10. Pathology and Oncology MCQs

A neonate is diagnosed with Spondylothoracic Dysplasia (Jarcho-Levin syndrome). What is the primary cause of early mortality in patients with this specific condition?

A 45-year-old man presents with chronic knee pain and mechanical catching. Radiographs demonstrate multiple intra-articular calcifications of relatively uniform size.

Which of the following describes the underlying pathophysiology of this condition?

A 24-year-old male presents with a painless, slow-growing nodule on the volar aspect of his distal forearm. Biopsy reveals a granuloma-like architecture with central necrosis and epithelioid cells. Immunohistochemistry shows a loss of INI-1 (SMARCB1) expression. Due to the high rate of a specific metastatic pathway, which of the following evaluations should be included in his staging?

A 22-year-old female undergoes marginal excision of an incidentally found thigh mass. Pathology demonstrates alveolar soft-part sarcoma (ASPS). In addition to a chest CT, which of the following imaging modalities is uniquely indicated for staging this specific sarcoma?

A 35-year-old male presents with a slow-growing, painful mass near his knee joint. Biopsy reveals a biphasic tumor with both epithelial and spindle cell components. Cytogenetic analysis is most likely to show which of the following translocations?

A 42-year-old female presents with chronic knee pain, catching, and reduced range of motion. Radiographs demonstrate multiple stippled intra-articular loose bodies of relatively uniform size.

Which of the following describes the pathophysiology of this condition?

A 40-year-old male undergoes excision of a deep thigh mass. Pathology reveals a myxoid background, proliferating lipoblasts, and a distinct arborizing "chicken-wire" capillary network. Which of the following is true regarding this tumor's staging and behavior?

A 32-year-old male presents with a slowly enlarging, nodular skin lesion on his trunk that has been present for years. Biopsy reveals a storiform pattern of spindle cells that are CD34 positive. Which targeted therapy is FDA-approved for unresectable cases of this tumor?

A 15-year-old boy presents with a rapidly growing, deep soft tissue mass in his forearm. Histology shows aggregates of small round blue cells with central loss of cellular cohesion, resembling pulmonary alveoli. Which cytogenetic abnormality is most characteristic of this diagnosis?

A 65-year-old female undergoes marginal excision of an unexpectedly malignant, deep 8 cm mass in her posterior thigh. Pathology demonstrates highly pleomorphic spindle cells with bizarre giant cells, frequent atypical mitoses, and no specific line of differentiation on immunohistochemistry. What is the most appropriate next step in management?

A 24-year-old male presents with a firm, painless subcutaneous nodule on his volar forearm that he noticed 6 months ago. It is slowly enlarging and now has secondary proximal nodular lesions along the arm. Biopsy shows nodular aggregates of epithelioid cells with central necrosis. Immunohistochemistry will most likely demonstrate a loss of which of the following?

A 30-year-old female complains of recurrent knee effusions and pain. MRI shows a massive joint effusion with extensive synovial proliferation that exhibits low signal on both T1 and T2-weighted images due to hemosiderin. The pathogenesis of this condition is primarily driven by the overexpression of which of the following?

A 50-year-old male is newly diagnosed with a high-grade pleomorphic liposarcoma of the posterior thigh measuring 12 cm. What is the most critical staging study required prior to definitive surgical intervention to assess for systemic disease?

A 25-year-old male presents with a rapidly growing, slightly tender, 2 cm mass on his volar forearm that appeared 3 weeks ago. He reports a minor contusion to the area 1 month ago. Excisional biopsy demonstrates a poorly circumscribed lesion with plump, rapidly proliferating fibroblasts in a myxoid stroma with extravasated red blood cells. There is brisk mitotic activity but no atypical mitoses. What is the most appropriate management?

A 45-year-old male presents with chronic knee pain and mechanical symptoms. Radiographs show multiple loose bodies of uniform size. An MRI shows a joint effusion with multiple cartilaginous nodules.

Which of the following is true regarding this condition?

A 55-year-old presents with a painless, rapidly enlarging thigh mass. An MRI demonstrates a deep 8 cm heterogeneous mass.

When planning a core needle biopsy, which principle must be strictly adhered to?

A 28-year-old male presents with a slowly growing, painful mass near his knee. Radiographs reveal a soft tissue mass with stippled calcifications. Biopsy demonstrates a biphasic pattern of spindle and epithelial cells. What is the characteristic cytogenetic abnormality associated with this tumor?

An older adult presents with a massive, deep intramuscular mass in the posterior thigh. Biopsy shows mature adipocytes interspersed with atypical spindle cells and hyperchromatic stromal cells. Cytogenetic analysis reveals amplification of the MDM2 gene. Which of the following is the most likely diagnosis?

A 40-year-old male is diagnosed with a large myxoid liposarcoma of the thigh.

During staging, which imaging study is specifically indicated for this histologic subtype, unlike most other soft tissue sarcomas?

A 65-year-old female undergoes marginal excision of a presumed benign subcutaneous cyst in her thigh at an outside facility. Final pathology reveals an undifferentiated pleomorphic sarcoma with positive margins. What is the most appropriate next step in management?