenderness is noted on physical exam. What is the most likely diagnosis?

. Enchondroma
. Mucous cyst
. Glomus tumor
. Squamous cell carcinoma
. Pyogenic granuloma

Correct Answer & Explanation

. Enchondroma


Explanation

Glomus tumors are benign vascular hamartomas commonly found in the subungual region. They present with the classic triad of cold sensitivity, severe localized pain, and pinpoint tenderness (Love's test).

Question 4488

Topic: 10. Pathology and Oncology

A 28-year-old industrial painter accidentally injects his left index finger with a high-pressure paint gun. He presents to the ED 2 hours later with a small, seemingly benign puncture wound on the volar tip of the finger, but complains of intense, throbbing pain. What is the single most important prognostic factor determining the likelihood of eventual amputation in this injury?

. Volume of the injected material
. Pressure of the injection device
. Type of material injected
. Time elapsed from injury to surgical debridement
. Age of the patient

Correct Answer & Explanation

. Volume of the injected material


Explanation

While time to surgery and pressure of injection are important variables, the single most critical prognostic factor in high-pressure injection injuries is the TYPE of material injected. Organic solvents, oil-based paints, and paint thinners cause severe, rapid tissue necrosis and have the highest amputation rates (often >60-80%). Water-based paints or grease have significantly lower rates of amputation.

Question 4489

Topic: 10. Pathology and Oncology

A 40-year-old female presents with severe, pinpoint pain under the nail of her left ring finger. The pain is exacerbated by cold weather. On examination, there is a bluish discoloration beneath the nail plate. Love's pin test is positive. The pathology underlying this condition originates from which of the following cell types?

. Endothelial cells
. Schwann cells
. Modified smooth muscle cells
. Fibroblasts
. Keratinocytes

Correct Answer & Explanation

. Endothelial cells


Explanation

The clinical presentation is classic for a glomus tumor, characterized by the triad of severe localized pain, point tenderness (Love's pin test), and cold sensitivity. Glomus tumors are benign hamartomas originating from the neuromyoarterial glomus body, which regulates body temperature. The specific cells of origin are the glomus cells, which are modified smooth muscle cells located in the stratum reticulare of the dermis.

Question 4490

Topic: 10. Pathology and Oncology

A 30-year-old female presents with exquisite pinpoint pain in the pulp of her index finger, which worsens significantly upon exposure to cold water. Examination reveals pinpoint tenderness (positive Love's test), and her pain is relieved when a tourniquet is applied to the base of the digit (positive Hildreth's sign). The lesion responsible for these symptoms arises from which of the following cellular origins?

. Schwann cells
. Eccrine sweat glands
. Fibroblasts
. Neuromyarterial apparatus
. Vascular endothelial cells

Correct Answer & Explanation

. Schwann cells


Explanation

The clinical presentation is classic for a glomus tumor, characterized by the triad of severe pain, cold intolerance, and point tenderness. Love's test (pinpoint pain) and Hildreth's sign (relief of pain with a proximal tourniquet) are highly specific. Glomus tumors are benign hamartomas arising from the neuromyoarterial glomus body, an apparatus responsible for thermoregulation.

Question 4491

Topic: 10. Pathology and Oncology

A 42-year-old woman presents with severe, excruciating pain at the tip of her left index finger, which worsens in cold weather. There is point tenderness over the nail bed (Love's pin test), and the pain is relieved when a tourniquet is applied to the base of the finger (Hildreth's sign). What is the most likely diagnosis?

. Epidermal inclusion cyst
. Giant cell tumor of the tendon sheath
. Enchondroma
. Glomus tumor
. Mucous cyst

Correct Answer & Explanation

. Epidermal inclusion cyst


Explanation

A glomus tumor is a benign, painful hamartoma of the glomus body (a thermoregulatory structure) most commonly found in the subungual region. The classic clinical triad includes cold hypersensitivity, paroxysmal severe pain, and pinpoint tenderness. Hildreth's test (relief of pain with ischemia) is highly specific.

Question 4492

Topic: 10. Pathology and Oncology

A 40-year-old woman presents with severe pain in her right middle finger, exacerbated by cold weather. Examination reveals pinpoint tenderness beneath the nail plate. Pain is relieved when a tourniquet is applied to the base of the finger. What is the most likely diagnosis?

. Enchondroma
. Mucous cyst
. Glomus tumor
. Schwannoma
. Epidermal inclusion cyst

Correct Answer & Explanation

. Enchondroma


Explanation

A glomus tumor presents with the classic triad of cold sensitivity, pinpoint point tenderness (Love's pin test), and relief of pain with ischemia (Hildreth's test). It is a benign hamartoma of the neuromyoarterial glomus body.

Question 4493

Topic: 10. Pathology and Oncology

During a complicated resection of a palmar tumor, the vascular supply of the hand is meticulously dissected. The deep palmar arch is primarily formed by the terminal continuation of which artery?

. Ulnar artery
. Radial artery
. Anterior interosseous artery
. Posterior interosseous artery
. Median artery

Correct Answer & Explanation

. Ulnar artery


Explanation

The deep palmar arch is primarily formed by the terminal continuation of the radial artery, which anastomoses with the deep palmar branch of the ulnar artery. Conversely, the superficial palmar arch is primarily formed by the continuation of the ulnar artery, anastomosing with the superficial palmar branch of the radial artery.

Question 4494

Topic: 10. Pathology and Oncology

A 65-year-old female with a history of breast cancer presents with right thigh pain. Radiographs reveal a 2 cm lytic lesion in the peritrochanteric region of the proximal femur. It is moderately painful with weight-bearing but does not involve the cortex circumferentially. Based on Mirels' criteria, what is her calculated score, and what is the recommendation for prophylactic fixation?

. Score 7; Observation with radiation therapy
. Score 8; Borderline, consider prophylactic fixation
. Score 9; Prophylactic fixation recommended
. Score 10; Prophylactic fixation strongly recommended
. Score 11; Immediate prophylactic fixation and arthroplasty

Correct Answer & Explanation

. Score 7; Observation with radiation therapy


Explanation

Mirels' criteria assess the risk of pathological fracture based on four factors (Site, Pain, Lesion type, Size). Site: Peritrochanteric = 3 points. Pain: Moderate = 2 points. Lesion type: Lytic = 3 points. Size: 1/3 to 2/3 of cortex roughly equates to 2 points. 3+2+3+1 = 9. A score of 9 or greater indicates prophylactic internal fixation is recommended.

Question 4495

Topic: Bone Tumors

An 8-year-old boy presents with mild right arm pain after throwing a ball. Radiographs reveal a central, lytic, well-circumscribed lesion in the proximal humeral metaphysis with a "fallen leaf" sign. Assuming the fracture has healed and the lesion remains asymptomatic, what is the most appropriate natural history expectation and management?

. Progression to osteosarcoma requires en bloc resection
. High risk of malignant transformation dictates radiation therapy
. It is a unicameral bone cyst (UBC) that will likely resolve by skeletal maturity; observation is indicated
. It is an aneurysmal bone cyst (ABC) requiring immediate intralesional curettage
. Progressive diaphyseal expansion requiring prophylactic internal fixation

Correct Answer & Explanation

. Progression to osteosarcoma requires en bloc resection


Explanation

The "fallen leaf" (or fallen fragment) sign is pathognomonic for a Unicameral Bone Cyst (UBC). Asymptomatic UBCs typically migrate away from the physis into the diaphysis and spontaneously resolve after skeletal maturity.

Question 4496

Topic: 10. Pathology and Oncology

A 10-year-old girl undergoes a knee radiograph for a minor contusion. An incidental eccentric, bubbly, multiloculated lucent lesion with a sclerotic rim is noted in the posteromedial metaphysis of the distal femur. What is the most common natural history of this lesion?

. Spontaneous resolution and ossification by skeletal maturity
. Rapid expansion leading to an inevitable pathologic fracture
. Malignant transformation into a fibrosarcoma
. Invasion into the adjacent epiphysis causing growth arrest
. Requires prophylactic curettage and bone grafting

Correct Answer & Explanation

. Spontaneous resolution and ossification by skeletal maturity


Explanation

This presentation is classic for a Non-Ossifying Fibroma (NOF) or fibrous cortical defect. They are benign, asymptomatic, self-limiting lesions that typically fill in with bone and spontaneously resolve by skeletal maturity.

Question 4497

Topic: 10. Pathology and Oncology

A 55-year-old woman with a history of breast cancer presents with severe, progressive midthoracic back pain. Neurological exam reveals 4/5 strength in the bilateral iliopsoas and hyperreflexia at the knees. MRI shows a destructive lesion at T8 with epidural extension compressing the spinal cord. She has an estimated life expectancy of 18 months. What is the most appropriate management?

. Conventional external beam radiation therapy alone
. High-dose corticosteroids and hospice care
. Surgical decompression and stabilization followed by radiation
. Systemic chemotherapy
. Percutaneous vertebroplasty

Correct Answer & Explanation

. Conventional external beam radiation therapy alone


Explanation

In a patient with metastatic spinal cord compression from a solid tumor who has mechanical instability, progressive neurological deficit, and a reasonable life expectancy (>3-6 months), surgical decompression and stabilization followed by radiation therapy is superior to radiation alone.

Question 4498

Topic: Bone Tumors

A 16-year-old male presents with a 6-month history of well-localized mid-back pain that is worse at night and dramatically improves with ibuprofen. Imaging reveals a 1.2 cm sclerotic lesion with a central lucent nidus in the left lamina of T8. What is the most appropriate definitive management if conservative measures are poorly tolerated?

. Wide en bloc resection and fusion
. External beam radiation therapy
. Computed tomography-guided radiofrequency ablation
. Neoadjuvant chemotherapy followed by marginal resection
. Intralesional corticosteroid injection

Correct Answer & Explanation

. Wide en bloc resection and fusion


Explanation

The clinical presentation and imaging (lesion < 2 cm, posterior elements, nighttime pain relieved by NSAIDs) are classic for an osteoid osteoma of the spine. When medical management (NSAIDs) is poorly tolerated or fails, CT-guided radiofrequency ablation (RFA) is the minimally invasive treatment of choice, provided the nidus is safely distanced from the spinal cord or nerve roots.

Question 4499

Topic: 10. Pathology and Oncology

Within the Neurologic, Oncologic, Mechanical, and Systemic (NOMS) framework for evaluating metastatic spine tumors, which of the following tumor histologies is considered highly radioresistant, often prompting separation surgery followed by stereotactic radiosurgery (SRS) when causing epidural spinal cord compression?

. Multiple myeloma
. Non-Hodgkin lymphoma
. Breast carcinoma
. Renal cell carcinoma
. Prostate carcinoma

Correct Answer & Explanation

. Multiple myeloma


Explanation

Renal cell carcinoma, melanoma, thyroid carcinoma, and gastrointestinal malignancies are classically considered radioresistant to conventional fractionated external beam radiation therapy (cEBRT). In the NOMS framework, high-grade epidural spinal cord compression from these tumors typically requires separation surgery (decompression to create a margin around the cord) followed by high-dose stereotactic radiosurgery (SRS).

Question 4500

Topic: Soft Tissue Tumors & Metastasis

A 60-year-old man presents with severe back pain and a solitary L2 vertebral body metastasis secondary to renal cell carcinoma. He has impending cord compression, and his estimated life expectancy is 18 months. What is the most appropriate surgical treatment strategy?

. External beam radiation therapy alone
. Palliative posterior laminectomy without fusion
. Preoperative embolization followed by en bloc spondylectomy
. Vertebroplasty alone
. Corticosteroids and bracing

Correct Answer & Explanation

. External beam radiation therapy alone


Explanation

Renal cell carcinoma metastases are highly vascular and famously radioresistant. Therefore, optimal treatment for an isolated spinal metastasis requires preoperative embolization (to reduce bleeding) followed by wide excision or en bloc spondylectomy.